Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrosing interstitial lung disease that occurs predominantly in the older population. There is increasing incidence and prevalence in IPF globally. The emergence of anti-fibrotic therapies in the last decade have improved patient survival though a cure is yet to be developed. In this review article, we aim to summarize the existing and novel pharmacotherapies for the treatment of IPF (excluding treatments for acute exacerbations), focusing on the current knowledge on the pathophysiology of the disease, mechanism of action of the drugs, and clinical trials.
Idiopathic inflammatory myopathies (IIM) are rare disorders characterised by the presence of skeletal muscle inflammation, with interstitial lung disease (ILD) being the most frequent pulmonary manifestation. The spectrum of clinical presentations of myositis related ILD (M-ILD) encompasses a chronic process to a rapidly progressive ILD (RP-ILD); which is associated with a high mortality rate. The most effective treatments remain controversial and poses a unique challenge to both rheumatologists and respiratory physicians to manage. Given the rare heterogenous nature of M-ILD, there is a paucity of data to guide treatment. The cornerstone of existing treatments encompasses combinations of immunosuppressive therapies, as well as non-pharmacological therapies. In this review, we aim to summarize the current pharmacological therapies (including its dosing regimens and side effects profiles) and non-pharmacological therapies. Based on the existing literature to date, we propose a treatment algorithm for both chronic M-ILD and RP-ILD.
We read with interest the recent article from Bafadhel et al1 published in CHEST (August 2016) and the more recent article from Couillard et al2 (published in CHEST October 2016) examining the possible role of eosinophils as markers of disease severity and clinical outcome in exacerbations of COPD. The report by Bafadhel et al1 suggests that patients presenting to a hospital with an eosinophilic exacerbation of COPD had a shorter length of stay. However, in this study, the authors also reported that readmission rates at 1 year were similar in eosinophilic and noneosinophilic exacerbations. The subsequent study by Couillard et al2 suggests that eosinophilia was associated with an increased risk of 12-month COPD related readmission.