Clinical Presentation and Outcome of Orbital Mass in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides

Cécile-Audrey Durel¹, Arnaud Hot², Ludovic Trefond³, Olivier Aumaître⁴, Grégory Pugnet⁵, Maxime Samson⁶, Sébastien Abad⁷, Alexandre Belot⁸, Claire Blanchard-Delaunay⁹, Pascal Cathebras¹⁰, Pascal Cohen¹¹, Fleur Cohen¹², Vincent Cottin¹³, Bruno Crestani¹⁴, Stéphanie Dumonteil¹⁵, Claire de Moreuil¹⁶, Stéphane Durupt¹⁷, Jean-Gabriel Fuzibet¹⁸, Margaux Garzaro-regard¹⁹, Nicolas Girszyn²⁰, Bertrand Godeau²¹, Eric Hachulla²², Yvan Jamilloux²³, Patrick Jego²⁴, Estibaliz Lazaro²⁵, Thomas Le Gallou²⁶, Eric Liozon²⁷, Thierry Martin²⁸, Thomas Papo²⁹, Antoinette Perlat²⁶, Pascal Pillet³⁰, Karim Sacre³¹, Pascal Sève³², Loïc Guillevin for the French Vasculitis Study Group³³ and Benjamin Terrier¹¹, ¹Internal Medicine, Hôpital Edouard Herriot, lyon, France, ²Internal Medicine, Hopital Edouard Herriot, Lyon, France, ³Internal medicine, CHU Gabriel-Montpied, clermont-ferrand, France, ⁴CHU Pitié-Salpêtrière - Department of Internal Medicine 2. Referal center for SLE/APS, Paris, France, ⁵Service de Médecine Interne, CHU de Toulouse, Toulouse, Toulouse, France, ⁶Dijon University Hospital, Dijon, France, ⁷Internal medicine, Hôpital Avicennes, avicennes, France, ⁸Pediatric Rheumatology, CHU lyon, Hospices Civils de Lyon, HFME, lyon, France, ⁹Internal Medicine, Centre Hospitalier, Niort, France, ¹⁰Internal Medicine, University Hospital St Etienne, St Etienne, France, ¹¹Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, Paris, France, ¹²Department of Internal Medicine 2. Referal center for SLE/APS, Hôpital Pitié-Salpêtrière, AP-HP, UPMC Univ Paris 06 & French National Reference Center For Systemic Lupus and Antiphospholipid Syndrome, Paris, France, ¹³Louis Pradel Hospital, Claude Bernard University Lyon 1, Lyon, France, ¹⁴Pneumologie A, Hôpital Bichat - Claude Bernard, Paris, France, ¹⁵Internal medicine, CHU de Limoges, Limoges, France, ¹⁶CHU de Brest, Brest, France, ¹⁷Department of Internal and Vascular Medicine, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France, ¹⁸Internal medicine, Hôpital l'Archet, Nice, France, ¹⁹Internal medicine, CHU de Nice, Nice, France, ²⁰CHU de Rouen, Rouen, France, ²¹Internal medicine, Hôpital Henri-Mondor, Créteil, France, ²²CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, Lille, France, ²³Internal medicine department, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France, ²⁴Medecine Interne, CHU Rennes, Rennes, France, ²⁵service de médecine interne et maladies infectieuses, CHU de Bordeaux, Pessac, France, ²⁶Internal medicine, CHU de Rennes, Rennes, France, ²⁷Departement of Internal Medicine, Limoges University Hospital, Limoges, France, ²⁸Cnrs UPR9021, Department of Clinical Immunology and Internal Medicine, Strasbourg University Hospital, IBMC CNRS UPR9021, Strasbourg, France, ²⁹Paris Bichat, Paris, France, ³⁰Paediatrics, Hôpital Pellegrin, University Hospital of Bordeaux, Bordeaux, France, ³¹Department of Internal Medicine, Bichat Hospital, Paris, France, ³²Internal medicine, Internal medicine department, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France, ³³Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: ANCA, ocular involvement and vasculitis

Session Information

Date: Sunday, November 5, 2017

Title: Vasculitis Poster I: Large Vessel Vasculitis

Session Type: ACR Poster Session A

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

Background/Purpose: Orbital mass is a rare ophthalmologic manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) that remains a therapeutic challenge. This study aimed to describe clinical presentation, therapeutic management and outcome of orbital mass in the setting of AAV.

