From the Guidelines
Interstitial Lung Diseases with Upper Lobe Distribution
Interstitial lung diseases (ILDs) characterized by an upper lobe distribution are primarily Pulmonary Langerhans Cell Histiocytosis (PLCH) and Pleuroparenchymal Fibroelastosis (PPFE), which exhibit a predilection for involvement of the upper lobes 1.
Characteristics and Treatment
- Pulmonary Langerhans Cell Histiocytosis (PLCH) is associated with tobacco smoking and can lead to cough, dyspnea, and spontaneous pneumothorax. It is diagnosed based on clinical features, radiographic findings, and sometimes BAL or lung biopsy 1.
- Pleuroparenchymal Fibroelastosis (PPFE) presents with fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes, and can lead to pneumothorax and progressive disease 1.
- Treatment for these conditions may involve the use of corticosteroids, such as prednisone, at a dose of 20-40 mg per day for a duration of 3-6 months, with potential addition of immunosuppressive agents like azathioprine at 50-100 mg per day or methotrexate at 10-20 mg per week 1.
- In cases of progressive disease, consideration may be given to the use of anti-fibrotic agents, such as pirfenidone at 801 mg three times daily or nintedanib at 150 mg twice daily 1.
Key Points
- Upper lobe distribution is characteristic of PLCH and PPFE.
- Diagnosis is based on clinical, radiographic, and sometimes histopathological findings.
- Treatment involves corticosteroids, immunosuppressive agents, and potentially anti-fibrotic agents for progressive disease.
- Cessation of tobacco smoking is crucial in PLCH management 1.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Interstitial Lung Diseases with Upper Lobe Distribution
- The provided studies do not specifically mention interstitial lung diseases characterized by an upper lobe distribution 2, 3, 4, 5, 6.
- However, some interstitial lung diseases, such as idiopathic pulmonary fibrosis, are known to have a lower lobe predominance, while others, like sarcoidosis and histiocytosis, can have an upper lobe distribution.
- The studies focus on the diagnosis, management, and treatment of interstitial lung diseases, including fibrosing ILDs, progressive pulmonary fibrosis, and ILDs associated with connective tissue diseases 2, 3, 4, 5.
- There is no direct evidence in the provided studies to answer the question about interstitial lung diseases characterized by an upper lobe distribution.
Relevant Information
- Interstitial lung diseases are a heterogeneous group of disorders that require individualized treatment 5.
- The diagnosis and management of ILDs involve a thorough medical history, clinical examination, serologies, high-resolution computed tomography (CT) scan, and, in some cases, bronchoalveolar lavage or surgical lung biopsy 3.
- Antifibrotic agents, such as nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis and other chronic fibrosing ILDs with a progressive phenotype 2, 3, 4, 5.