Aortitis is an under-recognized manifestation of antiphospholipid syndrome: A case report and literature review

Abstract

Aortitis is a classic manifestation of large vessel vasculitis. Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is an acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis. Patients with APS may also suffer from various underlying diseases, most frequently systemic lupus erythematosus (SLE). Catastrophic antiphospholipid syndrome (CAPS) is a rare but serious complication of APS characterized by failure of several organs due to diffuse microcirculatory thrombi. Its main manifestations involve the kidneys, lungs, heart and central nervous system, and require early diagnosis and rapid therapeutic management. While APS can affect virtually any blood vessel, aortitis is not a known symptom of APS. We report the case of a 36-year-old patient with APS and SLE who presented with CAPS during pregnancy, with no concomitant SLE flare. The first manifestation of CAPS was aortitis, preceding renal, cardiac and haematological manifestations. The outcome was favourable with combined treatment including corticosteroids, anticoagulants, plasma exchange and rituximab. We then carried out a literature search for papers describing the presence of aortitis in APS and/or SLE. In the cases of aortic involvement identified in the literature, including another case of CAPS, the occurrence of aortitis in SLE, often associated with the presence of antiphospholipid antibodies/APS, suggests that aortitis should be considered as an under-recognized manifestation and potential non-criterion feature of APS.

Catastrophic antiphospholipid syndrome: challenging case and importance of multidisciplinary evaluation and management

“…While large vessel vasculitis was considered, given the finding of aortitis, involvement of the glomerulus made this less likely. Of note, aortitis is a rare finding in CAPS, with only two cases having previously been reported in the literature 16 . Nevertheless, few APLS patients would've undergone extensive whole‐body imaging like our case did to identify aortitis, which itself is often a manifestation or autoimmune and rheumatological condition so is likely associated with CAPS in our case.…”

“…Of note, aortitis is a rare finding in CAPS, with only two cases having previously been reported in the literature. 16 Nevertheless, few APLS patients would've undergone extensive whole‐body imaging like our case did to identify aortitis, which itself is often a manifestation or autoimmune and rheumatological condition so is likely associated with CAPS in our case. After thorough multidisciplinary case review, consensus was that CAPS was the most likely unifying diagnosis.…”

Unifying Diagnosis Explaining Multiorgan Clinical Manifestations: The Unique Case of Antiphospholipid Syndrome Leading to MINOCA

“…In this case, all clinical manifestations can be related to an underlying antiphospholipid syndrome: (1) aortitis secondary to vascular inflammation from autoimmune activation 2 ; (2) hemorrhagic adrenal infarction secondary to adrenal vein thrombosis as already described in other patients 3 ; and (3) the acute myocardial infarction without obstructive coronary arteries. 4…”

Antiphospholipid antibody-related hepatic vasculitis in a juvenile after non-severe COVID-19: a case report and literature review

“…Additionally, mesenteric vasculitis is considered to be one of aPLrelated microvascular manifestations as well (95). Sporadic cases with authentic associated vasculitic manifestations have also been reported in cerebral (96), renal (97), aortic (98), and cutaneous (99) vasculature, and no local thrombus or microthrombus was noted in these inflammatory lesions.…”