IgG4-Related Disease — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseaseIgG4-Related DiseaseSCE Rheumatology

A 40-year-old woman with IgG4-related disease affecting the lacrimal glands parotid glands and retroperitoneum has been treated with Prednisolone with good response. She relapses during steroid tapering. According to current evidence what is the best steroid-sparing option for relapsing IgG4-RD?

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Correct answer: D — Rituximab

Rituximab is the most effective steroid-sparing agent for IgG4-related disease with high response rates in relapsing or refractory disease. IgG4-RD is characterised by tumefactive lesions with storiform fibrosis and IgG4+ plasma cell infiltrates. Glucocorticoids are first-line and highly effective for initial treatment but relapse rates are high during tapering. Rituximab depletes the short-lived plasmablasts that are the key effector cells in IgG4-RD. Conventional immunosuppressives (Azathioprine Mycophenolate) have lower response rates than Rituximab.

Reference: Deshpande V et al 2012 IgG4-RD consensus; Carruthers MN et al 2015 Rituximab in IgG4-RD