Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines

@article{Asherson2003CatastrophicAS,
  title={Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines},
  author={Ronald A. Asherson and Ricard Cervera and Philip G. de Groot and Doruk Erkan and Marie Claire Boffa and Jean Charles Piette and Munther A. Khamashta and Yehuda Shoenfeld},
  journal={Lupus},
  year={2003},
  volume={12},
  pages={530 - 534}
}
The term ‘catastrophic’ antiphospholipid syndrome (APS) is used to define an accelerated form of APS resulting in multiorgan failure. Although catastrophicAPS patients represent less than 1% of all patients with APS, they are usually in a life-threatening medical situation that requires high clinical awareness. The careful and open discussion of several proposals by all participants in the pre-symposium workshop on APS consensus, held in Taormina on occasion of the 10th International Congress… Expand
Diagnosing catastrophic antiphospholipid syndrome: the necessity for clinical awareness
TLDR
The catastrophic variant of antiphospholipid syndrome (APS) is the most severe form of the syndrome and is now also referred to as Asherson’s syndrome, thus honoring the author who passed away in 2008. Expand
Treatment of catastrophic antiphospholipid syndrome.
TLDR
Attention to associated diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical at the time of diagnosis, and all patients should be treated with anticoagulants, corticosteroids, and possibly plasma exchange. Expand
Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease
TLDR
The diagnostic challenges and the recently updated diagnostic algorithms for CAPS are summarized providing a ‘step-by-step’ approach for clinicians (and researchers) in the assessment of patients with multiple organ thromboses. Expand
CAPS Registry
TLDR
An international registry of patients with catastrophic APS (CAPS Registry) was created in 2000 by the European Forum on aPL, and the analysis of this registry has allowed the characterization of the clinical and laboratory features of the catastrophic APs as well as the establishment of preliminary criteria for its classification and guidelines for its management. Expand
Clinical and Laboratory Features of the Catastrophic Antiphospholipid Syndrome
TLDR
The majority of patients presented with multiple organ involvement at the time of CAPS, and the combination of pulmonary, cardiac, and renal involvement was most commonly seen. Expand
Diagnosis and management of catastrophic antiphospholipid syndrome
TLDR
The diagnostic challenges and current approaches to the treatment of CAPS are discussed, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. Expand
The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review.
TLDR
Treatment is based on the administration of anticoagulants, corticosteroids, plasma exchange and/or intravenous immunoglobulins in patients with CAPS, and Cyclophosphamide is recommended in those CAPS cases associated to systemic lupus erythematosus. Expand
Task Force Report on Catastrophic Antiphospholipid Syndrome
TLDR
The first-line treatment should include the combination of anticoagulation against thrombosis plus glucocorticoids against the manifestations of SIRS plus plasma exchange and/or intravenous immunoglobulins to remove or block the antiphospholipid antibodies and the cytokines involved in the SirS. Expand
Catastrophic Antiphospholipid Syndrome
TLDR
Although less than 1% of patients with the antiphospholipid syndrome develop the catastrophic variant, its potentially lethal outcome emphasizes its importance in clinical medicine today and it is expected that the periodical analysis of these data will allow us to increase the authors' knowledge of this condition. Expand
Catastrophic antiphospholipid syndrome presenting with sudden renal failure: The lesson lies in vascular lesions.
TLDR
The case presented in this issue of Journal of Nephropatholgy by Mardani et al represents a de novo CAPS, at least on the face of it, and the current complication of CAPS seems most probably to be superimposed on the background of hitherto undiagnosed APS. Expand
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 13 REFERENCES
Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment.
TLDR
It is essential that this syndrome should be diagnosed early and treated aggressively and the combination of high doses of heparin and corticosteroids plus intravenous gamma globulins or plasmapheresis is proposed as the treatment of choice in patients with this severe condition. Expand
Catastrophic Antiphospholipid Syndrome: Clues to the Pathogenesis from a Series of 80 Patients
TLDR
The clinical and serologic features of the largest series of patients with catastrophic APS hitherto reported are described, including 30 new cases from interested physicians in many different countries, as well as a comprehensive literature review of 50 additional recently published case reports with this syndrome. Expand
Catastrophic antiphospholipid syndrome: remission following leg amputation in 2 cases.
TLDR
It is believed that infection plays a significant role in the pathogenesis and amplification of the antiphospholipid syndrome and in certain patients, this association probably is mediated via immune mechanisms, which also enhance the genesis of atherosclerosis. Expand
Catastrophic Antiphospholipid Syndrome: Clinical and Laboratory Features of 50 Patients
TLDR
The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Expand
Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients.
TLDR
An association with SLE, the patient's sex, and the patient’s age at disease onset can modify the disease expression and define specific subsets of APS. Expand
The Catastrophic Antiphospholipid Syndrome
TLDR
The addition of the word “catastrophic” to the term antiphospholipid syndrome (APS) was proposed 20 years ago by Ronald Asherson when he published an editorial describing a group of patients who develop multiple thrombosis in a short period of time. Expand
Thrombotic storm: when thrombosis begets thrombosis.
  • C. Kitchens
  • Medicine
  • The American journal of medicine
  • 1998
TLDR
Six cases are presented demonstrating key features of what may be termed thrombotic storm, with an underlying hypercoagulable disorder and a provocation to initiateThrombosis, which explains its control by anticoagulants. Expand
international consensus statement
TLDR
Around that time, a number of learned scientific articles began to appear in the medical literature, par­ ticularly in the United States, showing just how diffi­ cult it was to prove causation. Expand
...
1
2
...