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27th January 2025
Professor Christopher Denton, consultant rheumatologist at the Royal Free Hospital in London, led a recent British Society for Rheumatology guideline working group on managing systemic sclerosis. Here, as lead author of the published recommendations, he explains key aspects of the guideline and highlights new and emerging advances in treatment for this challenging condition.
Systemic sclerosis is a severe and incurable autoimmune rheumatic disease that causes damage and fibrosis in the skin, blood vessels and vital organs. It also impacts hand function, and almost all aspects of everyday living are affected.
It is uncommon, affecting around one in 10,000 of the population. Unfortunately, it has a high mortality and even greater morbidity through its impact on the lungs, heart, kidneys and gastrointestinal tract.1
The first guideline covering the management of systemic sclerosis in all age groups were published in September 2024 by the British Society for Rheumatology following working group discussions among healthcare professionals with expertise in systemic sclerosis, people with lived experience of the condition and patient organisation representatives.2 This is an updated version of an earlier 2015 guideline that only covered adults with this complex disease.3
Systemic sclerosis is a diverse disease, so guidelines need to consider the differences in how people may present with it. Often it takes time to make a firm diagnosis, which can lead to treatment delays.
Early diagnosis using techniques such as capillaroscopy and antinuclear autoantibody testing is important and allows stratification of risk for future complications, including lung fibrosis and severe renal involvement.4
Once diagnosed, there should be a systematic baseline assessment of all the organs that might be affected and regular follow-up testing through the disease course. A particular group highlighted in the new guideline is the subset of cases associated with concurrent malignancy.
This group has certain characteristics that allow for early recognition, including certain antibody associations and clinical features. The approach to these cases is analogous to that taken with adult-onset dermatomyositis.
In line with other recently published recommendations and aligned with the British Society for Rheumatology guideline protocol, young people with systemic sclerosis are included in the recommendations for the first time. This was achieved through strong engagement with the paediatric rheumatology community. For each of the evidence-based recommendations, a statement about relevance to children and adolescent patients is included.
The British Society for Rheumatology guideline includes four key sections:
Each section is framed around key questions that are front and centre of systemic sclerosis management, such as the best approaches to treating heart, lung or gastrointestinal complications. There is also much emphasis on non-lethal manifestations such as digital ulcers as well as non-pharmacological treatments.
The fourth section of the guideline covers specific aspects of service delivery within the NHS. It will help to plan the recommended practice and provide templates and standards for service and patient-specific future audit.
There has been nearly a decade of progress in managing systemic sclerosis, which is captured in the updated guideline.
There are now evidence-based treatments, approved drugs for interstitial lung disease in systemic sclerosis, and more robust support for approaches such as autologous haematopoietic stem cell transplantation. The latter has been shown in robust trials to have a profound benefit for appropriate cases, and the guideline includes specific expert direction on the assessment and evaluation of cases for this procedure.
There is growing support for the treatment of vascular disease, including pulmonary arterial hypertension, and the updated guideline complements guidance from other relevant bodies such as the World Symposium for Pulmonary Hypertension.5
The first major area of progress has been advances in cellular therapy. Large, robust clinical trials and registry studies have confirmed the long-term benefit of autologous haematopoietic stem cell transplantation in poor prognosis systemic sclerosis.6 The new guideline covers this for adults and highlights that it is also a treatment option for juvenile-onset systemic sclerosis.
Critical guidance to help select and evaluate patients so that maximum benefit and minimal harm are achieved is included with simple summaries for case selection. This treatment shows what can be accomplished and provides a pivotal demonstration of disease modification.
Progress has also been made in managing vasculopathy in systemic sclerosis and therefore scleroderma renal crisis, digital ulcer disease, Raynaud’s phenomenon and pulmonary hypertension are all covered clearly in the guideline. These sections summarise evidence-based practice and highlight that systemic sclerosis is as much a cardiovascular disease as a fibrotic condition.7
However, fibrosis of the lung is now the most frequent cause of systemic sclerosis-associated death, and there have been studies providing evidence for treatment.
The guideline includes expert direction on the evaluation and treatment of interstitial lung disease, including a combination of immunosuppression with antifibrotic treatment (nintedanib) in cases of progressive disease.8
Other approaches have been approved based on clinical trial results, including biological therapy with tocilizumab and rituximab.9 These may be especially effective in certain stages and subsets of systemic sclerosis, as indicated in the guideline.
While not yet routinely available in the NHS, it is hoped that the growing evidence base for these biological agents and general utilisation in other global healthcare settings will facilitate access to these treatments in the future. It is likely that, at present, the use will generally be restricted to cases of sclerosis fulfilling the requirements to access rituximab or tocilizumab for other manifestations of overlap syndrome such as arthritis, myositis or vasculitis.
Despite progress in treatment for some of the most serious aspects of systemic sclerosis, including interstitial lung disease, pulmonary hypertension and scleroderma renal crisis, there is still much more to address.
As survival overall has improved, the importance of non-lethal burden is pivotal for people living with systemic sclerosis. Inclusion of people with lived experience in the guideline working group highlighted this challenge, as did the strong involvement and endorsement of the guideline by the charity Scleroderma and Raynaud’s UK.
The British Society for Rheumatology guideline protocol includes an expectation that the guideline will be revised and updated. There is unprecedented interest in systemic sclerosis and its complications from the pharmaceutical industry and many clinical trials are ongoing.
The results of such trials, generally adding new potential treatments to background standard of care immunosuppression, will provide the evidence base for future guidelines. Still, it is likely that as one therapeutic mountain is scaled, another challenging peak will emerge. The updated guideline for systemic sclerosis represents an important milestone in the journey but the final destination is still some way off.
