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Abstract
Introduction Catastrophic Antiphospholipid Syndrome (CAPS) represents the most severe manifestation of Antiphospholipid Syndrome (APS), affecting approximately 1% of cases, and is associated with a high mortality rate. It is characterized by multiorgan failure and widespread microvascular thrombosis but mainly without vasculitis. This case report aims to contribute to the limited literature available on CAPS cases co-occurring with vasculitis.
Case Report A 36-year-old male diagnosed with primary APS in 2003 due two deep vein thrombosis episodes and testing positive for anti-beta 2 glycoprotein IgG antibodies and lupus anticoagulant. Systemic lupus erythematosus was ruled out, and the patient was treated with warfarin. Amid the COVID-19 pandemic, he lost regular follow-up and discontinued oral anticoagulation for a year.
In 2021, he developed an upper airway infection, tested negative for COVID-19, and exhibited focal neurological symptoms with global aphasia. Within four days, necrosis affected his 4th toe on the right foot and the 2nd and 3rd toes on the left foot. Three days later, he presented with proteinuria (1g/24hrs), a threefold increase in creatinine levels from baseline, and severe thrombocytopenia. A skin biopsy of the right foot dorsum (figure 1) prompted the initiation of treatment: methylprednisolone, intravenous immunoglobulin, and anticoagulation. During follow-up the patient developed pneumonia, sepsis and ultimately, death.
Discussion and Conclusion Secondary events in APS are often triggered by precipitating factors, which can be identified in approximately 55% of patients. In this case, infection and the discontinuation of anticoagulation therapy were identified as contributing factors. A noteworthy characteristic of this patient‘s case is the co-occurrence of CAPS and vasculitis, which is likely associated with multiorgan failure and a higher mortality rate. The scarcity of reported cases combining these phenomena may be attributed to the infrequency of biopsies being performed due to the severity of such patients.
Clinicians should be aware of this entity, given its challenging differential diagnosis with other thrombotic entities, including COVID-19. Timely identification is imperative, as it necessitates urgent intervention as a potential life-saving measure. Despite prompt diagnosis and treatment, this disease continues to exhibit a higher mortality rate compared to other manifestations of APS.
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