Clinically Silent Sjögren Syndrome Presenting With Cryoglobulinemia.
Ameya Deshpande, Md Ali Osama, Pallav Gupta et al.
✦ AI-curated · Sources linked
Case in brief
A 33-year-old woman presented with pedal edema, intermittent arthralgia, hematuria, and impaired renal function. Kidney biopsy showed intraluminal pseudothrombi with dominant immunoglobulin M deposition, consistent with cryoglobulinemic glomerulonephritis; serology confirmed mixed cryoglobulinemia. Strong anti-SSA and anti-SSB antibody positivity and a labial salivary gland biopsy meeting 2016 ACR/EULAR criteria established Sjögren syndrome despite no sicca symptoms. Renal function improved with corticosteroids and rituximab. Secondary thrombotic microangiopathy developed and responded to plasma exchange.
What made this case unusual
Sjögren syndrome was clinically silent, without sicca symptoms, and was uncovered through renal disease presenting with mixed cryoglobulinemic glomerulonephritis.
Diagnostic / clinical pearl
In unexplained cryoglobulinemic glomerulonephritis, kidney biopsy findings can prompt evaluation for an occult systemic autoimmune disease, including Sjögren syndrome even when sicca symptoms are absent.