Digital vasculitis in patients with cryptogenic fibrosing alveolitis. 1984

M E Hodson, and P L Haslam, and S G Spiro, and M Turner-Warwick

Twelve patients are described with features of cryptogenic fibrosing alveolitis (CFA) with associated florid digital vasculitis (DV). Nine patients had features of various 'connective tissue disorders' involving systems other than the lung, but three had no such features. The immunological features of these patients with DV have been compared with a group of 17 patients who had CFA alone ('lone CFA') and in whom the same range of special laboratory tests were being undertaken concurrently. The group with DV had a higher erythrocyte sedimentation rate and a higher serum IgM, but the most interesting immunological distinction was an increased prevalence of antinuclear antibodies with a nucleolar staining pattern. In addition, the group with DV also had a greater prevalence of single stranded DNA antibodies and of smooth muscle autoantibodies. Circulating immune complexes were not detected more frequently in the group with vasculitis. Ten of the 12 patients were initially treated with corticosteroids and neither the vasculitis nor the lungs improved. In eight the incapacitating DV responded to immunosuppressants (four) or penicillamine (four). In three of these eight patients the lungs also improved.

UI MeSH Term Description Entries
D007166 Immunosuppressive Agents Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging. Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010396 Penicillamine 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease. Dimethylcysteine,Mercaptovaline,beta,beta-Dimethylcysteine,Copper Penicillaminate,Cuprenil,Cuprimine,D-3-Mercaptovaline,D-Penicillamine,Metalcaptase,D 3 Mercaptovaline,D Penicillamine,Penicillaminate, Copper,beta,beta Dimethylcysteine
D011239 Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Di-Adreson-F,Predate,Predonine,Di Adreson F,DiAdresonF
D011658 Pulmonary Fibrosis A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D005260 Female Females
D005385 Fingers Four or five slender jointed digits in humans and primates, attached to each HAND. Finger
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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