title: [Unusual clinical forms of pseudo-LE syndrome: an induced immune-complex disease? (author's transl)].
abstract: Two of nine patients with pseudo-LE syndrome had previously undescribed organ manifestations. In one there was the full-blown picture of cutaneous lupus erythematodes, with renal and CNS involvement, ending fatally. Immunohistologically there was severe vasculitis and nephritis, histologically generalised necrotizing angitis. It is possible that the dramatic clinical picture was due to alpha-methyldopa. In the second case lymphadenopathy with bihilar involvement and splenomegaly predominated. Histologically there was no evidence of Boeck's sarcoid. Further studies revealed systemic involvement with immune-complex nephritis, electron-dense deposits in the glomerular basal membrane, and the typical changes of lupus erythematodes in the marrow. In a third case there were similar changes in kidney and marrow. These findings support the view of an immune-complex disease which can be fatal. An unusual feature in seven of the nine patients was the proven intake of Venopyronum¿ coated tablets in temporal relationship to the manifestation of the disease. It is possible that the described cases of pseudo-LE syndrome were drug-induced. The immunological mechanism of the disease would, in that case, be analogous to penicillamine nephropathy.
mesh_terms: D000328:Adult; D000368:Aged; D000906:Antibodies / Q000032:analysis; D001855:Bone Marrow Diseases / Q000276:immunology; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000276:immunology*; D008179:Lupus Erythematosus, Discoid / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000139:chemically induced / Q000175:diagnosis / Q000276:immunology*; D008750:Methyldopa / Q000009:adverse effects; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D009393:Nephritis / Q000139:chemically induced / Q000276:immunology; D010396:Penicillamine / Q000009:adverse effects; D010653:Phenylbutazone / Q000031:analogs & derivatives; D011720:Pyrazoles / Q000009:adverse effects; D012698:Serologic Tests; D014652:Vascular Diseases / Q000276:immunology
chemical_list: D000906:Antibodies; D011720:Pyrazoles; C010619:phenopyrazone; D008750:Methyldopa; D010653:Phenylbutazone; D010396:Penicillamine
vernacular_title: Ungewöhnliche Erscheinungsformen des Pseudo-LE-Syndroms. Eine induzierte Immunkomplexkrankheit?