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[Primary Sjögren's syndrome and glomerulonephritis]
U Tholl1, K Hartung, U Helmchen
1Medizinische Klinik II, Zentralkrankenhaus Reinkenheide, Bremerhaven.
Deutsche Medizinische Wochenschrift (1946)
|January 20, 1999
Summary
A rare case of nephrotic syndrome and focal segmental glomerulosclerosis associated with Sjögren's syndrome, potentially triggered by infection, highlights the role of HLA-DR3 in genetic predisposition and the efficacy of steroids and cyclosporin A in treatment.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Sjögren's syndrome is an autoimmune disease primarily affecting exocrine glands, but can have systemic manifestations.
- Glomerular diseases, including focal segmental glomerulosclerosis (FSGS), are rare but serious complications of Sjögren's syndrome.
Observation:
- An 82-year-old woman presented with nephrotic syndrome, acute renal failure, xerostomia, and dry eyes.
- Investigations revealed positive antinuclear antibodies (ANA), anti-Ro(SS-A), and anti-La(SS-B) antibodies, with HLA-DR3 alleles.
- Renal biopsy confirmed FSGS, benign nephrosclerosis, and tubular atrophy.
Findings:
- Treatment with hemodialysis and prednisone initially improved renal function and xerostomia.
- Recurrence of nephrotic syndrome upon prednisone dose reduction was managed with increased prednisone and addition of cyclosporin A, achieving remission.
- The patient's genetic predisposition was suggested by HLA-DR3 alleles, and the condition may have been triggered by viral infections.
Implications:
- This case underscores the association between FSGS and Sjögren's syndrome, emphasizing the role of viral triggers and genetic factors (HLA-DR3).
- Corticosteroids are crucial for managing nephrotic syndrome in this context, with cyclosporin A as an effective addition for refractory cases.
- Combined immunosuppressive therapy can stabilize renal function and improve associated symptoms like xerostomia.