[Primary Sjögren's syndrome and glomerulonephritis]

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.