Idiopathic pulmonary fibrosis (IPF) is the most prevalent type of progressive interstitial lung disease, characterised by irreversible fibrosis and a poor long-term prognosis. Dr Ahmed Fahim discusses his work on the UK IPF Registry, which has collected real-world data on patients diagnosed with IPF, and how these findings will enhance understanding and promote advances in the clinical management of this challenging condition.
The British Thoracic Society UK Idiopathic Pulmonary Fibrosis (IPF) Registry is a national, multicentre observational database collecting prospective and retrospective data from patients treated in secondary and tertiary care settings. It was created in 2013 to gather information on clinical features, treatment options and patient outcomes and was expanded in 2023 to include any interstitial lung disease (ILD) with evidence of fibrosis.
It is the largest registry of its kind in a single country, providing a decade’s worth of data on trends in diagnosis and management of IPF in the UK covering the period from 2013 to 2023. Over 5,000 cases were included, with the majority being males (78%) and an average age of 74 years. Two-thirds were ex-smokers, and more than three-quarters had at least one comorbidity. The most common comorbidities were hypertension and ischaemic heart disease, followed by diabetes and hiatus hernia.
The diagnosis of IPF was confirmed after a multidisciplinary team (MDT) discussion among specialists in 93% of cases. We observed a trend toward a decrease in the proportion of patients requiring lung biopsies over the 10-year period, highlighting the crucial role of high-resolution computed tomography (HRCT) scans in the diagnostic algorithm. Additionally, the use of antifibrotic therapies increased from 36% in 2013 to 56% in 2023, reflecting changes in the guidelines for IPF management in the UK.
Our analyses urge the need for rapid expansion of the workforce involved in managing IPF and consider increasing the uptake of clinical trial recruitment in this disease where the quest for a cure is ongoing.
Influencing clinical decision-making in IPF
The findings emphasise the importance of diagnosing IPF following MDT discussions and highlight the necessity of referring these patients to specialist ILD centres at the earliest opportunity, enabling the prompt initiation of antifibrotic therapy.
Furthermore, the findings underscore the need to consider the diagnosis earlier, as more than a third of patients experienced symptoms for over two years before receiving a diagnosis and being referred for specialist care.
Therefore, it is crucial to enhance awareness and disease recognition for IPF at the primary care level and within the public domain, encouraging individuals to seek medical advice promptly to prevent significant delays in diagnosis and appropriate treatment.
Many patients are diagnosed with airway disease or heart failure when the underlying condition is IPF, which leads to delays in referring patients to specialist centres in a timely manner.
We must invest in raising awareness of IPF in primary care and promote face-to-face reviews of patients with symptoms of cough and breathlessness. If they exhibit typical ‘Velcro crackles’ during lung auscultation, it should prompt an HRCT scan to diagnose fibrotic lung disease as early as possible.
Close collaboration between primary care physicians and specialist centres could offer immense benefits in reducing diagnostic delays and developing pathways for urgent outpatient reviews of suspected IPF patients, similar to the fast-tracking of cancer patients, as the prognosis for IPF is akin to that of cancer.
Current best practices in diagnosing and managing IPF
The advancements in HRCT have led to more accurate diagnoses of IPF, resulting in less invasive procedures and a decline in the proportion of lung biopsies over time. We have observed that most IPF patients are discussed in MDT meetings, and the expertise of the MDT is crucial in determining whether the radiological pattern of definite and probable usual interstitial pneumonia (UIP) supports the diagnosis of IPF, thereby eliminating the need for surgical lung biopsy.
International guidelines for IPF strongly advise against surgical biopsy when a definite UIP pattern is present. Histological confirmation is not required for cases with a probable UIP pattern. Some patients with an indeterminate pattern may benefit from a bronchoalveolar lavage differential cell count. The gold standard for diagnosing IPF involves an ILD MDT discussion, which follows a thorough clinical assessment and is supported by laboratory and physiological data.
The management of associated comorbidities is crucial, as treatment with antifibrotics requires careful consideration to balance the adverse effect profile and drug interactions in the presence of comorbidities. It is also essential to evaluate the impact of comorbid conditions on IPF, such as gastro-oesophageal reflux, which can contribute to refractory cough in this patient cohort. Furthermore, managing pulmonary hypertension and cardiac failure is significant for the outcomes and quality of life of these patients.
Close collaboration
MDTs are essential for diagnosing IPF and recommending appropriate management plans for patients with life-limiting diseases. All members of the MDT make significant contributions to this management, including pulmonologists, radiologists, pathologists (when a lung biopsy is performed), rheumatologists, specialist ILD nurses, physiotherapists and pharmacists.
In advanced cases, close collaboration with the lung transplant team and the palliative care team is crucial to ensuring high-quality care for these patients. Unfortunately, the number of patients suitable for lung transplantation is limited due to advanced age and associated comorbidities.
Therefore, it is essential to seek an opinion from a lung transplant centre at the earliest opportunity when dealing with moderate to severe disease. ILD centres should also establish close connections with the nearest transplant centre and palliative care teams. This approach will promote holistic care in IPF, where treatment options are limited, and the disease is progressive.
Ensuring adequate resources and training
All healthcare professionals involved in the assessment and management of IPF patients – whether in outpatient settings or acute medical environments – should receive updates on the latest management guidelines for IPF. These guidelines should include indications for antifibrotic therapies, management of comorbidities, drug interactions, effective symptom control, especially for intractable cough and dyspnoea, and palliative care.
Investment in educational resources should occur at both local and regional levels, along with workforce expansion to increase specialist nursing teams for ILD. Furthermore, healthcare professionals who provide support – including physiotherapists, pharmacists and psychological services – should be included to enhance the quality of care.
Regional specialist ILD centres can significantly upskill referral centres, ensuring many patients receive care locally without travelling long distances. Additionally, patient support groups should be established, similar to those in England at each regional location served by specialist ILD centres.
The pivotal roles of registries and research
Registries play a crucial role in understanding disease behaviour and management changes over time in real-world settings. Therefore, we must utilise the data captured in these registries to inform future research, as it can help answer questions both prospectively and retrospectively.
Research into developing new therapies that could potentially halt fibrosis and promote disease stability is much needed, as we currently lack pharmacological treatments that can stop or reverse scarring or fibrosis.
We are likely to witness personalised therapies based on genetic profiles and multimodality treatments targeting multiple molecular pathways to enhance clinical outcomes and survival in this devastating disease.
It is essential to consider adaptive platform trials in IPF to evaluate the efficacy of various pharmacological and non-pharmacological interventions simultaneously and promptly, ensuring that the treatment landscape evolves rapidly in the near future.
Moreover, managing comorbid conditions such as pulmonary hypertension and cardiovascular diseases – and understanding their impact on clinical outcomes and survival – will be crucial for future research.
Author
Ahmed Fahim MD FRCP (UK) MRCP (Resp Med)
Consultant respiratory physician, New Cross Hospital, The Royal Wolverhampton NHS Trust, Wolverhampton, UK
