Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients

Ronald A. Asherson, Ricard Cervera, Jean Charles Piette, Yehuda Shoenfeld, Gerard Espinosa, Michelle Petri, Eugene Lim, Tang Ching Lau, Anagha Gurjal, Anna Jedryka-GóRal, Hanna Chwalinska-Sadowska, Robin J. Dibner, Jorge Rojas-RodríGuez, Mario GarcíA-Carrasco, John T. Grandone, Ann L. Parke, Paulo Barbosa, Carlos Vasconcelos, Manuel Ramos-Casals, Josep FontMiguel Ingelmo

Research output: Contribution to journalArticle

Abstract

We analyzed the clinical and laboratory characteristics of 80 patients with catastrophic antiphospholipid syndrome (APS) (30 new cases and 50 from a MEDLINE computer-assisted review of the literature from 1996 through 2000). Thirty-seven suffered from defined systemic lupus erythematosus (SLE), 33 from primary APS, 5 from "lupus-like" syndrome, 2 from systemic sclerosis, and 1 each had ulcerative colitis, rheumatoid arthritis and polychondritis. Mean age of patients in this series was 37 years (range, 7-67 yr). The most frequent precipitating factors were infections (35%), trauma and invasive procedures (13%), tumors (8%), and withdrawal of anticoagulants or low international normalized ratio (8%). The presentation of the acute multiorgan failure was usually complex, involving multiple organs simultaneously or in a very short time. The majority of patients manifested microangiopathy - that is, occlusive vascular disease affecting predominantly small vessels or organs, particularly kidney, lungs, brain, heart, and liver - with a minority of patients experiencing large vessel occlusions. Thrombocytopenia was reported in 48 (60%) patients, hemolytic anemia in 31 (39%), disseminated intravascular coagulation in 15 (19%), and schistocytes in 7 (9%). The following antibodies were detected: anticardiolipin antibodies (98%), lupus anticoagulant (68%), anti-dsDNA (53% of patients with SLE), antinuclear antibodies (63%), and anti-ENA (17%). Anticoagulation was used in 84% of the episodes, steroids in 80%, cyclophosphamide in 35%, plasmapheresis in 20%, and intravenous gammaglobulins in 19%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 38 of the 80 (48%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Recovery occurred in 62% of episodes treated with anticoagulation versus 23% in those who were not (p = 0.014), and in 64% of episodes treated with anticoagulation plus steroids versus 38% in those who were not (p = 0.053). No other treatment combination was associated with a significant improvement in outcome.

Original languageEnglish (US)
Pages (from-to)355-377
Number of pages23
JournalMedicine
Volume80
Issue number6
DOIs
StatePublished - 2001

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Antiphospholipid Syndrome
Systemic Lupus Erythematosus
Steroids
Precipitating Factors
Lupus Coagulation Inhibitor
Anticardiolipin Antibodies
International Normalized Ratio
Plasmapheresis
Disseminated Intravascular Coagulation
Systemic Scleroderma
Antinuclear Antibodies
Hemolytic Anemia
Adult Respiratory Distress Syndrome
Vascular Diseases
Ulcerative Colitis
MEDLINE
Respiratory Insufficiency
Thrombocytopenia
Cyclophosphamide
Anticoagulants

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Asherson, R. A., Cervera, R., Piette, J. C., Shoenfeld, Y., Espinosa, G., Petri, M., ... Ingelmo, M. (2001). Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients. Medicine, 80(6), 355-377. https://doi.org/10.1097/00005792-200111000-00002

Catastrophic antiphospholipid syndrome : Clues to the pathogenesis from a series of 80 patients. / Asherson, Ronald A.; Cervera, Ricard; Piette, Jean Charles; Shoenfeld, Yehuda; Espinosa, Gerard; Petri, Michelle; Lim, Eugene; Lau, Tang Ching; Gurjal, Anagha; Jedryka-GóRal, Anna; Chwalinska-Sadowska, Hanna; Dibner, Robin J.; Rojas-RodríGuez, Jorge; GarcíA-Carrasco, Mario; Grandone, John T.; Parke, Ann L.; Barbosa, Paulo; Vasconcelos, Carlos; Ramos-Casals, Manuel; Font, Josep; Ingelmo, Miguel.

In: Medicine, Vol. 80, No. 6, 2001, p. 355-377.

