Catastrophic Antiphospholipid Syndrome Following Treated Antineutrophilic Cytoplasmic Antibody (ANCA) Vasculitis: Unusual Case Presentation

Abstract

Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune disorder that affects small blood vessels, leading to systemic symptoms and kidney damage due to the production of autoantibodies. A limited number of case reports have explored the potential link between AAV and antiphospholipid syndrome (APS). Catastrophic antiphospholipid syndrome (CAPS), a severe variant of APS, is associated with high mortality and poor prognosis. We present a case of AAV that initially achieved complete remission, only to later develop CAPS. The presence of antiphospholipid antibodies at baseline may increase the risk of CAPS and thrombotic events in AAV patients. A high level of suspicion for this association, along with early detection of these antibodies, could facilitate prompt diagnosis and enable early, appropriate treatment, potentially improving the patient's outcome.

Keywords: acute renal injury; anca associated vasculitis; antiphospholipid antibody; catastrophic antiphospholipid syndrome (caps); thrombocytopenia.

Figure 1. CT chest with contrast.

Arrows: Scattered ground glass opacities and honeycombing. Small pericardial effusion noted. No size significant enlarged mediastinal, hilar or axillary lymph nodes sub-centimeter.

Figure 2. Run off CT with contrast.

A: Coronal cut with almost complete occlusion in the abdominal aorta above the level of renal arteries with minimal collateral seen. B: Axial view with the bilateral iliac arteries is faintly opacified with reconstitution via the abdominal wall collaterals at the femoral level bilaterally. There is a faint hyperdensity asymmetric seen at the right glutes muscle keeping with intramuscular active arterial hematoma.