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

被引：117

作者：

Danlos, Francois-Xavier ^{[1
]}

Rossi, Giovanni Maria ^{[2
]}

Blockmans, Daniel ^{[3
]}

Emmi, Giacomo ^{[4
]}

Kronbichler, Andreas ^{[5
]}

Durupt, Stephan ^{[6
]}

Maynard, Claire ^{[7
]}

Luca, Luminita ^{[8
]}

Garrouste, Cyril ^{[9
]}

Lioger, Bertrand ^{[10
]}

Mourot-Cottet, Rachel ^{[11
]}

Dhote, Robin ^{[12
]}

Arlet, Jean-Benoit ^{[13
]}

Hanslik, Thomas ^{[14
]}

Rouvier, Philippe ^{[15
]}

Ebbo, Mikael ^{[16
,17
]}

Puechal, Xavier ^{[1
]}

Nochy, Dominique ^{[18
]}

Carlotti, Agnes ^{[19
]}

Mouthon, Luc ^{[1
,20
]}

Guillevin, Loic ^{[1
]}

Vaglio, Augusto ^{[2
]}

Terrier, Benjamin ^{[1
,20
]}

机构：

[1] Hop Cochin, AP HP, Natl Referral Ctr Rare Autoimmune & Syst Dis, Dept Internal Med, Paris, France

[2] Univ Hosp, Nephrol Unit, Parma, Italy

[3] Univ Hosp Leuven, Lab Clin Infect & Inflammatory Disorders, Res Dept Microbiol & Immunol, Clin Dept,Gen Internal Med Dept, Leuven, Belgium

[4] Univ Florence, Dept Expt & Clin Med, Florence, Italy

[5] Med Univ Innsbruck, Dept Internal Med Nephrol & Hypertens 4, Anichstr 35, A-6020 Innsbruck, Austria

[6] Univ Lyon, Grp Hosp Sud, Hosp Civils Lyon, Dept Internal & Vasc Med, Pierre Benite, France

[7] Ctr Hosp Metropole Savoie, Dept Nephrol, Chambery, France

[8] Hop Poitiers, Dept Internal Med, Poitiers, France

[9] Clermont Ferrand Univ Hosp, Dept Nephrol, 58,Rue Montalembert, F-63000 Clermont Ferrand, France

[10] Univ Med Ctr Tours, Dept Internal Med, Tours, France

[11] CHRU Strasbourg, Dept Internal Med, F-67000 Strasbourg, France

[12] Avicenne Univ Hosp, Dept Internal Med, Bobigny, France

[13] Hop Europeen Georges Pompidou, AP HP, Dept Internal Med, Paris, France

[14] Versailles St Quentin Univ, Univ Hosp, AP HP, Dept Internal Med, Boulogne, France

[15] Grp Hosp Pitie Salpetriere, AP HP, Dept Pathol, Paris, France

[16] CHU Timone, AP HM, Dept Internal Med, Marseille, France

[17] Aix Marseille Univ, Marseille, France

[18] Hop Europeen Georges Pompidou, AP HP, Dept Pathol, Paris, France

[19] Hop Cochin, AP HP, Dept Pathol, Paris, France

[20] Univ Paris 05, Paris, France

来源：

AUTOIMMUNITY REVIEWS | 2017年 / 16卷 / 10期

关键词：

ANCA-associated vasculitis; Granulomatosis with polyangiitis (GPA); IgG4-related disease; RETROPERITONEAL FIBROSIS; WEGENERS-GRANULOMATOSIS; POLYANGIITIS WEGENERS; RITUXIMAB; PERIAORTITIS; CELLS; PROTEINASE-3; CRITERIA; CT;

D O I：

10.1016/j.autrev.2017.07.020

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Objective: 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. Methods: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for MV and IgG4-RD Comprehensive Diagnostic Criteria. Results: Eighteen 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 MV in 2/18 (11%). MV 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), MV 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. Conclusion: MV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during MV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. (C) 2017 Elsevier B.V. All rights reserved.

引用

页码：1036 / 1043

页数：8

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