Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of a series of 280 patients from the “CAPS Registry”
Introduction
The descriptive adjective “catastrophic” was added to the term antiphospholipid syndrome (APS) by Asherson [1] in order to highlight an accelerated form of this syndrome resulting in multi-organ failure. Patients with catastrophic APS (currently also known as Asherson's syndrome) [2] have in common: a) clinical evidence of multiple organ involvement developing over a very short period of time; b) histopathological evidence of multiple small vessel occlusions, and c) laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titer. Furthermore, the majority of the catastrophic episodes are preceded by a precipitating event, mainly infections [3], [4].
Although less than 1% of patients with the APS develop this complication [5], its potentially lethal outcome emphasizes its importance in clinical medicine today. The majority of patients with catastrophic APS end up in Intensive Care Units (ICU) with multi-organ failure and, unless the condition is considered in the differential diagnosis by attending physicians, it may be completely missed, resulting in a disastrous outcome for these patients [6].
The rarity of this syndrome makes it extraordinarily difficult to study in any systematic way. In order to compile all the published case reports as well as newly diagnosed cases from all over the world, an international registry of patients with catastrophic APS (“CAPS Registry”) was created in 2000 by the European Forum on Antiphospholipid Antibodies, a study group devoted to the development of multicenter projects with large populations of APS patients [7]. This registry can be freely consulted through Internet at www.med.ub.es/MIMMUN/FORUM/CAPS.HTM. The initial analysis of this registry allowed the first characterization of the clinical and laboratory features of the catastrophic APS [8], [9], [10], [11], [12], [13] as well as the establishment of preliminary criteria for its classification and guidelines for its management [14], [15].
Currently, the CAPS Registry documents the entire clinical, laboratory and therapeutic data of 280 patients whose data have been fully registered. In the present study, we describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of these 280 patients with catastrophic APS.
Section snippets
Methods
We analyzed the case reports included in the website based international registry of patients with catastrophic APS (CAPS Registry) until September 2008. It contains clinical, laboratory, and therapeutic data on all reported cases of catastrophic APS. The sources of information are the personal communications of the physicians who treated these patients and the periodically computer-search assisted (Medline) of published reports to locate all cases of patients with catastrophic APS. We
General characteristics
The entire series includes 201 (72%) female and 79 (28%) male patients with a mean age of 37 ± 14 years (range, 11–60 years). A total of 129 (46%) patients suffered from primary APS, 112 (40%) from systemic lupus erythematosus (SLE), 14 (5%) from lupus-like disease, and 25 (9%) from other autoimmune diseases. The catastrophic episode was the first manifestation of the APS in 129 (46%) patients.
Precipitating factors and clinical presentation
The main precipitating factors and clinical features of the 280 patients with catastrophic APS from the
Discussion
In 1992, an attempt to single out a seemingly different and important group of patients was adopted by introducing the term catastrophic APS to describe their potentially life-threatening clinical course [1]. From the current review of the 280 patients from the CAPS Registry, certain differences which appear to distinguish this minority group of patients suffering from catastrophic APS from the overwhelming majority of APS patients have been confirmed. The major differences are that large
References (44)
- et al.
V Meeting of the European Forum on Antiphospholipid Antibodies
Autoimmun Rev
(2006) - et al.
Molecular mimicry between host and pathogen: examples from parasites and implication
Immunol Lett
(1999) Common infections, idiotypic dysregulation, autoantibody spread and induction of autoimmune diseases
J Autoimmun
(1996)- et al.
Different types of antiphospholipid antibodies in AIDS: a comparison with syphilis and the antiphospholipid syndrome
Thromb Res
(1999) - et al.
Antiphospholipid antibodies in HIV infection and SLE with or without anti-phospholipid syndrome; comparisons of phospholipid specificity, avidity and reactivity with beta2-GPI
J Autoimmun
(1999) - et al.
Antiphosphatidylserine antibodies in human immunodeficiency virus-1 patients with evidence of T-cell apoptosis and mediate antibody-dependence cellular cytotoxicity
Blood
(1996) - et al.
New developments in viral peptides and APL induction
J Autoimmun
(2000) Tissue factor pathway and the antiphospholipid syndrome
J Autoimmun
(2000)- et al.
Predictive, protective, orphan autoantibodies: the example of anti-phospholipid antibodies
Autoimmun Rev
(2008) - et al.
Antiphospholipid antibodies, antiphospholipid syndrome and infections
Autoimmun Rev
(2008)
Pathogenesis of the antiphospholipid syndrome: an additional example of the mosaic of autoimmunity
J Autoimmun
J Autoimmun
Hughes syndrome (the antiphospholipid syndrome): ten clinical lessons
Autoimmun Rev
Microvascular and microangiopathic antiphospholipid-associated syndromes (“MAPS”): semantic or antisemantic?
Autoimmun Rev
Autoantibody explosion in antiphospholipid syndrome
J Autoimmun
OxLDL/beta2GPI-anti-oxLDL/beta2GPI complex and atherosclerosis in SLE patients
Autoimmun Rev
Insights into atherosclerosis therapy in antiphospholipid syndrome
Autoimmun Rev
The catastrophic antiphospholipid antibody syndrome
J Rheumatol
The catastrophic antiphospholipid syndrome – Asherson's syndrome
Ann Med Intern
Catastrophic antibody syndrome. Clinical and laboratory features of 50 patients
Medicine (Baltimore)
Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients
Medicine (Baltimore)
Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients
Arthritis Rheum
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See complete list of members of the CAPS Registry at the end of the article