CoChat
Sign Up
Cited by 85Systemic Lupus Erythematosus ResearchVasculitis and related conditionsBlood Coagulation and Thrombosis Mechanisms

The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment

Asherson, RA·1998·Lupus
DOI 10.1177/096120339800700214
Open this paper in CoChat — free account

Abstract

A review of 50 patients who manifest features of the catastrophic antiphospholipid syndrome (CAPS) is presented. The clinical features comprise mainly organ involvement as opposed to large-vessel venous or arterial occlusions as is seen in patients with 'simple' antiphospholipid syndrome (APS), which makes the pathogenesis of this unusually rare complication perhaps somewhat different from that of patients with the APS. The mortality of the condition is 50%, most patients dying as a result of a combination of cardiac and respiratory failure. Fifteen patients (28%) suffered from disseminated intravascular coagulation (DIC) as well, which may have contributed to the multiorgan thrombotic microangiopathy characteristic of the CAPS. Although most patients were treated with high-dose i.v. steroids, heparin, cyclophosphamide and other modalities of therapy (such as i.v. globulin), plasmapheresis (advocated for TTP, a similar microangiopathic condition) seemed to offer some benefit (68% recovery). The systemic inflammatory response syndrome (SIRS) was responsible for some of the clinical manifestations such as adult respiratory distress syndrome (ARDS) seen in 15 patients. Pathogenesis of the CAPS seems dependent on a 'two-hit' or even 'three-hit' hypothesis in patients already suffering from a hypercoagulable state. Precipitating factors include infections, trauma (surgical), drug administration and warfarin withdrawal. A recent view that the multiple thrombotic lesions themselves may contribute to further thrombosis ('thrombotic storm') is also discussed.

Integrity signals

Registry

Crossref · 10.1177/096120339800700214

Cites

27 of 27 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • LA positivity is more strongly associated with both arterial and venous thrombosis than either aCL or anti-β2-glycoprotein I antibodies.

    “It is well known that LA positivity is more strongly associated with both arterial and venous thrombosis than either aCL or anti-β2-glycoprotein I antibodies [8, 9].”

    From Antiphospholipid patterns predict risk of thrombosis in systemic lupus erythematosus (2021) · cites this paper as [8]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Related

Authors
Asherson, R.
Affiliations
Rheumatic Diseases Unit, Department of Medicine, University of Cape Town School of Medicine and The Groote Schuur Hospital, Cape Town, South Africa
Venue
Lupus
Publisher
SAGE Publications
Published
1998-02
Volume
7
Issue
2_suppl
Pages
55-62
ISSN
  • 0961-2033 (ISSN-L)
  • 1477-0962
DOI
10.1177/096120339800700214
OpenAlex
W2090382095
Cited by
85
References
27
Citations by year
2026: 1 · 2025: 1 · 2024: 3 · 2023: 4 · 2022: 1 · 2021: 4 · 2020: 2 · 2019: 5 · 2018: 1 · 2017: 4 · 2016: 5 · 2015: 1 · 2014: 1 · 2013: 2 · 2012: 2
FWCI
3.60
Type
journal article
Language
en
Open access
closed
Licence
journals.sagepub.com/page/policies/text-and-data-mining-license (tdm)
Locations
  • Lupus (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
  • Health Sciences › Medicine › Hematology › Blood Coagulation and Thrombosis Mechanisms
Keywords
catastrophic antiphospholipid syndrome, antiphospholipid syndrome, thrombotic microangiopathy, disseminated intravascular coagulation, plasmapheresis, intravenous corticosteroids, heparin, cyclophosphamide, intravenous immunoglobulin, systemic inflammatory response syndrome, acute respiratory distress syndrome, pathogenesis, hypercoagulability, mortality
MeSH terms
Adult, Anti-Inflammatory Agents, Anticoagulants, Autoimmune Diseases, Capillaries, Coma, Cyclophosphamide, Disseminated Intravascular Coagulation, Female, Fibrinolytic Agents, Humans, Hypertension, Immunosuppressive Agents, Inflammation, Ischemia, Male, Multiple Organ Failure, Plasmapheresis, Epoprostenol, Steroids, Streptokinase, Thrombolytic Therapy, Purpura, Thrombocytopenic, Idiopathic, Antiphospholipid Syndrome, Immunoglobulins, Intravenous, Treatment Outcome, Thrombophilia

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review