Luca Richeldi

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort. The “European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease” was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity. The task force proposes the term “interstitial pneumonia with autoimmune features” (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features. A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort. ERS/ATS task force provides nomenclature and classification criteria for patients with IIP and autoimmune features http://ow.ly/O7qao

Chapter 4 – Management of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive and inevitably fatal lung disease. Although the etiology and pathogenesis of IPF remain incompletely understood, two drugs (e.g., pirfenidone and nintedanib) have proven effective in slowing down functional decline and disease progression and are now approved worldwide for treatment. Yet, as outlined by the recent guideline document on treatment of IPF, every therapeutic decision needs to be tailored to the individual patient, after discussing potential benefits and pitfalls. Comorbidities, which almost invariably complicate IPF, impact significantly clinical course and prognosis of the disease, making a holistic approach to the best care for these patients. Randomized-controlled trials remain a valid choice for selected IPF patients and their completion is critically important to achieving the ultimate goal of improving both survival and quality of life of patients suffering from this devastating disease.

Effect of baseline FVC on decline in lung function with nintedanib: results from the INPULSIS™ trials

Background: Nintedanib, an intracellular inhibitor of tyrosine kinases, is in development for the treatment of idiopathic pulmonary fibrosis (IPF). The INPULSIS™ trials were two replicate randomized, double-blind, placebo-controlled, 52-week Phase III trials that assessed the efficacy and safety of nintedanib 150 mg twice daily in patients with IPF. The primary endpoint was the annual rate of decline in forced vital capacity (FVC), which was significantly reduced in the nintedanib group compared with placebo in both trials. Aim: To assess the impact of baseline FVC on the effect of nintedanib on rate of decline in FVC. Methods: A pre-specified subgroup analysis of patients with baseline FVC >70% versus ≤70% of predicted value was undertaken using pooled data from both trials. Results: 700 patients (nintedanib 431, placebo 269) had baseline FVC >70% predicted and 361 patients (nintedanib 207, placebo 154) had baseline FVC ≤70% predicted. For patients with a baseline FVC >70% predicted, mean age was 67.4 years, 76.9% were male, 55.7% were White and mean carbon monoxide diffusion capacity (DL CO ) was 4.0 mmol/min/kPa. For patients with a baseline FVC ≤70% predicted, mean age was 65.5 years, 83.9% were male, 60.4% were White and mean DL CO was 3.6 mmol/min/kPa. There was no significant treatment by subgroup interaction: the difference in adjusted annual rate of decline in FVC between the nintedanib and placebo groups was comparable in both subgroups. Conclusion: A subgroup analysis of pooled data from the INPULSIS™ trials showed that nintedanib 150 mg twice daily slowed the decline in lung function in patients with IPF, independent of severity of lung function impairment at baseline.