Research output: Contribution to journalArticle

Asherson, RA, Cervera, R, Piette, JC, Shoenfeld, Y, Espinosa, G, Petri, M, Lim, E, Lau, TC, Gurjal, A, Jedryka-GóRal, A, Chwalinska-Sadowska, H, Dibner, RJ, Rojas-RodríGuez, J, GarcíA-Carrasco, M, Grandone, JT, Parke, AL, Barbosa, P, Vasconcelos, C, Ramos-Casals, M, Font, J & Ingelmo, M 2001, 'Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients', Medicine, vol. 80, no. 6, pp. 355-377. https://doi.org/10.1097/00005792-200111000-00002
Asherson, Ronald A. ; Cervera, Ricard ; Piette, Jean Charles ; Shoenfeld, Yehuda ; Espinosa, Gerard ; Petri, Michelle ; Lim, Eugene ; Lau, Tang Ching ; Gurjal, Anagha ; Jedryka-GóRal, Anna ; Chwalinska-Sadowska, Hanna ; Dibner, Robin J. ; Rojas-RodríGuez, Jorge ; GarcíA-Carrasco, Mario ; Grandone, John T. ; Parke, Ann L. ; Barbosa, Paulo ; Vasconcelos, Carlos ; Ramos-Casals, Manuel ; Font, Josep ; Ingelmo, Miguel. / Catastrophic antiphospholipid syndrome : Clues to the pathogenesis from a series of 80 patients. In: Medicine. 2001 ; Vol. 80, No. 6. pp. 355-377.
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T2 - Clues to the pathogenesis from a series of 80 patients

AU - Asherson, Ronald A.

AU - Cervera, Ricard

AU - Piette, Jean Charles

AU - Shoenfeld, Yehuda

AU - Espinosa, Gerard

AU - Petri, Michelle

AU - Lim, Eugene

AU - Lau, Tang Ching

AU - Gurjal, Anagha

AU - Jedryka-GóRal, Anna

AU - Chwalinska-Sadowska, Hanna

AU - Dibner, Robin J.

AU - Rojas-RodríGuez, Jorge

AU - GarcíA-Carrasco, Mario

AU - Grandone, John T.

AU - Parke, Ann L.

AU - Barbosa, Paulo

AU - Vasconcelos, Carlos

AU - Ramos-Casals, Manuel

AU - Font, Josep

AU - Ingelmo, Miguel

PY - 2001

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N2 - We analyzed the clinical and laboratory characteristics of 80 patients with catastrophic antiphospholipid syndrome (APS) (30 new cases and 50 from a MEDLINE computer-assisted review of the literature from 1996 through 2000). Thirty-seven suffered from defined systemic lupus erythematosus (SLE), 33 from primary APS, 5 from "lupus-like" syndrome, 2 from systemic sclerosis, and 1 each had ulcerative colitis, rheumatoid arthritis and polychondritis. Mean age of patients in this series was 37 years (range, 7-67 yr). The most frequent precipitating factors were infections (35%), trauma and invasive procedures (13%), tumors (8%), and withdrawal of anticoagulants or low international normalized ratio (8%). The presentation of the acute multiorgan failure was usually complex, involving multiple organs simultaneously or in a very short time. The majority of patients manifested microangiopathy - that is, occlusive vascular disease affecting predominantly small vessels or organs, particularly kidney, lungs, brain, heart, and liver - with a minority of patients experiencing large vessel occlusions. Thrombocytopenia was reported in 48 (60%) patients, hemolytic anemia in 31 (39%), disseminated intravascular coagulation in 15 (19%), and schistocytes in 7 (9%). The following antibodies were detected: anticardiolipin antibodies (98%), lupus anticoagulant (68%), anti-dsDNA (53% of patients with SLE), antinuclear antibodies (63%), and anti-ENA (17%). Anticoagulation was used in 84% of the episodes, steroids in 80%, cyclophosphamide in 35%, plasmapheresis in 20%, and intravenous gammaglobulins in 19%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 38 of the 80 (48%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Recovery occurred in 62% of episodes treated with anticoagulation versus 23% in those who were not (p = 0.014), and in 64% of episodes treated with anticoagulation plus steroids versus 38% in those who were not (p = 0.053). No other treatment combination was associated with a significant improvement in outcome.

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