ANCA-Associated Vasculitides and IgG4-Related Disease: An Overlapping Syndrome? a European Observational Study of 18 Patients

Francois-Xavier Danlos¹, Giovanni Maria Rossi², Daniel Blockmans³, Giacomo Emmi⁴, Andreas Kronbichler⁵, Stéphane Durupt⁶, Claire Maynard⁷, Luminita Luca⁸, Cyril Garrouste⁹, Bertrand Lioger¹⁰, Rachel Mourot¹¹, Robin Dhote¹², Jean-Benoit Arlet¹³, Thomas Hanslik¹⁴, Mikael Ebbo¹⁵, Agnès Carlotti¹⁶, Luc Mouthon¹⁷, Loïc Guillevin¹⁷, Augusto Vaglio¹⁸ and Benjamin Terrier¹⁹, ¹Internal Medicine, Cochin Hospital, Paris, France, ²Nephrology, Parma, Parma, Italy, ³General Internal Medicine, University Hospitals Gasthuisberg, Leuven, Belgium, ⁴Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁵Nephrology, Hospital, Innsbruck, Austria, ⁶Internal Medicine, CHU, Lyon, France, ⁷Internal Medicine, CH, Chambéry, France, ⁸Internal Medicine, CHU, Poitiers, France, ⁹Nephrology, CHU, Clermont-Ferrand, France, ¹⁰GICC UMR 7292, University François Rabelais, Tours, France, ¹¹Internal Medicine, CHU, Strasbourg, France, ¹²Service de médecine interne. Hôpital Avicenne, Paris, France, ¹³Service de médecine interne, Hopital Européen Georges Pompidou, Paris, France, ¹⁴Internal Medicine, CHU, Boulogne Billancourt, France, ¹⁵Internal Medicine, Aix-Marseille Université, AP-HM, Marseille, France, ¹⁶Pathology, Cochin Hospital, Paris, France, ¹⁷Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France, ¹⁸Nephrology, University Hospital of Parma, Parma, Italy, ¹⁹National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

Meeting: ACR/ARHP Annual Meeting 2016

Date of first publication: September 28, 2016

Keywords: ANCA, IgG4 Related Disease and vasculitis

Session Information

Date: Monday, November 14, 2016

Title: Vasculitis - Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Atypical manifestations have been described in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), such as periaortic fibrosis, pachymeningitis and orbital mass. Because these manifestations have been frequently described in 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 with AAV fulfilling 1990 ACR criteria and/or EMA algorithm and/or the definitions from the 2012 Chapel Hill Consensus Conference, and IgG4-RD fulfilling Umehara criteria. Such criteria defined IgG4-RD as possible, probable or definite. Data were collected using a standardized form by physicians in charge of patients.

Results: Eighteen patients were included (mean age 56.8 years, 13 men and 5 women). AAV and IgG4-RD diagnoses were made concomitantly in 12/18 patients (67%). AAV diagnosis preceded IgG4-RD diagnosis in 4/18 (22%) with a mean interval of 90 months (24-156), and IgG4-RD preceded AAV in 2/18 (11%). AAV diagnosis included granulomatosis with polyangiitis (GPA) in 14 (78%), microscopic polyangiitis (MPA) in 2 (12%), eosinophilic granulomatosis and polyangiitis (EGPA) in 1 (5%) and renal-limited AAV in 1 (5%). ANCA were positive in 15 patients (83%), including PR3-ANCA in 9 cases and MPO-ANCA in 5 cases. At diagnosis, mean BVAS was 16 (4-36). IgG4-RD diagnosis included definite IgG4-RD in 5 cases (28%), probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD involvement were chronic periaortitis (i.e., retroperitoneal fibrosis, periaortitis and/or aortic aneurysm) in 9/18 patients (50%), orbital mass and tubulointerstitial nephritis in 4 cases each (22%), prevertebral fibrosis in 3 (16,7%), pachymeningitis and autoimmune pancreatitis in 2 cases each (11%), and salivary gland involvement, dacryoadenitis, mesenteric fibrosis, interstitial lung disease and myocardial involvement in 1 case each (5%). Medium serum IgG4 level was 2.3 g/L (range <0.07-4.85). Nineteen biopsies were performed and were contributive in 16/18 (84%) patients. Overall, 9/18 had histological evidence of vasculitis and 10/18 evidence of IgG4-RD. Histological features of IgG4-RD included dense lymphoplasmacytic infiltrate in 100% and dense fibrosis in 80%, but no obliterative phlebitis. IgG4/IgG ratio >40% was obtained in 50% and a number of IgG4-positive plasma cells >10 per high-power field in 80%. Patients required a median number of 2 (range 0-4) lines of immunosuppressants in association with corticosteroids. During a mean follow-up was 65.6 months (range 4-156), AAV relapsed in 9/18 (50%) whereas IgG4-RD relapsed in 3/18 (17%). One patient died because of diverticular peritonitis.

Conclusion: This study illustrates that AAV and IgG4-RD may overlap, suggesting that they could represent various expressions of similar Th2 dominant imbalance of T cell responses. Identification of manifestations suggesting IgG4-RD in AAV patients could be useful, in particular in case of IgG4-RD manifestations mimicking refractory granulomatous lesions.

Disclosure: F. X. Danlos, None; G. M. Rossi, None; D. Blockmans, None; G. Emmi, None; A. Kronbichler, None; S. Durupt, None; C. Maynard, None; L. Luca, None; C. Garrouste, None; B. Lioger, None; R. Mourot, None; R. Dhote, None; J. B. Arlet, None; T. Hanslik, None; M. Ebbo, None; A. Carlotti, None; L. Mouthon, None; L. Guillevin, None; A. Vaglio, None; B. Terrier, None.

To cite this abstract in AMA style:

Danlos FX, Rossi GM, Blockmans D, Emmi G, Kronbichler A, Durupt S, Maynard C, Luca L, Garrouste C, Lioger B, Mourot R, Dhote R, Arlet JB, Hanslik T, Ebbo M, Carlotti A, Mouthon L, Guillevin L, Vaglio A, Terrier B. ANCA-Associated Vasculitides and IgG4-Related Disease: An Overlapping Syndrome? a European Observational Study of 18 Patients [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/anca-associated-vasculitides-and-igg4-related-disease-an-overlapping-syndrome-a-european-observational-study-of-18-patients/. Accessed .

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