Rituximab for the Treatment of IgG4-Related Disease

• DOI: 10.1097/md.0b013e3182431ef6
• Publication Date: 2011-12-30T06:35:19Z
• Authors: Arezou Khosroshahi; Mollie N. Carruthers; Vikram Deshpande; Sebastian Unizony; Donald B. Bloch; John H. Stone
• Keywords: IgG4-Related Disease

Patients with IgG4-related disease (IgG4-RD) typically have elevated serum concentrations of IgG4 and share histopathologic features that are similar across affected organ(s). IgG4-RD patients frequently require prolonged treatment glucocorticoids often unable to taper these medications. Traditional disease-modifying antirheumatic drugs (DMARDs) generally ineffective. We assessed the clinical serologic responses B lymphocyte depletion therapy in 10 consecutive steroid- DMARD-refractory IgG4-RD.Ten were treated rituximab (RTX) (2 infusions 1000 mg, 15 days apart). Clinical improvement was by monitoring patient's ability prednisone discontinuation stop DMARDs; serial measurements total IgG subclasses; follow-up radiologic assessments guided particular pattern organ involvement. also developed retrospectively applied Disease Activity Index Flare Tool.Organ involvement included pancreas, biliary tree, aorta, salivary glands (submandibular parotid), lacrimal glands, lymph nodes, thyroid gland, retroperitoneum. Nine demonstrated striking within 1 month starting RTX. One patient advanced fibrosis associated Riedel thyroiditis a history multiple other systems did not but progress involve new organs. All able discontinue DMARDs following RTX therapy. Significant decreases observed for subclass only. Four re-treated after 6 months because either symptom recurrence increasing concentration at time peripheral cell reconstitution (n = 2) or physician discretion 2). Repeated courses maintained their effectiveness resulted further concentrations. In who had an increased presentation, level appeared be reliable measure activity.IgG4-RD is idiopathic, multiorgan inflammatory which diverse manifestations linked characteristic immunohistochemical features. Treatment led prompt refractory all active inflammation. Serial treatments may lead progressive declines better control. Serum remain low, activity quiescent even significant proportion patients.