Christopher Denton PhD FRCP FMedSci
Consultant rheumatologist, Royal Free London NHS Foundation Trust, UK
24th January 2025
Speaking at Hospital Healthcare Europe’s Clinical Excellence in Respiratory Care event, Ravijyot Saggu discussed the challenges and management of severe respiratory disease in the context of the 2024 European Respiratory Society guideline on managing severe respiratory disease in adults.
In 2024, the European Respiratory Society (ERS) convened a multiprofessional respiratory task force group to undertake a review of the symptomatic treatment of advanced respiratory disease, noting that there is often a lack of large randomised controlled trials in this area, resulting in evidence for interventions being conflicting and of varying quality.
The result was the creation of a clinical practice guideline and recommendations to support clinicians and the wider multidisciplinary team in delivering optimum patient care in this area.
At the Clinical Excellence event, chair Dr Zaheer Mangera, respiratory consultant and lung cancer lead at North Middlesex University Hospital NHS Trust in London, introduced respiratory and medicines optimisation pharmacist Ravijyot Saggu, one of the guideline co-authors, to share her views.
Ms Saggu, who is also chair of the UKCPA Respiratory Committee and clinical member of the NHS England Respiratory Clinical Reference Group, discussed the challenges of respiratory care and severe lung disease, as well as the ERS advanced symptom management guideline 2024 and its implications for practice. Scroll down and click play to watch now.
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28th November 2024
Long-awaited joint UK guidelines for chronic asthma have been finalised, overhauling diagnosis and treatment recommendations in an effort to better manage the condition in primary care and reduce pressure on hospitals.
The final chronic asthma guidance from the National Institute for Health and Care Excellence (NICE), British Thoracic Society (BTS) and the Scottish Intercollegiate Guidelines Network (SIGN) is designed to support clinicians in making accurate diagnoses, promoting good practice, and providing effective, personalised treatment to control and prevent acute asthma attacks.
This is the first time the organisations have collaborated to produce joint UK-wide guidance on the diagnosis and management of chronic asthma for adults, young people and children.
Alongside its publication, NICE, BTS and SIGN have also developed a new joint asthma pathway. This digital resource collates tools and information for in a central hub, providing ‘a seamless user journey across the newly published guideline and existing asthma guidance‘.
In June, draft guidance revealed significant changes to the current treatment approaches. This included replacing the sole use of short-acting beta agonist (SABA) when asthma is first diagnosed with a low-dose combination of inhaled corticosteroids (ICS) and formoterol, which the final guidance has now reinforced.
Indeed NICE emphasised that healthcare professionals should ‘always prescribe maintenance or combination treatments’ rather than the ‘familiar blue “reliever-only” inhaler, when asthma is first diagnosed’.
This is based on evidence which ‘showed that using the combined ICS and formoterol inhalers when required led to people suffering fewer severe asthma attacks’.
The final chronic asthma guidance has also confirmed changes to recommendations on testing for asthma, with a new recommendation to use peak expiratory flow (PEF) variability as a method for diagnosis, which has been added following consultation.
It advised healthcare professionals to use a stepwise series of tests including eosinophil count, FeNO, spirometry and bronchial challenge in patients where the condition is suspected on clinical grounds.
However, NICE warned that these tests – recommended for both children and adults – are ‘not routinely carried out in current practice’, with ‘only a minority of GP practices’ having onsite access to FeNO tests, while bronchial challenge testing is not available at all in primary care.
As such, NICE recognised that there will be a ‘capacity problem’ and that implementing the recommended diagnostic pathway into clinical practice would ‘require significant investment’.
Another addition to the joint guidance following consultation was a recommendation to consider providing an additional metered SABA inhaler plus spacer for emergency use for children under 12 years who may be unable to activate a dry powder inhaler during an acute asthma attack.
NICE’s chief medical officer Professor Jonathan Benger said the new guidance aims to ‘ease pressure’ on the NHS by ‘reducing hospital admissions due to asthma and lowering the use of less effective monitoring tests’.
He continued: ‘Having one clear set of national asthma guidelines is vital to ensure people receive consistent and effective asthma care across the health service, so people across the UK receive the right diagnosis and treatment for them.’
BTS chair Dr Paul Walker said the changes to recommendations for testing ‘will simplify diagnostic processes and help with current diagnostic delays for adults, children and young people’.
‘The treatment changes represent a true pivot in the principles of asthma care and will contribute to improved outcomes,’ he added.
Dr Andy Whittamore, GP and Asthma and Lung UK clinical lead, said the new guidelines ‘have the potential to make a real difference’ to the 7.2 million people in the UK living with asthma.
He said the recommendation to ‘move away from over-reliance’ on SABA inhalers, which is a ‘key driver of poor asthma control’, is ‘particularly welcome’.
Dr Whittamore also highlighted that the changes to diagnostic recommendations will ‘need to be accompanied by good clinician education to ensure they can confidently and reliably work within the new diagnostic pathway’.
On resourcing for testing, he added: ‘It is very positive that the guidelines acknowledge the difficulties in diagnosing asthma correctly and identify poor access to FeNO and spirometry testing as a barrier that can block safe, good quality care.
‘Funding must be made available to support healthcare professionals to deliver these tests and make them available for every person with suspected asthma.’
Chair of the Primary Care Respiratory Society Dr Katherine Hickman, who is also a GP in Bradford, said the new guidelines ‘introduce a systematic and evidence-based approach to diagnosis’ and she is ‘confident these recommendations will help ensure more accurate assessments and better patient care’.
