Catastrophic Anti-Phospholipid Syndrome in Systemic Lupus Erythematosus: A Tsunami in the Ocean.

Antiphospholipid anti body syndrome is an autoimmune disorder characterized by arterial or venous thrombosis and/or pregnancy morbidity with foetal deaths or abortions in the presence of antiphospholipid antibodies. Catastrophic antiphospholipid antibody syndrome (CAPS) is an accelerated form of disease with rapid involvement of multiple organ systems often posing a diagnostic challenge. There is a paucity of literature on the presentations of CAPS owing to the orphan nature of the disease.We present three cases of CAPS in patients with systemic lupus erythematosus (SLE).Case 1 - A 22-year-old lady with SLE presented with anasarca, abnormal mentation, anaemia, thrombocytopenia, ANA (IIF) 4+ speckled, positive lupus anticoagulant with pulmonary thromboembolism involving right middle and left lower lobes. While in hospital, she developed infarct in left middle cerebral artery territory; was managed with IVIg, anticoagulation, pulse methylprednisolone and cyclophosphamide. She had a refractory course with cytokine storm syndrome, neutropenic sepsis and eventually succumbed to her illness. Case 2 - A 23-year-old lady presented with a history of oral ulcers, alopecia, photosensitive malar rash, polyarthritis of small joints of hands, Raynaud's phenomenon, intermittent fever with headache and arterial thrombosis resulting in gangrene of the right thumb. She had ANA (IIF) 3+ speckled, raised anti-ds-DNA, positive for lupus anticoagulant; was managed as SLE with hydroxychloroquine and prednisolone. She returned to hospital with generalized tonic-clonic seizures, papilledema but no focal neurological deficit. MRI brain showed superior sagittal sinus thrombosis; was managed with pulse methylprednisolone, anti-coagulation, anti-epileptic drugs, cyclophosphamide and hydroxychloroquine; patient survived. Case 3 - A 47- year-old lady with SLE and Lupus Nephritis Class IV on Euro-Lupus regime presented with paraparesis, cold and clammy left lower limb with absent femoral, popliteal, anterior tibial and dorsalis pedis pulses. CT angiography showed thrombosis infra-renal abdominal aorta and in the left popliteal artery; was positive for high titre β2-GPI IgM. She was managed with pulse methylprednisolone, anti-coagulation, broad-spectrum antibiotics but developed sudden haemodynamic deterioration after the first cycle of plasmapheresis and was switched to IVIg. However, she developed cardiac arrest and succumbed before MR angiography for suspected anterior spinal artery thrombosis and amputation for left lower limb.In our cases, timely diagnoses were made based on a high index of suspicion and were managed with a combination of IVIg, systemic glucocorticoids, plasmapheresis and other supportive measures. However, despite providing the standard of care, we encountered poor outcome in two patients, highlighting the high mortality associated with CAPS.