Clinical course of mild-to-moderate idiopathic pulmonary fibrosis during therapy with pirfenidone: Results of the non-interventional study AERplus.

INTRODUCTION: Pirfenidone was the first anti-fibrotic drug approved in Europe in 2011 for the treatment of mild-to-moderate idiopathic pulmonary fibrosis. OBJECTIVES: To investigate the clinical course of mild-to-moderate idiopathic pulmonary fibrosis in pirfenidone-treated patients in a real-world setting. METHODS: The non-interventional study was conducted at 18 sites in Germany from 6/2014-12/2016. Adult patients with mild-to-moderate idiopathic pulmonary fibrosis were treated with pirfenidone (escalated from 3×1 to 3×3 capsules of 267 mg/day within 3 weeks) for 12 months. The observation period comprised 4 follow-up visits at months 3, 6, 9 and 12. Disease progression was defined as decrease of ≥10% in vital capacity or ≥15% in diffusing capacity of the lung for carbon monoxide (DLCO) and/or ≥50m in 6-minute walking distance vs. baseline, or "lack of response/progression" as reason for therapy discontinuation. RESULTS: A total of 51 patients (80.4% male, mean age 70.6 years) were included in the full analysis set. Disease progression at any visit was reported for 23 (67.6%) of 34 patients with available data. Over the course of the study, lung function parameters, physical resilience, impact of cough severity on quality of life, and the mean Gender, Age and Physiology Index (stage II) remained stable. In total, 29 patients (56.9%) experienced at least one adverse drug reaction (11 patients discontinued due to adverse drug reactions); serious adverse reactions were reported in 12 patients (23.5%). CONCLUSIONS: The results of this study are in line with the established benefit-risk profile of pirfenidone. Therefore, pirfenidone can be considered a valuable treatment option to slow disease progression in mild-to-moderate idiopathic pulmonary fibrosis. NCT02622477.

[Lung involvement in patients with primary biliary cirrhosis]. / Lungenbeteiligung bei primär biliärer Zirrhose.

HISTORY AND CLINICAL FINDINGS: For several months a 29 years old woman suffered from dry cough, dyspnea, and weakness. The clinical examination was without any abnormal findings. INVESTIGATIONS: Liver function tests and erythrocyte sedimentation rate were raised. High resolution CT chest scan showed multiple patchy alveolar and interstitial infiltrates. Transbronchial and surgical lung biopsy confirmed a moderate alveolitis with granulomas. DIAGNOSIS: primary biliary cirrhosis (pbc) complicated by lung disease could be diagnosed through increased titer of antimitochondrial antibodies and the laparoscopy. TREATMENT AND COURSE: Under treatment of pbc with either corticosteroids or Ursodeoxycholic acid, liver enzymes decreased and pulmonary symptoms disappeared. CONCLUSION: To detect lung involvement in patients with pbc early, lung function tests and diffusion capacity should be monitored regularly. An otherwise unexpected rise in liver function tests, particularly in patients with underlying interstitial lung disease or sarcoid granulomatosis should promptly be investigated further.