She continued: ‘One of the most promising advancements is the move towards anti-inflammatory reliever therapy (AIR) and maintenance and reliever therapy (MART).
‘This innovative approach marks a significant shift in asthma treatment, offering new hope to patients and healthcare providers alike. It has the potential to transform how we manage asthma by reducing the burden on primary and secondary care, saving lives, and restoring control to patients over their condition.
‘The guidelines give me real hope for the future of asthma care. They represent a critical step in not only improving patient outcomes but also reshaping how asthma is managed at every level of the healthcare system.’
However, Dr Hickman advised GPs that no patient should be switched to a new inhaler without an ‘informed discussion’.
Professor Azeem Majeed, a GP and professor of primary care and public health at Imperial College London, said the recommendation to replace SABA inhalers alone with combination inhalers ‘reflects evidence that this approach can improve overall asthma control’.
He also said the new recommendations on testing will ‘help reduce misdiagnosis and ensure that treatment is appropriate’, but he warned that implementing the joint guideline will ‘require significant investment to ensure widespread access’ to diagnostic methods such as FeNo and spirometry.
Professor Majeed continued: ‘Another concern is ensuring that all patients, regardless of where they live, have equitable access to the recommended tests. Currently, geographic disparities exist in diagnostic capabilities, which could lead to unequal implementation and outcomes between areas.
‘Overcoming capacity and implementation challenges will require investment in diagnostic infrastructure; workforce expansion and training; and collaboration across primary and specialist care.’
Earlier this month, a report from UCLPartners revealed that an app to assist with asthma self-management could save the NHS up to £25m in a year if it were used by 100,000 patients for three months.
In February 2024, the BTS and Primary Care Respiratory Society published a position statement on integrated respiratory care models and the importance of putting patients at their centre.
A version of this article was originally published by our sister publication Pulse.
21st October 2024
Education programmes for healthcare professionals involved in the management of atrial fibrillation can increase adherence to guidelines and improve patient safety, according to a recent trial.
Researchers found that current adherence to treatment guidelines for atrial fibrillation is low across six European countries. They concluded that increased efforts are needed to improve the implementation of guidelines and to optimise the care of patients with atrial fibrillation to prevent the high rate of adverse outcomes.
The STEER-AF trial involved 739 healthcare professionals and 1,732 patients with atrial fibrillation in 70 treatment centres across France, Germany, Italy, Poland, Spain and the UK between May 2022 and February 2023. The average age of participants was 69 years, and 37% were female.
Researchers randomly assigned treatment centres to one of two groups to determine whether a structured educational programme could improve adherence to guideline recommendations and the care of patients with atrial fibrillation.
In the intervention group, a total of 195 healthcare professionals received a structured education programme delivered over 16 weeks, targeting stroke prevention, rhythm control and integrated care. Healthcare professionals spent an average of 9.2 hours on the online learning platform and expert local trainers provided learning support. This was in addition to any existing continued professional development.
In the control group, healthcare professionals received only their existing educational activities.
The researchers tracked how well healthcare professionals followed the European Society of Cardiology (ESC) guidelines, in particular adherence to ESC Class I (strongly recommended treatments) and Class III recommendations (treatments that are not recommended) at baseline and six to nine months later.
The study observed a significant improvement in guideline adherence for rhythm control, with adherence increasing from 21.4% to 33.9% in the intervention group and from 20.5% to 22.9% in the control group.
There was no significant improvement in guideline adherence for stroke prevention, which changed from 63.4% to 67.5% in the intervention group and from 58.6% to 60.9% in the control group.
There was also a significant improvement in the patient-reported secondary outcome that assessed eight domains of integrated atrial fibrillation management, improving to 77.0% with the intervention and to 71.0% in the control group. All results were statistically significant and had a reliable confidence level.
The findings of this study are incorporated into the 2024 ESC Guidelines for atrial fibrillation management, which were presented at the ESC Congress 2024.
Reference
Sterliński, M et al. Design and deployment of the STEEER-AF trial to evaluate and improve guideline adherence: a cluster-randomized trial by the European Society of Cardiology and European Heart Rhythm Association. EP Europace 2024; Jun 28: DOI: 10.1093/europace/euae178.
16th September 2024
The European Respiratory Society convened a multidisciplinary respiratory task force group in 2024 to create a clinical practice guideline including recommendations for symptomatic treatment of advanced respiratory diseases. Ravijyot Saggu, co-author of the guideline, summarises the findings and details how holistic, patient-centred care is required.
A 2019 report highlighted that chronic respiratory diseases were the third leading cause of death and had a high prevalence of 454.6 million cases globally.1 What’s more, the burden of chronic respiratory disease can be significant for patients and carers, adversely impacting health-related quality of life (HRQoL).
The symptoms of respiratory disease are not exhaustive. Exacerbations aside, they often include anxiety and depression, but fatigue and breathlessness are prominent features in more advanced disease.
Many patients with advanced lung disease suffer from breathlessness, which can be limiting.
Breathlessness can be frightening and debilitating and lead to both psychological and functional decline,2 propagating inactivity and deconditioning, which further increases risks of falls and mortality.3 It is also associated with high healthcare utilisation. In England, for example, it accounts for 5% of presentations to emergency departments and approximately 4% of general practitioner consultations, as well as being reported by patients in 12% of medical admissions.4
A combination of pharmacological and non-pharmacological approaches to management is often beneficial. Breathlessness is not always due to hypoxia, which can sometimes be misunderstood by patients. Education is important to manage expectations and concerns.
