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ACUTE SCLERODERMA IN STABLE MIXED CONNECTIVE TISSUE DISEASE: TREATMENT BY PLASMAPHERESIS
Author(s) -
CRAPPER R. M.,
DOWLING J. P.,
MACKAY I. R.,
WHITWORTH J. A.
Publication year - 1987
Publication title -
australian and new zealand journal of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0004-8291
DOI - 10.1111/j.1445-5994.1987.tb01237.x
Subject(s) - medicine , scleroderma (fungus) , plasmapheresis , microangiopathic hemolytic anemia , mixed connective tissue disease , pathology , connective tissue , connective tissue disease , disease , immunology , autoimmune disease , thrombotic thrombocytopenic purpura , antibody , platelet , inoculation
This report describes a case of stable mixed connective tissue disease (MCTD) with development of acute scleroderma with hypertension, oliguric renal failure, microangiopathic hemolytic anemia, and pulmonary infiltrates. The renal histology in the acute episode was that of scleroderma with intimal sclerosis and ‘onion skinning’ of vessels and glomerular ischemic injury but with no evidence of damage by immune complexes either histologically or by immunofluorescence. Improvement occurred after treatment with plasmapheresis, cyclosphamide, and captopril with return of near normal renal function.