Methods: We conducted a nationwide retrospective study including 59 patients with AAV fulfilling the American College of Rheumatology criteria or Chapel Hill Consensus Conference definitions and orbital mass based on clinical and radiological features. Clinical, biological, radiological and histological characteristics, treatment use and efficacy, and outcome, were analyzed.

Results: Fifty nine patients (33 women) were included. Fifty-six (95%) patients had granulomatosis with polyangiitis (GPA), two eosinophilic granulomatosis with polyangiitis, and one microscopic polyangiitis, with histological evidence of vasculitis found in 52 (90%) patients. Fifty-one (86%) patients were ANCA-positive, with a specificity against PR3 in 71%. Median age at AAV diagnosis and orbital mass onset were 46 and 49 years, respectively, with 46% patients presenting orbital mass concomitantly to AAV diagnosis, 41% patients during AAV course, and 13% previous to AAV diagnosis. Orbital mass was unilateral in 80% patients. Exophthalmia revealed orbital mass in 54 (93%) patients, while loss of visual acuity was noted in 20 (35%) and diplopia in 21 (36%). Fifty-six (95%) patients had a systemic disease at the time of orbital mass, with a median BVAS at 9. Orbital biopsy was performed in 32 (54%) patients, showing lymphoplasmocytic infiltrate in 65%, extravascular granulomas in 48% and necrotizing vasculitis in 36%. Four patients had histological evidence of IgG4-related disease. All but one patient received corticosteroids as first-line therapy, in combination with immunosuppressive agents in 82%. First-line immunosuppressive agents comprised cyclophosphamide in 24 patients (42%), rituximab in 11 (19%), methotrexate in 5 (9%), mycophenolate mofetil in 4 (7%), azathioprine in 2 (4%), and TNF antagonist in one (2%). Complete response was noted in 15 (27%) cases, partial response in 27 (48%), stabilization in 10 (18%) cases, and worsening in 4 (7%) cases. Among patients with complete response, seven (47%) were MPO-ANCA positive. Twenty seven patients (47%) required a second line of treatment because of relapse in 16 patients (59%) after a median delay of 13 months and refractory course in 11 cases (41%). Six of these 27 patients (22%) received corticosteroids alone as first line. All patients were treated with corticosteroids and one or combined immunosuppressive agent(s). After a median follow-up of 68 months after orbital mass diagnosis, ophthalmologic sequelae included visual impairment in 28% with blindness in 17%, orbital muscular extension on MRI with diplopia in 36%, and naso-sinusal bone defect on MRI in 22%.

Conclusion: Orbital mass represents a therapeutic challenge during AAV, especially during GPA. This rare manifestation is associated with refractory course and high-frequency of sequelae, in particular blindness. Histological findings could help physicians to manage orbital mass.

Disclosure: C. A. Durel, None; A. Hot, None; L. Trefond, None; O. Aumaître, None; G. Pugnet, None; M. Samson, None; S. Abad, None; A. Belot, None; C. Blanchard-Delaunay, None; P. Cathebras, None; P. Cohen, None; F. Cohen, None; V. Cottin, None; B. Crestani, None; S. Dumonteil, None; C. de Moreuil, None; S. Durupt, None; J. G. Fuzibet, None; M. Garzaro-regard, None; N. Girszyn, None; B. Godeau, None; E. Hachulla, None; Y. Jamilloux, None; P. Jego, None; E. Lazaro, None; T. Le Gallou, None; E. Liozon, None; T. Martin, None; T. Papo, None; A. Perlat, None; P. Pillet, None; K. Sacre, None; P. Sève, None; L. Guillevin for the French Vasculitis Study Group, None; B. Terrier, None.

To cite this abstract in AMA style:

Durel CA, Hot A, Trefond L, Aumaître O, Pugnet G, Samson M, Abad S, Belot A, Blanchard-Delaunay C, Cathebras P, Cohen P, Cohen F, Cottin V, Crestani B, Dumonteil S, de Moreuil C, Durupt S, Fuzibet JG, Garzaro-regard M, Girszyn N, Godeau B, Hachulla E, Jamilloux Y, Jego P, Lazaro E, Le Gallou T, Liozon E, Martin T, Papo T, Perlat A, Pillet P, Sacre K, Sève P, Guillevin for the French Vasculitis Study Group L, Terrier B. Clinical Presentation and Outcome of Orbital Mass in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/clinical-presentation-and-outcome-of-orbital-mass-in-antineutrophil-cytoplasmic-antibody-associated-vasculitides/. Accessed .

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