Seminars in respiratory and critical care medicine
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Obliterative bronchiolitis (OB) (formerly termed bronchiolitis obliterans), is a rare fibrotic disorder involving terminal and respiratory bronchioles. The term constrictive bronchiolitis is synonymous with OB. Clinically, OB is characterized by progressive (often fatal) airflow obstruction, the absence of parenchymal infiltrates on chest radiographs, a mosaic pattern of perfusion on high-resolution computed tomographic scan, poor responsiveness to therapy, and high mortality rates. ⋯ Other causes of OB include connective tissue disease (CTD) (particularly rheumatoid arthritis); lower respiratory tract infections; inhalation injury; exposure or inhalation of toxic fumes, metals, dusts, particulate matter, or pollutants; occupational exposures; drug reactions; consumption of uncooked leaves of Sauropus androgynus; chronic hypersensitivity pneumonia; diffuse neuroendocrine cell hyperplasia; miscellaneous. When no cause is identified, the term cryptogenic obliterative bronchiolitis is used. This review discusses the salient clinical, radiographic, and histological features of OB and presents a management approach.
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Semin Respir Crit Care Med · Oct 2012
Case ReportsNeurofibromatosis-associated diffuse lung disease: case report.
Since the initial report in 1963 several small case series described an association between neurofibromatosis (NF) and interstitial lung disease. To date, more than 60 cases of interstitial lung disease associated with NF have been reported, but relatively few reports included high-resolution computed tomographic (HRCT) scans. ⋯ We present the case of a 34-year-old male smoker with NF and HRCT findings of diffuse lung disease including bullous emphysema, thin-walled cysts, and diffuse ground glass. Although NF-associated diffuse lung disease (NF-DLD) is disputed as a clinical entity by some, the case presented here adds to the accumulating evidence that NF-DLD is a distinct manifestation of neurofibromatosis.