title: Catastrophic antiphospholipid syndrome.
abstract: Antiphospholipid syndrome (APS) is an autoimmune disease with clinical manifestations of arterial and venous thrombosis, concomitant fetal loss and the presence of antiphospholipid antibodies (APLA). This report focuses on the challenges of optimal treatment involving plasma exchange and intravenous human immunoglobulin infusions that is administered in patients with catastrophic APS (CAPS). CAPS is a rare variant of APS defined as acute failure of at least three tissues, organs or systems caused predominantly by small vessel thrombosis confirmed by histopathologic evidence. CAPS develops rapidly and leads to death in 50% of cases. We present the case of a 39-year-old male patient with APS with worsening renal function. Positive lupus anticoagulant, markedly high concentrations of anticardiolipin and anti-beta 2-glikoprotein I antibodies have been observed. According to the criteria introduced by Asherson, a catastrophic form of APS was diagnosed and the patient had been treated with low-molecular-weight heparin, glucocorticosteroids, and plasmapheresis. In order to maintain clinical improvement, the patient was given human immunoglobulins i.v. (1 g/kg body weight). After the procedure, gradual clinical improvement was observed and renal function remained stable (serum creatinine level of 1.5 mg/dl).
authors.affiliation: Department of Transplantation Medicine and Nephrology, Transplantation Institute, Medical University of Warsaw, Warszawa, Poland. afurmanczyk@gmail.com
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Furmańczyk
authors.forename: Ewa
authors.initials: E
authors.lastname: Komuda-Leszek
authors.forename: Wioletta
authors.initials: W
authors.lastname: Gadomska
authors.forename: Jerzy
authors.initials: J
authors.lastname: Windyga
authors.forename: Magdalena
authors.initials: M
authors.lastname: Durlik
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000628:therapy; D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D002388:Catastrophic Illness; D003131:Combined Modality Therapy; D005938:Glucocorticoids / Q000008:administration & dosage*; D006495:Heparin, Low-Molecular-Weight / Q000008:administration & dosage*; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D010956:Plasmapheresis / Q000379:methods*; D016896:Treatment Outcome; D053482:beta 2-Glycoprotein I / Q000097:blood
chemical_list: D017153:Antibodies, Anticardiolipin; D005938:Glucocorticoids; D006495:Heparin, Low-Molecular-Weight; D016682:Lupus Coagulation Inhibitor; D053482:beta 2-Glycoprotein I