Catastrophic antiphospholipid syndrome (CAPS) is a rare presentation but it can lead to life threatening complications so high index of clinical suspicion is required. It is categorized as the most severe form of APS and is defined by involvement of three or more organs, systems or tissues, clinical symptoms occurring rapidly within a week, histopathological confirmation of small vessel occlusion in at least one organ or tissue and laboratory confirmation of presence of antiphospholipid antibodies and exclusion of other causes. Although the catastrophic variant presents in less than 1% of all patients diagnosed as APS, its high mortality requires that physicians are aware of this variant. Here, we present a case of 23-year-old male with no known comorbidities, who presented to our department with cough and haemoptysis for 1 month. Chest x ray showed wedge shaped opacification in right middle and lower zone with raised D-dimers of 1186mg / dL (up to 251 mg/ dL). CTPA was done which showed right pulmonary artery embolism along with right atrial filling defect. All coagulation profile was negative with anticardiolipin IgG >280 and strongly positive lupus anticoagulant.
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