Rare Thrombotic Diseases Consortium

Catastrophic Antiphospholipid Antibody Syndrome (Thrombotic Storm)

Catastrophic Antiphospholipid Syndrome

The catastrophic antiphospholipid syndrome refers to an accelerated form of the antiphospholipid antibody syndrome (APS) which results in multiorgan failure. Because of the diversity of the clinical and serological presentations that have been reported with this syndrome, a consensus statement on classification criteria was recently proposed by the Catastrophic Antiphospholipid Syndrome Registry Project Group (1). Criteria for the diagnosis of this syndrome include the following:

Evidence of involvement of three or more organs, systems, and/or tissues.
Development of manifestations simultaneously or within a week.
Confirmation by histopathology of small vessel occlusion in at least one organ or tissue.
Laboratory confirmation of the presence of antiphospholipid antibodies.

Patients are considered to have definite catastrophic APS if they fulfill all four diagnostic criteria. Patients are considered to have probable APS if they meet all four criteria but only have involvement of two organs or systems, if they meet all four criteria but have only a single positive determination for antiphospholipid antibodies, or if they meet the first and last criteria, but only one of the two middle criteria.

Precipitating factors in patients who develop catastrophic APS include infections, primarily respiratory, recent surgery or an invasive procedure, and neoplasia (2). The organ systems most commonly involved at the onset include the cardiopulmonary system, primarily characterized by dyspnea and respiratory failure, the central nervous system, and the renal system (2). Thrombotic manifestations include pulmonary embolism and deep venous thrombosis, stroke, myocardial infarction, and intraabdominal thrombotic events. These patients are frequently thrombocytopenic and have a hemolytic anemia, and thrombotic thrombocytopenic purpura must be included in the differential diagnosis (3;4).

Treatment most commonly includes anticoagulation and steroids. Plasmapheresis (5) and intravenous immunoglobulin have also been used. Mortality remains high, however, approaching 50%. Of patients who survive an initial event, however, up to two thirds survived symptom free with anticoagulation therapy for an average follow-up of over five years (6).

Thrombotic Storm

Thrombotic storm refers to a process characterized by progressive thrombosis occurring in patients with an underlying hypercoagulable state (7). Many patients with catastrophic APS would fulfill the criteria for thrombotic storm, but patients with other hypercoagulable states have also been described with thrombotic storm. Treatment of patients with thrombotic storm focuses on the aggressive use of anticoagulant therapy (7).

References

(1) Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette J-C et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12:530-534.

(2) Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G, Petri MA et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine. 2001;80:355-77.

(3) Caramaschi P, Riccetti MM, Pasini AF, Savarin T, Biasi D, Todeschini G. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Report of three cases and review of the literature. Lupus. 1998;7:37-41.

(4) Musio F, Bohen EM, Yuan CM, Welch PG. Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum. 1998;28:1-19.

(5) Neuwelt CM, Daikh DI, Linfoot JA, Pfister DA, Young RG, Webb RL et al. Catastrophic antiphospholipid syndrome. Response to repeated plasmapheresis over three years. Arthritis & Rheumatism. 1997;40:1534-39.

(6) Erkan D, Asherson RA, Espinosa G, Cervera R, Font J, Piette J-C et al. Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. 2003;62:530-533.

(7) Kitchens CS. Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998;104:381-85.

Contact Registry for this Disorder | Find a Study | Advocacy Groups for this Disease