Antineutrophil Cytoplasmic Antibody-Associated Vasculitides and IgG4-Related Disease: A new overlap syndrome

Publication date: Available online 2 August 2017
Source:Autoimmunity Reviews
Author(s): François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
ObjectiveAtypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.MethodsWe conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria.ResultsEighteen patients were included (median age 55.5 years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0–4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8 months, range 17,25–108 months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases.ConclusionAAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.



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