New article on the importance of early diagnosis and treatment in idiopathic pulmonary fibrosis

A new call to improve IPF diagnosis has been launched by some Spanish experts in May 2018.

With an article that recalls the challenges of an early and correct diagnosis of this rare condition, the authors show the benefits of a rapid provision of treatment on the prognosis and quality of life for IPF patients. The authors conclude with recommendations to improve the current state of play: enhanced care management, better coordination among stakeholders, and increasing funding for IPF research and management.

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Challenges in the diagnosis of IPF

IPF shares a challenge with many rare diseases, as its symptoms are often confused with other general respiratory conditions, and this can lead to a significant amount of patients being initially misdiagnosed with asthma, chronic obstructive pulmonary disease (COPD), or pneumonia, among other diseases. In this regard, detection of velcro-like crackles during chest auscultation, which are strongly associated with the presence of lung fibrosis, has been proposed as a feasible and sensitive measure to improve early detection. However, most patients who refer the first symptoms at the primary care physician are symptomatically treated, and only when no improvement is warned, are referred to the respiratory physician.

Diagnosis of IPF requires the presence of a pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT), or combinations of UIP patterns on HRCT and on tissue samples from lung biopsies. Some hope to improve early diagnosis is also given by several clinical trials that are trying to identify molecular markers that are associated with the manifestation of IPF.

Benefits of early diagnosis and treatment of IPF

An early intervention is crucial to improve IPF prognosis. The ATS/ERS/JRS/ALAT guideline for the treatment of IPF includes recommendations for the use of nintedanib, pirfenidone, and antacid therapy, as well as of non-pharmacological treatments such as pulmonary rehabilitation, long-term oxygen therapy, and lung transplantation. Importantly, real world data from a multi-center, prospective, observational registry support the beneficial effect of anti-fibrotic therapy on survival (Hazard Ratio (HR) 0.56, 95%CI 0.34–0.92, p = 0.022) and transplant-free survival independent of baseline disease severity, compared to non-anti-fibrotic therapy.

Need for improved management, better coordination among stakeholders, and increasing funding

A rapid and accurate diagnosis of IPF requires a multidisciplinary discussion among physicians specialized in interstitial lung diseases (ILDs); however,  multidisciplinary teams (MDTs) are only available in tertiary care reference centers. Thus, prompt referral from primary care or non-specialist pulmonology services to centers of expertise is essential for early diagnosis. Lack of quick referral or coordination among centers hinders rapid and accurate treatment and is detrimental to the patient. For instance, an accurate early diagnosis could prevent the misdiagnosis with COPD and treatment with corticosteroids, discouraged in IPF patients.

Despite the widely accepted benefits of referral to a reference center, some respiratory physicians still do not seek support from MDTs for early diagnosis and treatment. In addition, a stronger information flow and support among physicians and patients would be desirable, to manage expectations and avoid misinformation.  An integral multidisciplinary approach in IPF patient will eventually help improve quality of life and treatment adherence.

All these management measures translate into more funding requirements. Since IPF is a rare disease, National Health Systems (NHS) should play a main role in the funding of disease management strategies. Without Health Authorities participation, improvement of management is compromised.