Clinics in chest medicine
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Clinics in chest medicine · Sep 2004
ReviewInterstitial lung disease in the patient who has connective tissue disease.
Interstitial lung disease is a common complication of many of the connective tissue diseases. Because the prognosis, degree of reversibility, and optimal therapy differs for each disease presentation, a thorough knowledge of the pulmonary presentations of each connective tissue disease is important. Additionally, the challenge of finding the patient who has occult connective tissue disease in an interstitial lung disease clinic is discussed.
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Pulmonary Langerhans' cell histiocytosis (PLCH) is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers. PLCH belongs to the spectrum of Langerhans' cell histiocytosis (LCH), diseases characterized by uncontrolled proliferation and infiltration of various organs by Langerhans' cells. ⋯ Although LCH is approximately three times more common in children than adults, pulmonary involvement is much more common in adults with LCH, in whom it frequently occurs as the sole organ involved with disease. This article summarizes recent advances and current understanding of PLCH.
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Occupational interstitial lung diseases are a diverse group of disorders of varied cause. Occupational causes account for a significant portion of all interstitial lung diseases, and new causes continue to be described. ⋯ This article reviews the spectrum of diseases caused by exposure to metal dust and fumes, inorganic fibers, and nonfibrous inorganic dust. It also details an approach to the diagnosis, evaluation, and management of this group of illnesses.
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This article focuses on recent advances in the identification of genes and genetic polymorphisms that have been implicated in the development of human interstitial lung diseases. It focuses on the inherited mendelian diseases in which pulmonary fibrosis is part of the clinical phenotype and the genetics of familial idiopathic pulmonary fibrosis and other rare inherited interstitial lung diseases. The article also reviews the association studies that have been published to date regarding the genetics of sporadic idiopathic pulmonary fibrosis. The reader is directed to recent reviews on human genetic predisposition of sarcoidosis, environmental-related, drug-related, connective tissue related pulmonary fibrosis, and genetic predisposition of fibrosis in animal models.
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Pulmonary sarcoidosis is one of the most common causes of idiopathic interstitial lung disease. Clinical presentation can range from asymptomatic to respiratory failure. ⋯ Treatment options include corticosteroids. In the past few years, alternatives to corticosteroids have been developed,especially for patients with a chronic condition.