title: Plasma exchange and immunosuppressive therapy in a paediatric patient with systemic lupus erythematosus.
abstract: A case is reported of systemic lupus erythematosus (SLE) associated with steroid-resistant nephrotic syndrome. Serial plasma exchanges (PE) combined with prednisolone treatment induced complete normalization of the immunological findings: the anti-DNA antibody, antinuclear antibody, LE cell phenomenon and circulatory immune complexes became negative. The prostacyclin (PGI2) production-supporting activity in the plasma increased to the control range; inhibitors of PGI2 production were eliminated. The creatinine clearance normalized, the urinary protein excretion decreased significantly, and the facial erythema disappeared. Continued treatment with chlorambucil + low-dose prednisolone led to a complete and stable remission of the nephrotic syndrome, and the C3 complement normalized. The low level of PGI2 production-supporting activity in the plasma may be explained by the inhibitor of PGI2 production. PE + immunosuppressive therapy might have beneficial effects on the immunological changes and PGI2 metabolism, and also on the remission of SLE-nephrotic syndrome.
authors.affiliation: Department of Paediatrics, University Medical School of Szeged, Hungary.
authors.forename: S
authors.initials: S
authors.lastname: Túri
authors.forename: I
authors.initials: I
authors.lastname: Haszon
authors.forename: T
authors.initials: T
authors.lastname: Bodrogi
mesh_terms: D001323:Autoantibodies / Q000032:analysis; D002648:Child; D002699:Chlorambucil / Q000627:therapeutic use; D011464:Epoprostenol / Q000096:biosynthesis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D008181:Lupus Nephritis / Q000150:complications; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D010951:Plasma Exchange; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D002699:Chlorambucil; D011239:Prednisolone; D011464:Epoprostenol