Catastrophic Antiphospholipid Syndrome: Management Challenges and Lessons Learnt in the Third World Set-Up: Case Report

Abstract

Background: Antiphospholipid Syndrome (APS) is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss. Catastrophic Antiphospholipid Syndrome (CAPS) is a very severe variant of the classic APS. It is characterized by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titre. Although patients with catastrophic APS represent less than 1% of all patients with APS, this is usually a life-threatening condition. The majority of patients with catastrophic APS end up in intensive care units with multiorgan failure. Making the diagnosis is challenging and can be missed. Unless the condition is considered in the differential diagnosis by attending physicians, it may be completely missed, resulting in a disastrous outcome. Catastrophic APS develops rapidly and can result in death of up to 30-50% of cases.

Case presentation: A nineteen year old nulliparous lady diagnosed with Systemic Lupus Erythematosus (SLE) four months prior to admission with no prior history of thrombo-embolic events presented at the accident and emergency department with one day history of fevers and convulsions. This was associated with history of progressively worsening memory loss and confusion associated with incoordination of hands. She also reported to have had a productive cough of 3 months which was episodic. The patient was admitted and developed multiple organ failure from lungs, heart and the kidney during treatment in hospital attributed to this disease. She succumbed during treatment.

Keywords: Antiphospholipid Syndrome, Catastrophic Antiphospholipid Syndrome, Arterial and Venous Thrombosis, Clinical Features, Diagnosis, Management