- N Engl J Med 2022; 386:2178-2187
In a placebo-controlled trial, treatment with the preferential phosphodiesterase 4B inhibitor BI 1015550 prevented a decrease in lung function over a 12-week period in patients with idiopathic pulmonary fibrosis.
- N Engl J Med 2022; 386:2235-2236
Fibrosis, defined as the pathologic accumulation of extracellular matrix, is the final outcome of several common chronic inflammatory, immune-mediated, and metabolic diseases and accounts for up to 45% of all deaths in the industrialized world.1 The approval of pirfenidone and nintedanib for the treatment of idiopathic pulmonary fibrosis...
- N Engl J Med 2022; 386:1187-1188
Little is known regarding appropriate patient selection for and clinical outcomes with lung transplantation for respiratory failure due to Covid-19. This study analyzes lung transplantations reported in the United Network for Organ Sharing registry from August 2020 through September 2021.
- N Engl J Med 2022; 386:1058-1069
COP (formerly bronchiolitis obliterans organizing pneumonia) causes respiratory symptoms, with pulmonary infiltrates that usually respond well to therapy, but it is often misdiagnosed. This review discusses the pathobiology, diagnosis, and treatment of COP.
- N Engl J Med 2021; 385:1018-1032
Sarcoidosis is a lung disease of unknown cause characterized by noncaseating granulomas; the heart and nervous system may also be involved. It is often misdiagnosed; tissue biopsy is needed for a definitive diagnosis. Glucocorticoids are recommended as initial treatment, when needed, but not for long-term care.
- N Engl J Med 2020; 383:2127-2137
The causal role of neutrophil serine proteases in the pathogenesis of bronchiectasis was studied with brensocatib, an inhibitor of protease activation. Brensocatib therapy resulted in a longer time to the first bronchiectasis exacerbation than placebo.
- N Engl J Med 2020; 383:958-968
This review covers fibrotic pulmonary diseases. Although idiopathic pulmonary fibrosis is very common, the review focuses mainly on fibrotic diseases other than IPF. The biology, clinical presentation, and treatment of the more common disorders are discussed.
- N Engl J Med 2020; 382:1443-1455
AAT is a protease inhibitor targeting neutrophil elastase. It prevents the destruction of tissue, particularly in the lung, from elastase activity. AAT deficiency is a genetic disorder characterized by pulmonary disease, especially emphysema and bronchiectasis, and hepatic disease.
- N Engl J Med 2020; 382:1478-1480
In Ireland, augmentation treatment of patients with genetic alpha1-antitrypsin deficiency was abruptly discontinued when government–industry price negotiations failed. After treatment was stopped, the incidence of hospitalization for COPD and mortality exceeded those observed while treatment was ongoing.
- N Engl J Med 2020; 382:1068-1070
Nine patients who were scheduled to undergo lung biopsy for pulmonary fibrosis were given epigallocatechin gallate (EGCG) for 2 weeks. Levels of fibrotic markers in lung-biopsy samples were lower in the EGCG-treated patients than in 10 similar, but untreated, patients.
- N Engl J Med 2019; 381:1718-1727
In patients with a progressive interstitial lung disease, 62% of whom had a CT pattern of usual interstitial pneumonia, those who received nintedanib had a lower annual rate of decline in the forced vital capacity than those who received placebo at 52 weeks.
- N Engl J Med 2019; 381:1775-1777
In the era before antifibrotic therapy, idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic interstitial pneumonias, had a survival rate of 20 to 30% at 5 years,1 a poorer outcome than that of many common forms of cancer. Patients with IPF still have a progressive course...
- N Engl J Med 2019; 381:291-293
The autoimmune disease amyopathic dermatomyositis–associated interstitial lung disease, which is related to anti–melanoma differentiation–associated protein 5 antibodies, is often rapidly progressive. Patients who received glucocorticoids and tofacitinib had improved survival.
- N Engl J Med 2019; 380:2518-2528
Patients with interstitial lung disease associated with systemic sclerosis were treated with usual care plus placebo or nintedanib. The annual rate of change in forced vital capacity assessed over a 52-week period was −52.4 ml per year with nintedanib and −93.3 ml per year with placebo. There were no differences...
- N Engl J Med 2019; 380:1268-1277
A 69-year-old man was evaluated at this hospital because of progressively worsening dyspnea. Results of chest imaging were consistent with fibrotic lung disease, and tests of pulmonary function revealed a restrictive ventilatory deficit with diffusion impairment. A diagnosis was made.
- N Engl J Med 2019; 380:94-96
Pathogenic findings in a mouse model of idiopathic pulmonary fibrosis confirm the pivotal role played by dysfunction of the type 2 alveolar (AT2) epithelial cells in causing the disease. The findings support the use of this model in future investigations.
- N Engl J Med 2018; 379:2209-2219
In this study, a gain-of-function variant in the promoter of MUC5B that was previously found to be associated with idiopathic pulmonary fibrosis was associated with rheumatoid arthritis–associated interstitial lung disease.
- N Engl J Med 2018; 379:2265-2266
Clinical heterogeneity is a hallmark of many autoimmune disorders, and clinical or subclinical pulmonary involvement is a common extraarticular feature of the rheumatoid arthritis (RA) phenotype. High-resolution computed tomography reveals evidence of pulmonary abnormalities in more than half of patients with RA, and clinically significant interstitial lung disease (ILD) will...
- N Engl J Med 2018; 379:1889-1891
Despite an increasing emphasis on patient-centered outcomes in medicine, transplant centers do not systematically report information on health-related quality of life, nor are such measures incorporated into algorithms for organ allocation or program assessment.
- N Engl J Med 2018; 379:1722-1731
In a trial, patients with moderate to severely advanced idiopathic pulmonary fibrosis were treated with nintedanib plus sildenafil or nintedanib alone, with the goal of decreasing IPF symptoms. There were no between-group differences in any of three symptom measures.