There is often a lack of large randomised controlled trials in advanced symptom management of lung disease. Any available evidence for interventions, which may include palliation, is mixed, of varying quality or conflicting.
To address this need, the European Respiratory Society (ERS) convened a multiprofessional respiratory task force group to undertake a review, create a clinical practice guideline and set out recommendations for the symptomatic treatment of advanced respiratory illness. The group also included patients affected by severe respiratory illness and informal carers. The resulting clinical practice guideline was published in the European Respiratory Journal in May 2024.5
The task force defined ‘serious respiratory illness’ as a respiratory condition that carries a high risk of mortality; negatively impacts quality of life and daily function; and/or is burdensome in symptoms, treatments, or caregiver stress.5,6
The task force adopted a rigorous approach, following ERS methodology and setting out Population, Intervention, Comparison, Outcome (PICO) questions for six interventions and one narrative question. Systematic reviews were undertaken, and as expected, there were some limitations on the included populations, the methods and the quality of evidence available.
The patient population in the trials reviewed included mixed lung diseases but chronic obstructive pulmonary disease (COPD) and interstitial lung disease were mainly represented. Patients in these studies had moderate to severe lung disease.
The ERS guideline reviewed six interventions, which were intended as ‘add-ons’ to complement the usual standard of care for the respective lung conditions: multicomponent services, graded exercise therapy, hand-held fans, breathing techniques, opioid use and supplemental oxygen.
Question 1: Should a multicomponent service be used to reduce symptoms in people with serious respiratory illness?
Question 2: Should GET be used to reduce fatigue in people with serious respiratory illness?
Question 3: Should increased airflow be used to reduce breathlessness in people with serious respiratory illness?
Question 4: Should supplemental oxygen be used to reduce symptoms in people with serious respiratory illness?
Question 5: Should opioids be used to reduce symptoms in people with serious respiratory illness?
Question 6: Should breathing techniques be used to reduce symptoms in people with serious respiratory illness?
Evidence was reviewed using ‘grading of recommendation, assessment, development and evaluation’ (GRADE) – a systematic approach for rating the certainty of evidence.
Recommendations were made based on the available included evidence, as well as noting the strength and certainty of these and the fact that the relevance of interventions may vary over time along a patient’s disease trajectory.
A multicomponent model of care offers more than one intervention and includes at least one non-pharmacological intervention. Pulmonary rehabilitation (PR) was outside the scope of the review. Patients are enrolled in multicomponent services due to their symptoms, not diagnoses.
As a lower-risk and lower-cost intervention, the multicomponent model of care addresses a gap in healthcare, however, such services may not be widespread across all countries.
Graded exercise therapy (GET)is part of PR programmes, supervised over 12 weeks in an outpatient setting. It may include aerobic or water-based exercise and usually does not require specific equipment but usually needs staff supervision. It can also be delivered remotely.
Patients may have to wait a long time to access PR programmes or may be ineligible to be referred to PR if their lung disease is too advanced. They may also have a personal lack of confidence and reservations about participating in PR. The guideline serves as a lever to promote physical activity in patients and increase the availability and access of GET across countries.
A hand-held fan to ease the feeling of breathlessness is already established as part of COPD care in parallel to the use of usual inhaled treatments and breathing techniques.
Usually held six inches from the face, a fan generating cool air reduces the sensation and modulates the perception of, and patient response to, breathlessness.7 Use of a hand-held fan requires little patient training and is portable with virtually no associated adverse effects. However, there may be variations in the frequency, positioning and timing of patients’ use.
The guideline reaffirms its benefits and will hopefully promote greater uptake of this relatively inexpensive and accessible intervention.
People with lung disease may hyperventilate or have shallower or mouth breathing. Anxiety can also contribute to a dysfunctional breathing pattern, which can be further compounded by the side effects of beta-agonist medication use and overuse in lung disease.
Breathing techniques essentially slow breathing, enabling deeper breathing and increased mindfulness of respiration. This can be useful when a person undertakes an activity to help expel air and pace themselves. Breathing exercises also help manage panic attacks and are an established part of COPD care.
Opioids are commonly prescribed for pain but sometimes for a non-analgesic effect in lung disease. To look at this in more detail, the ERS task force reviewed studies which mainly included patients with COPD (and none that were at end of life) comparing opioids to placebo for the treatment of breathlessness or cough when used for >4 days.
Based on the evidence included in the review, the guideline recommends against opioid use for breathlessness or improving HRQoL, which is contrary to current practice in some countries.
The review found that regular use of opioids also had no significant effect on cough scores. Opioids have a range of adverse effects associated with their use; these may be heightened in people with advanced lung disease who may be more sensitive to their effects, for example, due to older age or if there is renal or liver dysfunction.
Before the guideline, patients with more advanced lung disease might have been prescribed low-dose morphine (such as 1.25–2.5 mg orally) for breathlessness, which is an unlicensed use of opioids; however, the evidence of benefit is variable.
This practice may be harder to change but publication of the guideline offers an opportunity to deprescribe and reduce unnecessary medication burden, or equally prevent the prescribing in the first place where it may not be beneficial.
This is also in line with national patient safety concerns related to opioid use in the UK. There may be a role for morphine in the palliation of refractory breathlessness in advanced or terminal illnesses.8
The ERS practice guidance reviewed supplemental oxygen use at rest or exertion. It points out that there is little reliable evidence that oxygen positively affects HRQoL or breathlessness scores in daily life. Hence, oxygen should not be routinely prescribed for breathlessness.
It has utility in an emergency setting or for longer-term use, such as in respiratory failure or palliative care, but also has risks relating to the oxygen itself but also equipment, which include local side effects, trip hazards and burns. Therefore, it should be prescribed as per national guidelines to ensure safe and clinically appropriate use.
