The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review

Cervera, Ricard; Rodríguez‐Pintó, Ignasi; Espinosa, Gerard

doi:10.1016/j.jaut.2018.05.007

Cited by 160 publications

(198 citation statements)

References 80 publications

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“…For recurrent venous thrombosis, unfractionated heparin or low-molecularweight heparin (anti-Fxa, 0.3 to 0.7 U/mL) may be used in combination with warfarin for 3 to 5 days, followed by warfarin alone for 12 months to maintain prothrombin time/international normalized ratio (PT-INR) at 2.5. For recurrent arterial thrombosis, warfarin may be used for 12 months to maintain PT-INR at 3.0 [100,101].…”

Section: Medical Treatmentmentioning

confidence: 99%

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Song¹,

Liu

Zhu

et al. 2020

Military Med Res

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Thrombocytopenia is a common complication of critical care patients. The rates of bleeding events and mortality are also significantly increased in critical care patients with thrombocytopenia. Therefore, the Critical Care Medicine Committee of Chinese People's Liberation Army (PLA) worked with Chinese Society of Laboratory Medicine, Chinese Medical Association to develop this consensus to provide guidance for clinical practice. The consensus includes five sections and 27 items: the definition of thrombocytopenia, etiology and pathophysiology, diagnosis and differential diagnosis, treatment and prevention.

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Section: Medical Treatmentmentioning

confidence: 99%

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Song¹,

Liu

Zhu

et al. 2020

Military Med Res

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“…Catastrophic APS is characterised by multiple thrombi with a systemic inflammatory response and has a high mortality rate . Due to the rarity of this condition and the high mortality rate, there are no controlled trials evaluating optimal treatment.…”

Section: Treatmentmentioning

confidence: 99%

Antiphospholipid syndrome: a clinical review

Mezhov

Segan

Tran

et al. 2019

Medical Journal of Australia

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Summary Antiphospholipid syndrome is characterised by recurrent thrombosis (arterial, venous, microvascular) and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (lupus anticoagulant, anti‐β2‐glycoprotein 1 and anticardiolipin). It can be a primary disease or associated with another autoimmune disease (especially systemic lupus erythematosis). Testing for antiphospholipid antibodies should be considered in patients < 50 years of age with unprovoked venous or arterial thromboembolism, thrombosis at unusual sites or pregnancy complications. The mainstay of treatment is antithrombotic therapy and recommendations vary based on arterial, venous or pregnancy complications. If associated with systemic lupus erythematosis, hydroxychloroquine is recommended both as primary and secondary prophylaxis. Antithrombotic treatment is gold standard and effective.

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“…[1] The prevalence of the catastrophic antiphospholipid syndrome (CAPS) is scarce, less than 1% of all cases of APS, [2] it manifests clinically as disseminated thrombotic disease with multiple organ disfunction and a 37% mortality rate. [3]…”

Section: Introductionmentioning

confidence: 99%

Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

Anjo

Fortuna

Reis

et al. 2019

CRIM

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Catastrophic antiphospholipid syndrome (CAPS), an infrequent autoimmune disease, manifests as a widespread thrombotic disease with multiorgan failure with a 37% mortality rate. We report 39-year-old Caucasian women, medical history of Systemic Lupus Erythematous (SLE) complicated with APS and proliferative glomerulonephritis under dialysis. Patient was admitted at the ICU with septic shock with thoracotomy dehiscence origin. During the ICU stay, extensive lower limbs ulcers with polymorphonuclear infiltrate and steatonecrosis areas in adipose and muscular tissue. Lab studies: anti-β2-glycoprotein-Iantibody was detected. As probable CAPS diagnosis was made, triple therapy with methylprednisolone, immunoglobulin and anticoagulation immediately was started. Serious vasoplegic shock occurs with failure in vasopressor support increment and the patient died. The authors report an APS with rapid progression to catastrophic stage, rare complication that occurs only in 1% of catastrophic APS. In CAPS treatment, a statistical association between triple therapy and the decrease of mortality rate of patients with CAPS was observed, when compared with other combinations or with none of the treatments included in the triple therapy. Although the case had a lethal outcome, the authors emphasize the use of triple therapy in CAPS and the challenge of treating patients with CAPS and infection where the balance between the need for anticoagulation, aggressive immunosuppression and infection control is difficult to manage.

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The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review

Cited by 160 publications

References 80 publications

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Antiphospholipid syndrome: a clinical review

Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

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