Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review
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Rheumatology International https://doi.org/10.1007/s00296-019-04343-3
INTERNATIONAL
CASE BASED REVIEW
Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)‑associated vasculitis: a case‑based review Nedaa Skeik1 · Gopika Hari1 · Rawad Nasr2 Received: 28 November 2018 / Accepted: 9 June 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019
Abstract Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a systemic necrotizing small vessel vasculitis primarily affecting elderly patients. Neutrophil apoptosis and release of pro-inflammatory mediators promote small vessel inflammation and hence multi-organ disease. It rarely affects larger vessels with extremely rare aortic involvement. Diagnosis is made based on clinical presentation, tissue biopsy of affected organ, as well as immunofluorescence and ELISA assays for ANCA. Management includes immunosuppression (e.g., glucocorticoids, cyclophosphamide and rituximab) and supportive therapy. We present a rare case of a younger patient with AAV involving the aorta. The patient’s diagnosis was supported by clinical presentation, systemic organ involvement, strongly positive c-ANCA, and skin as well as aortic tissue biopsy results. After failing multiple immunosuppressants, he responded well to rituximab with improved symptoms, inflammatory markers, and imaging findings. Based on our literature review, we were only able to find ten cases of ANCA-related vasculitis involving the aorta. This is the first reported case of successful treatment of AAV-related aortitis using rituximab. Our case report and literature review provide insight into treatment of severe cases of AAV with aortic involvement. Keywords Antineutrophil cytoplasmic antibodies (ANCA) · Vasculitis · Aortitis · Rituximab
Introduction Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a necrotizing small vessel vasculitis that is mostly associated with positive antibodies against myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) [1, 2]. ANCA-negative AAV has been rarely reported in the literature [3]. The major subtypes of AAV include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), renal-limited vasculitis (RLV), and eosinophilic granulomatosis with polyangiitis (EGPA) [1, 2]. * Nedaa Skeik [email protected] Gopika Hari [email protected] Rawad Nasr [email protected] 1
Minneapolis Heart Institute®, at Abbott Northwestern Hospital, Part of Allina Health, Mail Route: 33300, 920 E. 28th Street, Suite 300, Minneapolis, MN 55407, USA
Hennepin Healthcare, University of Minnesota, Minneapolis, MN, USA
2
AAV equally affects both genders and is commonly diagnosed in older Caucasian adults at their 65–74 years of age [4–6]. An overall incidence rate of 13–20 million AAV cases has been reported in Europe [6]. Patients with AAV usually present with constitutional symptoms including fatigue, weight loss, decreased appetite and fever [4, 7, 8]. Patients with MPA and GPA can also present with orbital, ear
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