Primäres Sjögren-Syndrom und Glomerulonephritis

Autor: M Anlauf, U Helmchen, U Tholl, K Hartung
Rok vydání: 2008
Předmět:
Zdroj: DMW - Deutsche Medizinische Wochenschrift. 123:1541-1546
ISSN: 1439-4413
0012-0472
DOI: 10.1055/s-2007-1024220
Popis: History and clinical findings An 82-year-old woman with hypertension for 20 years developed a nephrotic syndrome with severe oedema followed by acute oliguric renal failure after a bout of bronchitis and a gastrointestinal infection. She also complained of xerostomia and dry eyes of recent onset. Investigations Biochemical tests showed a serum creatinine level of 6.1 mg/dl, a 1:5120 antinuclear antibody (ANA) titre, and positive values for Ro(SS-A) and La(SS-B) antibodies. HLA-DR typing demonstrated HLA-DR3 (HLA-DRB1*0301) and DR13 (HLA-DRB1*13) antigens. Renal biopsy revealed minimal glomerular lesions with focal and segmental glomerulo-sclerosis as well as (hypertension-induced) benign nephrosclerosis and focal tubular atrophy with interstitial fibrosis. Treatment and course After two hemofiltrations and concomitant administration of 100 mg prednisone renal function quickly improved and the proteinuria fell to 1 g/dl. At the same time the xerostomia improved. The nephrotic syndrome recurred 7 months later after the prednisone dose had been reduced to 10 mg/d, but after the dose had been raised to 50 mg/d and cyclosporin A (150 mg/d) had been added a lasting remission occurred and renal function became stable though impaired. Conclusion The relatively rare association of glomerular disease (here focal segmental glomerulosclerosis) with Sjogren's syndrome can, as in this case, be triggered by a viral infection. A genetic predisposition for Sjogren's syndrome is suggested by the demonstration of HLA-DR3 alleles. Administration of steroids is indicated for the treatment of the nephrotic syndrome and, in case of recurrence, can be combined with cyclosporin A. Both drugs also influence the symptoms of Sjogren's syndrome.
Databáze: OpenAIRE