Although the guidance suggests not using oxygen, there may be individuals who may derive small benefit and these patients should be reviewed on a case-by-case basis. For selected patients with severe breathlessness and exertional desaturation, and who will be able to use it safely, a trial of oxygen can be instituted. If so, the lowest concentration and flow rate to ease symptoms should be trialled.
Positive recommendation:
Conditional recommendations, very low certainty of evidence:
Negative recommendations:
The task force wanted to maintain rigour and a robust review process and acknowledge that the certainty of the available evidence for review was often low or very low. Certainty of evidence was impacted by various factors in the studies, including the risk of bias, imprecision and reporting bias.
Sometimes, a variety of scales for measuring a clinical difference were used, and sometimes multiple measures within a study were used at inconsistent times. Scores did not always correlate with an improved perception of symptoms or have a positive impact on other non-study-specified outcomes.
Acute improvements and reductions in breathlessness may be more clinically meaningful than a change over time.
HRQoL is impacted by various factors, including when it is measured and by breathlessness, which were, in some cases, measured separately and by the same and different scales, making results challenging to interpret. This reiterates that the studies reviewed had heterogeneity and that patients’ symptoms and the relevance of interventions change over time.
Additionally, the task force noted ‘the low certainty of evidence, modest impact of interventions on patient-centred outcomes, and absence of effective strategies to ameliorate cough highlight the need for new approaches to reduce symptoms and enhance wellbeing for individuals who live with serious respiratory illness.’5
The guideline highlights several research priorities and priorities for future work.
It is intended to complement the usual good practice management of respiratory diseases in line with national and international guidelines. The standard fundamentals of care should be applied, including, where relevant, optimising medicines, inhaler technique and adherence to therapy; ensuring uptake of vaccinations such as pneumococcal and influenza; and the optimal management of co-morbidities.
Smoking cessation is a key intervention at any stage, and patients should be supported to achieve this. In some countries, smoking status may impact eligibility for oxygen therapy.
Considerations of different payers and how healthcare is accessed in different countries are also important in influencing the uptake of care and interventions, recognising that this may widen health inequalities and poor health outcomes if people cannot afford to pay for treatments.
The guideline highlights the need for shared decision-making conversations with patients and a balance of benefit versus risk and adverse events on an individual basis alongside regular review and monitoring, including trialling and stopping interventions if they are not beneficial. This is important to manage patient expectations and personalise care and may be included in advanced care planning conversations.
Additionally, equity of access to interventions, such as oxygen, may be variable depending on the set-up of healthcare in different countries and an individual’s ability to pay for and access care, but, equally, the availability of services and treatments. It is also influenced by geographical location – be that coastal, rural or urban. The guidance hopefully levels this inequity by dissuading the use of oxygen, although it can be difficult to change historical practices.
There is also the opportunity and consideration to employ different models of care for multidisciplinary review and interventions going forward. This is useful in re-imagining pathways of service provision and when considering our respiratory workforce, including growing this for the future; capacity plans; and working in more integrated ways.
Studies of multicomponent services reviewed have been respiratory- or palliative-based and not in primary care. Therefore, the feasibility of implementation in expanded settings must be considered in line with local infrastructure and scope for expansion.
Many of the interventions reviewed are relatively inexpensive and can be implemented with little or no training. Pharmacological interventions, including oxygen, have their own associated potential adverse events and this guideline offers an evidence-based approach to support clinicians with patient discussions as to how and when these interventions may be utilised.
Ultimately, we need to provide holistic, patient-centred care, offering both pharmacological and non-pharmacological interventions which take account of individual needs, balancing risk and benefit to ease symptoms in advanced lung disease.
Ravijyot Saggu
Respiratory pharmacist, London, UK, and chair of the UK Clinical Pharmacy Association Respiratory Committee
1st July 2024
A world-first guideline looking at Sjögren disease across all ages has been published by the British Society for Rheumatology to raise awareness of the condition among a wide range of healthcare professionals and ensure timely treatment for patients. Dr Elizabeth Price, the consultant rheumatologist who led the development, discusses the context around the guideline, the unmet need it aims to tackle and the expected impact on patients’ quality of life.
Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with a significant impact on quality of life and no curative treatment. Although sicca of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common.
SD significantly impacts patients’ quality of life. A literature review found that health-related quality of life (HRQoL) was markedly reduced in SD in multiple studies across many countries compared with healthy controls.1
The reduction in HRQoL was similar to that observed in other chronic diseases, such as rheumatoid arthritis and systemic lupus erythematosus, suggesting that it is not a ‘benign’ disease. This reduction in quality of life has been noted in multiple domains and across all populations studied worldwide.
Anxiety, depression, pain and fatigue are all increased in SD compared with healthy controls and significantly impact quality of life.2 The loss of taste and smell that accompanies SD also has a negative effect on quality of life,3 as does the ocular dryness.4,5
There is a significant reduction in sexual quality of life6 due to the combined effects of vaginal dryness,7 atrophy8 and psychosocial factors such as coping strategies and illness perceptions.9
Systemic involvement, including nervous system manifestations such as peripheral neuropathy,10 respiratory system involvement11 and arthralgia,12 also negatively impacts quality of life.
A meta-analysis suggests an increase in cardiovascular13 and respiratory morbidity14 and a small excess mortality has been observed in people with SD,15 particularly in males and those with underlying lung disease.16
Systemic (extra-glandular) features affect at least 70% of patients and include inflammatory arthritis, skin involvement, haematological abnormalities, neuropathies, interstitial lung disease and B-cell lymphoma (5–10% lifetime risk).17,18
SD remains a chronic illness with no disease-modifying or curative treatments available to date. People can accumulate morbidity over time.
