A rare cause of diffuse peripheral and central thromboses

ABSTRACT

Content Catastrophic Antiphospholipid Syndrome (cAPS) is the most severe form of Antiphospholipid Syndrome with a high mortality. We present the case of a fifty year old lady with recurrent thromboses, initially treated with endovascular stents, progressing to require immunosuppression and anticoagulation. A fifty year old lady presented with bilateral leg and abdominal pain. Co-morbidities included Diabetes Mellitus and three miscarriages. Examination revealed pulse deficits in the lower limbs with gangrene on her toe digits. CT Angiogram demonstrated complete thrombus of the Infrarenal Aorta and an Aortic stent was inserted. Two weeks later readmission occurred with leg pain;Doppler revealed a tight stenosis at the distal aortic region which mandated Kissing stent insertion. One month later the patient presented with bilateral leg pain and necrotic right toes;this led to a right forefoot amputation. A triphasic finger colour change was noted with livedo reticularis on her lower limbs;a decision to institute Iloprost and Methylprednisolone ensued. Antiphospholipid antibodies returned showing triple positivity. Management subsequently included the addition of Rituximab, Plasma Exchange, IVIG and Sildenafil. Two months later readmission occurred with lower limb paralysis due to a complete thrombus of the Aortic Bi-Iliac stent- thrombolysed with good result. No further endovascular stenting was advised due to risk of embolic seeding. We have described a case of cAPS on a previously asymptomatic patient who presented with diffuse thrombosis. Our patient suffered from organ infarction, recurrent vascular occlusion over a period of 12 months and previous pulmonary emboli. Livedo reticularis and gangrene were demonstrated. CAPS accounts for less than 1% of APS with a mortality of 50%;this means early specialist input is important. The cAPS registry demonstrates that the majority of patients are female (72%) with a mean age of 37 years, 46% have primary APS, 40% suffer from SLE and 9% from other Autoimmune diseases. This patient does not have a secondary autoimmune condition. The most common clinical features to present before cAPS develops include foetal loss, previous DVT or thrombocytopenia- two of which our patient demonstrated. The prognosis and clinical features of cAPS have been shown to depend on the extent of thrombosis, organs affected and the presence of a systemic immune response. Our patient required aggressive treatment due to accelerating thrombosis as determined by new gangrene, ongoing livedo reticularis and thrombocytopenia. Our case demonstrates the importance of keeping a high index of suspicion for cAPS as up to 46% will have this as their first presenting feature of APS. The diagnosis was secure with high titre of IgG anticardiolipin antibody, anti-Beta-2 glycoprotein 1 antibodies and Lupus Anticoagulant. Patients may present to surgical specialties in view of peripheral vascular signs and symptoms. It would be appropriate to identify patients with APS early to prevent multiple surgeries or endovascular stents, as they are frequently not successful. This case highlights the need for discussion and education within the multi-disciplinary setting for patients with APS. Finally, the risk of immunosuppression for patients who have received Rituximab can persist for up to 12 months following treatment. This patient's risk stratification was high in view of COVID-19 and she was advised to shield until government guidelines ended.