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Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review

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.

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No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript.

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All listed co-authors have contributed toward case design and data collection and interpretation. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work in accordance with ICMJE authorship recommendation.

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Correspondence to Nedaa Skeik.

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Skeik, N., Hari, G. & Nasr, R. Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review. Rheumatol Int 39, 1983–1988 (2019). https://doi.org/10.1007/s00296-019-04343-3

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Keywords

  • Antineutrophil cytoplasmic antibodies (ANCA)
  • Vasculitis
  • Aortitis
  • Rituximab