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Sjogren Syndrome — SCE Rheumatology MCQ

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HardConnective Tissue DiseaseSjogren SyndromeSCE Rheumatology

A 50-year-old woman with known Sjogren syndrome presents with bilateral lower limb purpura peripheral neuropathy and renal impairment. Cryoglobulins are positive (type II - monoclonal IgM kappa with RF activity). Hepatitis C serology is negative. What is the first-line treatment for severe cryoglobulinaemic vasculitis in Sjogren syndrome?

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Correct answer: A — Rituximab with glucocorticoids

For severe cryoglobulinaemic vasculitis complicating primary Sjogren syndrome (in the absence of hepatitis C), Rituximab with glucocorticoids is the preferred treatment. Rituximab targets the B-cell clone producing the pathogenic monoclonal immunoglobulin. BSR 2024 Sjogren guidelines support Rituximab for systemic manifestations including vasculitis. Plasma exchange may be added for hyperviscosity or life-threatening manifestations. In HCV-associated cryoglobulinaemia, antiviral therapy is first-line, but this patient is HCV-negative.

Reference: BSR 2024 - Guideline on management of Sjogren disease; De Vita S et al 2012 Rituximab in cryoglobulinaemia