No abstract

BACKGROUNDCatastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of APS, affecting approximately 1% of patients. The majority of CAPS events (65%) are triggered by a precipitating factor. We herein report the case of a patient who developed CAPS after receiving mRNA COVID-19 vaccine. CASE REPORTA 40-year-old male patient presented with sudden dysarthria, dyspnea and hypoxemia, requiring hospitalization. The symptoms started 72 h after receiving the second dose of BNT162b2 mRNA vaccine against SARS-CoV-2. He had a past history of several recurrent unprovoked deep vein thrombosis in the upper and lower limbs and was treated with warfarin followed by rivaroxaban, prescribed by his clinician at that time. He had a negative neoplastic screening and no previous investigation for APS. He was transferred to our facility for further inpatient diagnostic workup and treatment. On admission, the patient had livedo reticularis and signs of small and medium caliber vasculopathy in the lower limbs. Laboratory workup revealed thrombocytopenia (39,980 per mm³) and elevated creatinine (1.9 mg/dL). Urinalysis showed hematuria, protein ++/4+ and granular casts. SARS-CoV-2 infection was ruled out after two negative antigen rapid tests and two negative RT-PCR. Antiphospholipid antibodies (aPL) were tested and retrieved positive results for IgG anticardiolipin (aCL) (97.8 GPL) and IgG anti-beta-2 glycoprotein I (aβ2GP1) (177 IU/mL). Cranial computed tomography (CT) was compatible with multiple acute/subacute ischemic stroke events, and transthoracic echocardiogram revealed signs of right ventricle dysfunction and intracardiac thrombus measuring 2.3 × 2.1 cm in right atrium. Chest CT angiography revealed multiple areas suggestive of pulmonary infarction, without identification of emboli. A probable CAPS diagnosis was made based on the rapidly progressive multiple thrombotic events and positivity to aPL, and the patient was treated with enoxaparin and intravenous methylprednisolone pulses. He promptly evolved with significant improvement in renal function and thrombocytopenia and with resolution of dysarthria, dyspnea, and hypoxemia. The hematology team decided to postpone plasmapheresis, since it was not readily available. Renal biopsy was not feasible due to thrombocytopenia and urgent need of anticoagulation. The patient was discharged without neurological deficits and with improved renal function. He is being treated with warfarin as an outpatient in our APS clinic. Confirmatory aPL tests revealed triple positivity to aPL. CONCLUSIONThis report shows that CAPS may be triggered by mRNA vaccine against SARS-CoV-2. Physicians should be aware of this potential complication in patients with antiphospholipid syndrome receiving these type of vaccine.