Idiopathic pulmonary fibrosis in the United States: time to diagnosis and treatment.

OBJECTIVE: Create a timeline of diagnosis and treatment for IPF in the US. DESIGN, SETTING, AND PARTICIPANTS: A retrospective analysis was performed in collaboration with the OptumLabs Data Warehouse using an administrative claims database of Medicare Fee for Service beneficiaries. Adults 50 and over with IPF were included (2014 to 2019). EXPOSURE: To focus on IPF, the following diagnoses were excluded: post-inflammatory fibrosis, hypersensitivity pneumonitis, rheumatoid arthritis, sarcoidosis, scleroderma, and connective tissue disease. MAIN OUTCOMES AND MEASURES: Data were collected from periods prior, during, and following initial clinical diagnosis of IPF. This included prior respiratory diagnoses, number of respiratory-related hospitalizations, anti-fibrotic and oxygen use, and survival. RESULTS: A total of 44,891 with IPF were identified. The most common diagnoses prior to diagnosis of IPF were upper respiratory infections (47%), acute bronchitis (13%), other respiratory disease (10%), chronic obstructive pulmonary disease and bronchiectasis (7%), and pneumonia (6%). The average time to a diagnosis of IPF was 2.7 years after initial respiratory diagnosis. Half of patients had two or more respiratory-related hospitalizations prior to IPF diagnosis. Also, 37% of patients were prescribed oxygen prior to diagnosis of IPF. These observations suggest delayed diagnosis. We also observed only 10.4% were treated with anti-fibrotics. Overall survival declined each year after diagnosis with median survival of 2.80 years. CONCLUSIONS AND RELEVANCE: Our retrospective cohort demonstrates that IPF is often diagnosed late, usually preceded by other respiratory diagnoses and hospitalizations. Use of available therapies is low and outcomes remain poor.

Assessment and Management of Occupational Hypersensitivity Pneumonitis.

The diagnosis and treatment of occupational hypersensitivity pneumonitis (OHP) remain complex and challenging in the absence of diagnostic gold standards or clinical guidelines. This review provides an update of the recent literature regarding the different presentations of OHP and the diagnostic yield and value of the diagnostic tests currently available, which include occupational and medical history, laboratory tests (serum-specific immunoglobulins, environmental sampling), imaging, bronchoalveolar lavage, transbronchial biopsy, transbronchial cryobiopsy, surgical lung biopsy, and specific inhalation challenges. These tools provide a precise differential diagnosis within the framework of interstitial lung diseases. However, among the chronic fibrotic forms, distinguishing OHP from sarcoidosis, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis remains a diagnostic challenge. Avoidance of exposure is pivotal for OHP management, whereas corticosteroids are fundamental in the pharmacological approach to this disease. In addition, studies describing the long-term benefits of immunosuppressive and antifibrotic agents have increased the use of these treatments in OHP.