title: Combined plasmapheresis and immunosuppression as rescue treatment of a patient with catastrophic antiphospholipid syndrome occurring despite anticoagulation: a case report.
abstract: We report the case of a 33-year-old female with known primary antiphospholipid syndrome who, despite full-dose oral anticoagulation, presented with myocardial infarction, acute respiratory distress syndrome, purulent bronchitis, and septic shock. Antiphospholipid antibodies and anti-beta2-glycoprotein-1 titres were markedly elevated. The patient was diagnosed with catastrophic antiphospholipid syndrome and treated with unfractionated intravenous heparin. However, she developed thromboembolism of the right foot and skin marmoration of her extremities during heparin therapy, and therefore plasmapheresis, immunoglobulins, cyclophosphamide, and methylprednisone under a broad spectrum of anti-infective therapy were instituted. This treatment led to a rapid decrease of antiphospholipid antibody and anti-beta2-glycoprotein-1 titres, and the patient's condition gradually improved. Upon discharge from the hospital, pulmonary infiltrates had markedly regressed, and she was feeling well. Given the high mortality of catastrophic antiphospholipid syndrome, this report emphasizes the need for rapid diagnosis and effective multimodal treatment in an intensive care unit setting for these patients.
authors.affiliation: Zentrum für Innere Medizin, Universitätsklinikum, Johann Wolfgang Goethe-Universität, Frankfurt, Germany. skoschmi@caregroup.harvard.edu
authors.forename: Steffen
authors.initials: S
authors.lastname: Koschmieder
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Miesbach
authors.forename: Florian
authors.initials: F
authors.lastname: Fauth
authors.forename: Joerg
authors.initials: J
authors.lastname: Bojunga
authors.forename: Inge
authors.initials: I
authors.lastname: Scharrer
authors.forename: Hans-Reinhard
authors.initials: HR
authors.lastname: Brodt
mesh_terms: D000208:Acute Disease; D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood / Q000187:drug effects; D000925:Anticoagulants / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000276:immunology / Q000628:therapy*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004562:Electrocardiography / Q000379:methods; D005260:Female; D006023:Glycoproteins / Q000276:immunology; D006493:Heparin / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage / Q000627:therapeutic use; D007165:Immunosuppression Therapy / Q000379:methods*; D007275:Injections, Intravenous; D019934:International Normalized Ratio; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D009102:Multiple Organ Failure / Q000209:etiology / Q000276:immunology / Q000628:therapy; D010956:Plasmapheresis / Q000379:methods*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000276:immunology / Q000628:therapy; D013997:Time Factors; D020246:Venous Thrombosis / Q000150:complications / Q000188:drug therapy; D053482:beta 2-Glycoprotein I
chemical_list: D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D006023:Glycoproteins; D007136:Immunoglobulins; D053482:beta 2-Glycoprotein I; D003520:Cyclophosphamide; D006493:Heparin; D008775:Methylprednisolone