The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome: Analysis of a Series of 47 patients

Silvia Bucciarelli ¹, Gerard Espinosa ¹, Ronald A Asherson ², Ricard Cervera ^1*, Gisela Claver ¹, José A Gómez-Puerta ¹, Manuel Ramos-Casals ¹, Miguel Ingelmo ¹ and Josep Font ¹

¹ Hospital Clìnic, Barcelona, Spain
² University of Cape Town, South Africa

^* To whom correspondence should be addressed. E-mail: rcervera{at}clinic.ub.es.

Accepted 14 May 2005

Abstract

Background: The acute respiratory distress syndrome (ARDS) is a non-cardiogenic pulmonary edema characterised by severe hypoxemia refractory to oxygen therapy and diffuse pulmonary infiltrates on chest radiographs. It can be precipitated by a wide variety of serious medical and surgical conditions, including systemic autoimmune diseases. The 'catastrophic' variant of the antiphospholipid syndrome (APS) is an accelerated form of this systemic autoimmune condition resulting in multiorgan failure due to multiple small vessel occlusions.

Objective: To analyze the clinical and laboratory characteristics of catastrophic APS patients with ARDS. Methods: We analyzed the web-site based international registry of patients with catastrophic APS ("CAPS Registry") (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) and selected those cases with ARDS.

Results: Pulmonary involvement was reported in 150 out of 220 (68%) patients with catastrophic APS and 47 (21%) patients were diagnosed as having ARDS. 19 (40%) of these patients died. Pathologic studies were performed in 10 patients and thrombotic microangiopathy was present in 7. There were not differences in age, gender, precipitating factors, clinical manifestations and mortality rate between catastrophic APS patients with and without ARDS.

Conclusions: ARDS is the dominant pulmonary manifestation of catastrophic APS. Therefore, the existence of ARDS in the context of an APS mandates the ruling out of the existence of the catastrophic variant of this syndrome.

Keywords: Acute respiratory distress syndrome, anticardiolipin antibodies, antiphospholipid syndrome, lupus anticoagulant

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