SD is an under-recognised condition with significant unmet needs. The underlying pathogenesis is the subject of ongoing research but, as with other autoimmune diseases, there are multiple factors involved, including genetic vulnerability, hormonal status and environmental insults to the immune system.
Research into pathogenetic mechanisms is ongoing, and studies investigating non-pharmacological treatments, novel biologic drugs and the repurposing of existing conventional and biologic immunosuppressive agents are underway.
The development of effective treatments has been hampered by the disease’s heterogeneity. We now understand that accurate stratification of patients into disease subgroups and collaborative studies are essential in providing large enough cohorts to demonstrate meaningful effects of interventions.
There is a need to develop better measures of disease activity as the currently used parameters do not include fatigue and dryness, underestimate the disease burden and are not sensitive to change.
The HQIP-mandated New Early Inflammatory Arthritis Audit covering England and Wales (NEIAA) has recently expanded to include new connective tissue disease diagnoses, including SD. Teams should be encouraged to record all new cases to build an accurate picture of the incidence of new presentations and current delays to diagnosis.
Individuals with SD currently experience long delays in diagnosis and there is poor awareness of the condition and its management among healthcare professionals who often underestimate the burden of SD.
Patients struggle with the chronic nature of the condition and are frustrated by the paucity of treatments and lack of knowledge in the medical field.
Successful management requires the personalisation of care. Although sicca of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations, including fatigue and arthralgia, are common.
Therefore, the key management aims should be to empower individuals to manage their condition; conserving, replacing and stimulating secretions; preventing damage; and suppressing underlying systemic disease activity.
A new guideline from the British Society for Rheumatology aims to provide comprehensive advice on the diagnosis and management of SD in adults and juvenile-onset SD for rheumatology teams in the UK19 and expands on the recommendations of the original 2017 guideline on the management of adults.20
We know that with appropriate care, quality of life and long-term outcomes improve, and we hope that these guidelines will provide a framework for healthcare professionals to diagnose and manage those with SD effectively and proactively.
The full guideline provides detailed evidence-based advice, which is broken down into 19 key areas. The recommendations are summarised in the executive summary and are accompanied by a one-page summary highlighting the key steps in managing and treating patients with SD effectively.
The British Society for Rheumatology guideline needs to be publicised and widely disseminated to ensure it reaches its target audience. This should include clinicians caring for individuals with SD and those not satisfying criteria but who present with sicca symptoms.
The audience will also include, but is not limited to, paediatric and adult rheumatologists, general practitioners, ophthalmologists, oral medicine specialists, dentists, opticians, optometrists, specialist nurses, allied healthcare professionals and patients themselves.
Raising awareness of SD and its management should promote earlier diagnosis and lead to better outcomes for these individuals.
Elizabeth Price MBBCh PhD
Department of Rheumatology, Great Western Hospital NHS Foundation Trust, Swindon, UK
1. Miyamoto ST, Valim V, Fisher BA. Health-related quality of life and costs in Sjogren’s syndrome. Rheumatology 2019;60:2588–601.
2. Omma A et al. Do the European League Against Rheumatism (EULAR) Sjogren’s syndrome outcome measures correlate with impaired quality of life, fatigue, anxiety and depression in primary Sjogren’s syndrome? AMS 2018;14(4):830–7.
3. Al-Ezzi MY et al. Primary Sjogren’s syndrome impact on smell, taste, sexuality and quality of life in female patients: A systematic review and meta-analysis. Mod Rheumatol 2017;27(4):623–9.
4. Zhang Y et al. Vision-related quality of life and psychological status in Chinese women with Sjogren’s syndrome dry eye: a case-control study. BMC Women’s Health 2016;16(1):75.
5. Mertzanis P et al. The relative burden of dry eye in patients’ lives: comparisons to a U.S. normative sample. Invest Ophthalmol Vis Sci 2005;46(1):46–50.
6. Priori R et al. Quality of Sexual Life in Women with Primary Sjogren Syndrome. The Journal of rheumatology. 2015;42(8):1427–31.
7. Maddali Bongi S et al. Gynaecological symptoms and sexual disability in women with primary Sjogren’s syndrome and sicca syndrome. Clin Exp Rheumatol 2013;31(5):683–90.
8. Capriello P et al. Sjogren’s syndrome: clinical, cytological, histological and colposcopic aspects in women. Clinical and experimental obstetrics & gynecology. 1988;15(1-2):9–12.
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23rd May 2024
Adopting a risk-stratified approach to breast cancer screening could help improve the benefit-to-harm ratio and the cost-effectiveness of screening programmes, European experts say.
In an invited review published in the journal European Radiology outlining the European Society of Breast Imaging’s general recommendations on breast cancer screening, the authors said that programmes usually included all women irrespective of their breast cancer risk, with age being the only determining factor.
Senior author Dr Ritse Mann, breast and interventional radiologist at the Radboud University Medical Center in Nijmegen, The Netherlands, and colleagues said breast cancer was the most frequently occurring cancer in Europe, accounting for about 15% of all new cancer cases and for about 30% of all new cancer cases in women.
Mammographic screening allowed for early cancer diagnosis, thereby reducing cancer mortality and the need for aggressive treatments.
‘Nevertheless, breast cancer remains the leading cause of cancer death among women worldwide, and although the benefits of mammographic screening outweigh the harms in the general population and the “one-size-fits-all” approach remains easier to implement, further improvements are sought,’ Dr Mann and colleagues wrote.
