title: Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.
abstract: Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.
authors.affiliation: Division of Nephrology, University of Miami, Clinical Research Building, Suite 825, 1120 NW 14th St., Miami, FL 33136, USA.
authors.forename: M L
authors.initials: ML
authors.lastname: Rangel
authors.forename: I
authors.initials: I
authors.lastname: Alghamdi
authors.forename: G
authors.initials: G
authors.lastname: Contreras
authors.forename: T
authors.initials: T
authors.lastname: Harrington
authors.forename: D B
authors.initials: DB
authors.lastname: Thomas
authors.forename: L
authors.initials: L
authors.lastname: Barisoni
authors.forename: D
authors.initials: D
authors.lastname: Andrews
authors.forename: M
authors.initials: M
authors.lastname: Wolf
authors.forename: A
authors.initials: A
authors.lastname: Asif
authors.forename: A
authors.initials: A
authors.lastname: Nayer
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D002388:Catastrophic Illness; D004305:Dose-Response Relationship, Drug; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology* / Q000473:pathology; D006801:Humans; D010951:Plasma Exchange / Q000379:methods; D011650:Pulmonary Alveoli / Q000473:pathology; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology; D013927:Thrombosis / Q000209:etiology* / Q000473:pathology
chemical_list: D000974:Antibodies, Antinuclear; D005938:Glucocorticoids
keywords: CAPS; Catastrophic antiphospholipid syndrome; diffuse alveolar hemorrhage; glucocorticoids; hemorrhage; interstitial hemorrhage; plasma exchange; plasmapheresis; thrombotic microangiopathy