BackgroundCatastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening manifestation of antiphospholipid syndrome (APS), which is frequently later diagnosed with high mortality.Case reportA 54-year-old woman with a history of livedo reticularis presented with fever for four days, hematuria for two days, and impaired consciousness and petechiae for one day. Laboratory tests indicated thrombocytopenia, anemia, elevated indirect bilirubin and lactate dehydrogenase levels, as well as elevated free hemoglobin, isolated C3d positivity on direct antiglobulin test, and positive lupus anticoagulant and anti-cardiolipin IgG (aCL-IgG). A diagnosis of autoimmune hemolytic anemia and thrombocytopenia was established, and high-dose methylprednisolone combined with intravenous immunoglobulin therapy was initiated. On day 4, a tricuspid valve thrombus was detected and argatroban was initiated. Although the isolated LA positivity and low-titer aCL-IgG did not fulfill classic APS criteria, the definitive thrombosis led us to strongly suspect quasi-seronegative APS, prompting an increased methylprednisolone dose. On day 6, the platelet count dropped again, and a diagnosis of CAPS was suspected, prompting the administration of methylprednisolone pulse therapy along with plasma exchange. On day 6, the platelet count dropped again, and the patient received methylprednisolone pulse along with plasma exchange treatment. On day 10, leukopenia complicated mixed bacterial infections were developed. On day 11, subarachnoid hemorrhage occurred, leading to discontinuation of anticoagulant. Ultimately, the patient died with the worsened infections. Autopsy revealed extensive thrombosis and severe disseminated mixed infections.ConclusionsEarly manifestations of CAPS may be atypical, so early identification is crucial for treatment. Following steroid pulse therapy, vigilance for fungal infections is essential, with empirical antifungal therapy initiated when necessary.
Zhou et al. (Wed,) studied this question.