Presentation, Prognosis and Clinical-Pathological Correlations of Cutaneous Manifestations in ANCA-Associated Vasculitides

Laure Frumholtz¹, Sara Laurent², Olivier Aumaître³, Francois Maurier⁴, Guillaume Le Guenno⁵, Agnès Carlotti², Alexiane Dallot², Jean-Louis Kemeny⁶, Laurent Antunes⁷, Nicolas Froment⁷, Sylvie Fraitag⁸, Jonathan London⁹, Claire Le Jeunne¹⁰, Benoit Terris², Luc Mouthon¹⁰, Selim Aractingi¹¹, Loïc Guillevin¹⁰, Nicolas Dupin¹¹ and Benjamin Terrier¹², ¹Internal Medicine, Cochin Hospital, Paris, France, ²Pathology, Cochin Hospital, Paris, France, ³CHU Pitié-Salpêtrière - Department of Internal Medicine 2. Referal center for SLE/APS, Paris, France, ⁴Department of Internal Medicine, HP Metz Belle Isle Hospital, Metz, France, ⁵Internal Medicine department, Clermont-Ferrand, France, ⁶Pathology, CHU, Clermont-Ferrand, France, ⁷Pathology, CH, Metz, France, ⁸Pathology, Necker, Paris, France, ⁹INSERM U1016, Institut Cochin, Equipe Neutrophiles et Vascularites, Paris, France, ¹⁰Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France, ¹¹Dermatology, Cochin Hospital, Paris, France, ¹²National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: ANCA, Cutaneous manifestations, histopathologic 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: Cutaneous involvement is frequent during ANCA-associated vasculitis (AAV) and can reveal the disease. However, no large study on presentation and clinical-pathological correlations is available. In the present study, we aimed to analyze the spectrum and impact of cutaneous manifestations (CM) during AAV, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), and to perform clinical-pathological correlations.

Methods: We conducted a French multicenter retrospective study on patients with AAV included in the French Vasculitis Study Group database. Clinical and biological presentation, and relapse-free and overall survival have been analyzed in patients with and without CM. Clinical-pathological correlations of 38 biopsies from 32 patients were performed by 3 pathologists in a blind manner.

Results: 1553 patients were included: GPA (n=743), EGPA (n=436) and MPA (n=374). Cutaneous manifestations were more frequent in EGPA (53%) and MPA (52%) compared to GPA (35%) (p<0.0001), and palpable purpura was the most frequent reported CM (21%). Oral ulcers were more frequent in GPA than in MPA and EGPA (4.6% vs. 0.3% and 2.5%, p<0.001). Pitting edema and livedo were more frequent in MPA than in GPA and EGPA (19.5% vs. 4.3% and 13%, p<0.001, and 12.4% vs. 2.6% and 0.5%, p<0.001, respectively). Urticarial lesions were more frequent in EGPA than in GPA and MPA (11.5% vs. 2% and 3.5%, p<0.001), such as nodules (p<0.0001) and purpura (p=0.0004). Pyoderma gangrenosum (1.1%) and gingival hyperplasia (0.9%) were only observed in GPA. GPA patients with CM had more frequent vasculitis manifestations (i.e., alveolar hemorrhage, renal and gastrointestinal involvement) than granulomatosis manifestations (pulmonary nodules, pachymeningitis). Finally, relapse-free survival [HR 1.29, p=0.03] and overall survival [HR 1.89, p=0.005] were poorer in GPA patients with CM, but not in MPA and EGPA. For clinical-pathological correlations, CM biopsy specimen included purpura (n=13), nodules (n=10), papules (n=5), urticaria (n=4) and unspecified lesions (n=6). Pathological analysis showed vasculitis (68%), granulomatous infiltrates (72%), and both (50%). Vasculitis was mainly observed in purpura and nodules (86 and 80%). Granulomatous infiltrates were variable and observed regardless of the type of AAV: granulomatous vasculitis was noted in half of purpura and nodules, whereas interstitial granulomatous dermatitis was mainly noted in urticaria and papules in 75 and 60%, respectively. Neutrophils were predominant in 63%, and eosinophils seemed to be more frequent in EGPA than GPA.

Conclusion: Cutaneous manifestations are common in AAV, and more frequently observed in EGPA and MPA than GPA. Some CM are quite specific to the type of AAV. Cutaneous involvement is associated with more severe disease and poorer outcome in GPA. Clinical-pathological correlations revealed that no histological feature was specific to the type of AAV, but granulomatous infiltrates differs according to the type of lesions.

Disclosure: L. Frumholtz, None; S. Laurent, None; O. Aumaître, None; F. Maurier, None; G. Le Guenno, None; A. Carlotti, None; A. Dallot, None; J. L. Kemeny, None; L. Antunes, None; N. Froment, None; S. Fraitag, None; J. London, None; C. Le Jeunne, None; B. Terris, None; L. Mouthon, None; S. Aractingi, None; L. Guillevin, None; N. Dupin, None; B. Terrier, None.

To cite this abstract in AMA style:

Frumholtz L, Laurent S, Aumaître O, Maurier F, Le Guenno G, Carlotti A, Dallot A, Kemeny JL, Antunes L, Froment N, Fraitag S, London J, Le Jeunne C, Terris B, Mouthon L, Aractingi S, Guillevin L, Dupin N, Terrier B. Presentation, Prognosis and Clinical-Pathological Correlations of Cutaneous Manifestations in ANCA-Associated Vasculitides [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/presentation-prognosis-and-clinical-pathological-correlations-of-cutaneous-manifestations-in-anca-associated-vasculitides/. Accessed .

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