‘Moving to screening programmes adjusted to personal risk level instead of age-based population screening could improve the performance of a screening programme by reducing underdiagnosis, false positives, and over-diagnosis as well as improving cost-effectiveness.’
Several risk prediction models were available to estimate an individual’s risk of developing breast cancer, they said.
If possible, risk assessment should be performed at a young age (approximately 25 years) to effectively tailor screening recommendations.
Women found to be at a high risk of developing breast cancer should start screening as early as 25 years of age with annual breast MRI (evidence level 1), the recommendations stated, supplemented with annual or biennial mammography from age 35-40 years.
Evidence also supported the use of breast MRI screening in women with extremely dense breast tissue, preferably every two to three years.
‘If MRI is not available, supplemental ultrasound can be performed as an alternative, although the added value of supplemental ultrasound regarding cancer detection remains more limited,’ the experts wrote.
Breast density category should always be reported after mammography screening, they noted, as this had important implications for the performance of supplemental and alternative imaging methods.
Overall, regular mammography should be considered the mainstay of breast cancer screening (evidence level 1), but digital breast tomosynthesis could be performed as an alternative.
For women at intermediate risk of breast cancer, supplemental imaging modalities, including digital breast tomosynthesis, ultrasound, breast MRI, and, more recently, contrast-enhanced mammography, were available and had already shown potential to further increase diagnostic performance.
Artificial intelligence had also shown promising results in supporting risk categorisation and interval cancer reduction, Dr Mann and colleagues noted.
Women should be properly informed about the advantages as well as disadvantages of a breast cancer screening test to be able to make informed choices. Indeed, the experts said: ‘Individual screening recommendations should be made through a shared decision-making process between women and physicians.’
If there was a move to risk-based cancer screening rather than relying on age-based population screening, informed decision-making would become even more important to increase acceptability among those lower risk women who were candidates for reduced screening intensity, Dr Mann and colleagues suggested.
26th April 2024
An expanded guideline providing evidence-based recommendations for the management of adult and juvenile-onset Sjögren disease has been published by the British Society for Rheumatology (BSR).
Updated from the original 2017 version which focused on the systemic effects of the condition, the new guideline provides a framework for healthcare professionals to effectively and proactively manage individuals with Sjögren disease.
Its aim is to standardise care across the UK and enable non-specialist rheumatologists and allied healthcare professionals involved in the treatment of Sjögrens disease to manage patients holistically and in a personalised way.
The guideline working group established a set of 19 key questions around Sjögren disease diagnostics, comorbidities, clinically effective and timely treatments, considerations in pregnancy, non-pharmacological recommendations and tailored long-term follow-up.
They then interrogated the literature to determine the answers, which were then used to develop the recommendations.
For the first time, the guidelines include advice on Sjögren disease in adolescence and recurrent parotitis. They also note medication advancements such as access to ciclosporin for very severe eye dryness from four years of age and evidence around conventional and biologic disease-modifying antirheumatic drugs.
Dr Coziana Ciurtin, consultant in adult and adolescent rheumatology, said: ‘This is a first at an international level – there is no other guideline that looks at Sjögren disease across all ages. We recognise the need to incorporate recommendations for an under-diagnosed disease phenotype that starts in younger patients, who present slightly differently and may have different needs. We aim to support all clinicians and allied health professionals in diagnosing and looking after these younger patients.’
The guideline working group included members from rheumatology, including adult, adolescent and paediatric specialists, alongside a diverse working group consisting of GP, occupational therapy, ophthalmology, renal specialists and experts by experience.
As part of their work, the group took the decision to change the name of the condition from Sjögren syndrome to Sjögren disease. Dr Elizabeth Price, consultant rheumatologist at Great Western Hospital who led the BSR guideline development, said: ‘We had feedback from [European colleagues] and the patients that they preferred the name disease as it changed the emphasis of the condition and there is a move away from eponymous syndromes.’
She added: ‘I think in time we might lose the disease as well and it might just become Sjögren but we felt it was a little bit too early to do that.’
Alongside the full guideline, the working group have produced an easy-access summary sheet, which is free to download, as well as an audit tool that supports clinicians in making a correct diagnosis.
The BSR has recently extended the data capture for the New Early Inflammatory Arthritis Audit to include patients with newly diagnosed connective tissue disorders, including Sjögren’s.
Speaking at a roundtable on the guideline’s publication, Dr Price said: ‘A plea from me: if you can put all your newly diagnosed Sjögren’s patients into the audit, that will help us tremendously. Can I urge you to use the most modern criteria, which are referred to in the guideline as the ACR-EULAR combined 2016 criteria. If you look at the audit tool that goes along with the guideline, the first item in the audit tool is have you met the guidelines and there’s a little tick box for you to do that.’
1st March 2024
A new study challenges the ‘one size fits all’ approach to current aspirin guidelines for patients at risk of atherosclerotic cardiovascular disease (ASCVD).
The findings of three trials published in 2018 (ASPREE, ASCEND and ARRIVE), which assessed the effects of starting aspirin therapy versus a placebo on ASCVD outcomes in aspirin-naive adults, resulted in guidelines no longer recommending low-dose aspirin for the primary prevention of ASCVD.
The amended guidelines were motivated by findings showing an increased risk of significant bleeding for patients taking aspirin across the three trials; however, questions remain for patients who were already taking aspirin before the trials.
In a meta-analysis of a subset of the trial results, researchers from the University of Galway found that people who had been previously taking aspirin but had chosen to stop without consultation with their doctor faced a 28% increased risk of ASCVD.
