Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis.
We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.
D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis; D001172:Arthritis, Rheumatoid / Q000150:complications; D003095:Collagen Diseases / Q000150:complications; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000150:complications; D007136:Immunoglobulins / Q000032:analysis; D008168:Lung / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000209:etiology / Q000276:immunology* / Q000401:mortality; D012217:Rheumatoid Factor / Q000032:analysis
D000974:Antibodies, Antinuclear; D007136:Immunoglobulins; D012217:Rheumatoid Factor