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Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Aortitis Presenting With Acute Dissection: Case Report and
Obteene Azimi-Ghomi1, Jorge De La Portilla1
1Department of General and Vascular Surgery, HCA Florida Kendall Hospital, Miami, FL.
Insights
This study details a rare case of ANCA-associated aortitis, a condition affecting large blood vessels, complicated by acute iliac artery dissection. Successful management involved corticosteroids and endovascular stenting.
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Vasculitides encompass inflammatory diseases of blood vessel walls, classified by vessel size (large, medium, small).
- ANCA-associated vasculitis (AAV) typically affects small vessels, but large vessel involvement, such as aortitis, is occasionally reported.
- ANCA-associated aortitis is a rare condition with limited diagnostic and treatment evidence.
Observation:
- An 80-year-old male presented with ANCA-associated aortitis.
- The patient experienced a complication of acute dissection of the left common iliac artery.
- This presentation represents a potentially novel association of ANCA-associated aortitis with acute dissection.
Findings:
- The case was successfully managed using corticosteroid therapy.
- Endovascular stenting was employed to treat the involved iliac artery.
- This approach highlights a viable treatment strategy for this rare vascular complication.
Implications:
- This case expands the understanding of ANCA-associated aortitis presentations.
- It underscores the importance of considering AAV in large vessel pathologies.
- The successful management provides insights for future clinical practice in rare vasculitic diseases.
Abstract:
Description Vasculitides are a group of diseases that produces vasculitis, which is characterized by inflammatory infiltrates within blood vessel walls and results in intimal injury as well as progressive mural destruction. Infiltrates are characterized per the Chapel Hill classification, into large, medium, and small-vessel vasculitides. ANCA-associated vasculitis (AAV) is a disease that has been described as involving small-sized vessels. However, some cases of large vessel disease involvement have been documented. ANCA-associated aortitis is a rare entity poorly described in the literature. Due to the rarity of this pathology, there is no Level I evidence available regarding diagnosis and treatment. We present the rare case of an 80-year-old male presenting with ANCA-associated aortitis complicated by acute dissection of the left common iliac artery. His case was successfully managed by corticosteroid therapy and endovascular stenting of the involved iliac artery. ANCA-associated aortitis is a rare entity that has not been described well in the current literature. We believe this case to be the first involving ANCA-associated aortitis presenting with an acute dissection.
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