Details

Autor(en) / Beteiligte

• Danlos, François-Xavier
• Rossi, Giovanni Maria
• Blockmans, Daniel
• Emmi, Giacomo
• Kronbichler, Andreas
• Durupt, Stéphane
• Maynard, Claire
• Luca, Luminita
• Garrouste, Cyril
• Lioger, Bertrand
• Mourot-Cottet, Rachel
• Dhote, Robin
• Arlet, Jean-Benoit
• Hanslik, Thomas
• Rouvier, Philippe
• Ebbo, Mikael
• Puéchal, Xavier
• Nochy, Dominique
• Carlotti, Agnès
• Mouthon, Luc
• Guillevin, Loïc
• Vaglio, Augusto
• Terrier, Benjamin

Titel

Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome

Ist Teil von

• Autoimmunity reviews, 2017-10, Vol.16 (10), p.1036-1043

Ort / Verlag

Netherlands: Elsevier B.V

Erscheinungsjahr

2017

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• Atypical 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. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 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 49,8months, range 17,25–108months), 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. AAV 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. •ANCA-associated vasculitis and IgG4-related diseases may overlap, mainly in patients with granulomatosis with polyangiitis.•IgG4-related diseases manifestations were mainly chronic periaortitis, orbital mass and tubulointerstitial nephritis.•Rituximab could be preferentially used in these overlap syndromes to treat both diseases.