[Unusual clinical forms of pseudo-LE syndrome: an induced immune-complex disease? (author's transl)]. 1975

J Maintz, and B Henningsen, and K Buchholz, and H Harders

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.

UI MeSH Term Description Entries
D007105 Immune Complex Diseases Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA. Hypersensitivity, Type III,Type III Hypersensitivity,Disease, Immune Complex,Diseases, Immune Complex,Hypersensitivities, Type III,Immune Complex Disease,Type III Hypersensitivities
D008179 Lupus Erythematosus, Discoid A chronic form of cutaneous lupus erythematosus (LUPUS ERYTHEMATOSUS, CUTANEOUS) in which the skin lesions mimic those of the systemic form but in which systemic signs are rare. It is characterized by the presence of discoid skin plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy. Lesions are surrounded by an elevated erythematous border. The condition typically involves the face and scalp, but widespread dissemination may occur. Lupus Erythematosus, Chronic Cutaneous,Lupus Erythematosus, Cutaneous, Chronic,Discoid Lupus Erythematosus
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008750 Methyldopa An alpha-2 adrenergic agonist that has both central and peripheral nervous system effects. Its primary clinical use is as an antihypertensive agent. Methyldopate,alpha-Methyldopa,Aldomet,Alphamethyldopa,Apo-Methyldopa,Dopamet,Dopegit,Dopegyt,Dopergit,Hydopa,Meldopa,Nu-Medopa,Sembrina,alpha-Methyl-L-Dopa,Apo Methyldopa,Nu Medopa,alpha Methyl L Dopa,alpha Methyldopa
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D008928 Mitochondria Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed) Mitochondrial Contraction,Mitochondrion,Contraction, Mitochondrial,Contractions, Mitochondrial,Mitochondrial Contractions
D009393 Nephritis Inflammation of any part of the KIDNEY. Nephritides
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
D010653 Phenylbutazone A butyl-diphenyl-pyrazolidinedione that has anti-inflammatory, antipyretic, and analgesic activities. It has been used in ANKYLOSING SPONDYLITIS; RHEUMATOID ARTHRITIS; and REACTIVE ARTHRITIS. Diphenylbutazone,Fenilbutazon,Butacote,Butadion,Butadione,Butapirazol,Butapyrazole,Butazolidin
D011720 Pyrazoles Azoles of two nitrogens at the 1,2 positions, next to each other, in contrast with IMIDAZOLES in which they are at the 1,3 positions.

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