Renal involvement in Churg-Strauss syndrome. 1990

E J Clutterbuck, and D J Evans, and C D Pusey
Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London.

Churg-Strauss syndrome is part of the spectrum of systemic vasculitis, but can be distinguished from the other necrotising vasculitides on the basis of clinical and histological criteria. Renal involvement is not regarded as a prominent feature and is generally mild. We report a series of 19 patients with Churg-Strauss syndrome referred to the Hammersmith Hospital between 1976 and 1986. Renal involvement was common (84%) and often severe: three patients had nephrotic syndrome; four presented with serum creatinine greater than 500 mumol/l; and two required dialysis. Focal segmental glomerulonephritis was the predominant lesion on biopsy (85%), often with necrotising features and crescent formation. Extravascular granulomata and eosinophilic infiltrates were uncommon. Treatment with high-dose prednisolone was generally successful in milder cases, but was not always sufficient to halt the progression of more severe vasculitis. In these cases, additional immunosuppressive therapy with azathioprine, cyclophosphamide or plasma exchange was of benefit. Renal disease improved in 14 of 16 patients, ten of whom have maintained normal renal function on follow-up. Only one patient died, as a result of progressive cardiac disease.

UI MeSH Term Description Entries
D007674 Kidney Diseases Pathological processes of the KIDNEY or its component tissues. Disease, Kidney,Diseases, Kidney,Kidney Disease
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
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
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003520 Cyclophosphamide Precursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the LIVER to form the active aldophosphamide. It has been used in the treatment of LYMPHOMA and LEUKEMIA. Its side effect, ALOPECIA, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer. (+,-)-2-(bis(2-Chloroethyl)amino)tetrahydro-2H-1,3,2-oxazaphosphorine 2-Oxide Monohydrate,B-518,Cyclophosphamide Anhydrous,Cyclophosphamide Monohydrate,Cyclophosphamide, (R)-Isomer,Cyclophosphamide, (S)-Isomer,Cyclophosphane,Cytophosphan,Cytophosphane,Cytoxan,Endoxan,NSC-26271,Neosar,Procytox,Sendoxan,B 518,B518,NSC 26271,NSC26271
D004359 Drug Therapy, Combination Therapy with two or more separate preparations given for a combined effect. Combination Chemotherapy,Polychemotherapy,Chemotherapy, Combination,Combination Drug Therapy,Drug Polytherapy,Therapy, Combination Drug,Chemotherapies, Combination,Combination Chemotherapies,Combination Drug Therapies,Drug Polytherapies,Drug Therapies, Combination,Polychemotherapies,Polytherapies, Drug,Polytherapy, Drug,Therapies, Combination Drug
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup

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