The findings are published in the journal Circulation, and, in the absence of further data, the researchers suggest that patients already safely treated with low-dose aspirin for primary prevention of ASCVD can continue to do so.
The three original randomised trials involved over 47,140 patients from 10 countries, including the US, the UK and Australia.
In a previously published subgroup analysis, the ASPREE trial authors studied 1,714 participants taking aspirin at least twice weekly before enrolment.
In a randomised analysis, those who stopped taking aspirin and took a placebo had a trend to increased risk for ASCVD events compared with continuing aspirin (55 of 841 [6.5%] versus 44 of 873 [5.0%]). However, the findings have limitations since they were based on a small sample size of 99.
In the present investigation, trial-level meta-analyses were performed to study the subgroup of participants taking aspirin at baseline in both the ASPREE and ASCEND trials (n=7,222).
Data from the subset of patients revealed that there was a higher risk of both fatal or non-fatal ASCVD among baseline users who were randomised to stop aspirin and take a placebo versus those randomised to continue aspirin (450 of 3,609 [12.5%] versus 374 of 3,613 [10.4%]).
The hazard ratios in both subset meta-analyses (1.32 for the ASPREE data and 1.21 for the ASPREE and ASCEND combined analysis) suggest that although there is a potential trend towards increased risk for ASCVD events among those who stopped taking aspirin, the results are not statistically significant.
The Galway researchers found the difference in the risk of major bleeding was not statistically significant between baseline aspirin users randomised to discontinue aspirin and those who continued (123 out of 3609 [3.4%] versus 142 out of 3613 [3.9%]; hazard ratio, 0.86).
Professor John W. McEvoy from the University of Galway said the ‘results are hypothesis-generating’ and challenge the ‘one size fits all’ guidelines.
He said: ‘Our findings of the benefit of aspirin in reducing heart disease or stroke without an excess risk of bleeding in some patients could be due to the fact that adults already taking aspirin without prior bleeding problems are inherently lower risk for a future bleeding problem from the medication. Therefore, they seem to get more of the benefits of aspirin with less of the risks.’
Professor McEvoy explained that more research was needed but added: ‘Until further evidence becomes available, it seems reasonable that persons already safely treated with low-dose aspirin for primary prevention may continue to do so unless new risk factors for aspirin-related bleeding develop.’
A study published in March 2023 found that the benefits of aspirin use in myocardial infarction are offset by statin use in patients without ASCVD.
9th February 2024
A study of patients with hypertension has found that those who had variable blood pressure readings between clinic visits appeared to be at greater risk of heart attack and stroke than those with high blood pressure readings and low variability.
Published in the European Heart Journal, the ASCOT Legacy Study, led by a team from Imperial College London, analysed data from a 20-year study of more than 8,000 UK patients with hypertension.
They found that variation in systolic pressure over time was a strong predictor of stroke, heart attack and atrial fibrillation even in those who appeared to have well-controlled blood pressure. Highly variable blood pressure was also a strong predictor of risk at all levels of average blood pressure from low to high.
The researchers noted that based on current guidelines, clinical practice dictates that treatment decisions in patients with hypertension are determined by levels of systolic and diastolic blood pressure.
‘Our studies, however, provide robust evidence that visit-to-visit blood pressure variability is a far more powerful determinant of cardiovascular outcomes and that at least half of all cardiovascular events in our cohort occurred in those with controlled blood pressure but high blood pressure variability,’ they said.
As a result, the researchers are calling for medical guidelines to be changed to take into account interventions where a patient’s blood pressure varies between readings.
Professor Peter Sever is professor of clinical pharmacology and therapeutics at Imperial College London, honorary consultant physician at the Imperial Healthcare NHS Trust and co-director of the International Centre for Circulatory Health, as well as senior author of the study.
He said: ‘We’ve long known that high blood pressure increases the risk of heart attacks and stroke, but our latest findings highlight the importance of tracking variability in blood pressure over time as well.
‘While a degree of this was known to doctors, without clinical trials it has been difficult to quantify the risk of blood pressure variability over the long term, or the impact of interventions such as calcium blockers to reduce patients’ risk.‘
The ASCOT Legacy Study followed up 8,580 participants from the original ASCOT study, 50% of whom were taking amlodipine to manage their hypertension.
The researchers split the group into thirds, based on their average systolic blood pressure and their blood pressure variability.
They found that among patients with an average systolic blood pressure of less than 140 mmHg, the group with the highest variability was at a 16% greater risk for heart attacks, strokes and other cardiovascular events compared with the group with the lowest variability.
Some 53% of all cardiovascular events occurred in patients whose blood pressure was well-controlled with medication and who would not have been considered for additional treatment under the guidelines in place at the time of the trial.
The findings indicated that a systolic blood pressure variability of 13 mmHg or more over the course of five years may be associated with a significantly increased risk of cardiovascular events.
The Imperial team also confirmed that the drug amlodipine proved effective in lowering blood pressure variability during trials and could help to reduce risk.
Commenting on the next steps for the trial, Professor Sever said: ‘We urgently need to explore new practical ways to assess blood pressure variability and are currently studying the possibilities of incorporating data from home blood pressure monitoring into clinical decision-making.
‘The low cost and wide availability of digital blood pressure monitors and health apps means people can readily track their blood pressure over time and this could provide invaluable data for doctors to make the best treatment decisions – though we’d urge patients not to be overly concerned, as we would expect a degree of variation in their readings over time.
‘Crucially, we need international guidelines for clinicians to be updated to reflect these latest findings and to include blood pressure variability as a major risk factor for heart attack and stroke.‘
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