The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients

Espinosa, Gerard; Shoenfeld, Yehuda; Cervera, Ricard; Cervera, Ricard; Espinosa, Gerard; Rodríguez‐Pintó, Ignasi; Shoenfeld, Yehuda; Erkan, Doruk; Piette, Jean‐Charles; Jacek, Małyszko; Roca, B; Tektonidou, Maria G; Moutsopoulos, Haralampos Μ.; Boffa, J.J.; Chapman, Joab; Stojanovich, L; Veloso, Mariana P; Praprotnik, Sonja; Traub, Beverly; Levy, Ross M.; Daryl, Tan; Boffa, MC; Makatsaria, A.D.; Ruano, M; Allievi, Alberto; You, Wuyi; Khamastha, M; Hughes, Simon; Suso, Juan-Pablo; Pacheco, Jose M.; Boriotti, M F; Dias, Catarina Viegas; Pangtey, Ghan Shyam; Miller, S. F.; Policepatil, Seema M.; Larissa, L; Marjatta, S; Carolyn, S; Noortje, T; Reiner, Katinka; Arteaga, Selene Yañez; Leilani, T; Langsford, David; Niedźwiecki, Maciej; Queyrel, V.; Moroti-Constantinescu, R; Romero, Cristina; Jeremić, K.; Urbano, Ana Paula Signori; Hurtado-García, R.; Das, Ananya; Costédoat-Chalumeau, Nathalie; Yngvar, F; Gómez-Puerta, José A.; Meigs, E de; Smith, J P; Захарова, Елена Хакимовна; Nayer, Ali; Douglas, W.T. Parker; Lyndsey, R; Blanco, Vanessa; Vicent, Carmen Herrero; Natalya, K; Damian, Laura; Valentini, Erica; Giula, B; Moura, Marta Casal; Loperena, O. Araujo; Susan, Y Ritter; Imbert, Geneviève; Almasri, Hanan; Hospach, Toni; Bouraoui, Mouna; Robles, A; Wilson, Holly; Guisado, Pablo; Ruiz, R; Rodríguez, J

doi:10.1093/rheumatology/key082

Cited by 83 publications

(51 citation statements)

References 10 publications

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“…Triple therapy use in CAPS is described in several studies, Rodríguez-Pintó et al study shows a statistical association between triple therapy and the decrease of mortality rate in patients with CAPS, when compared with other combinations or with none of the treatments included in triple therapy. [4] 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.…”

Section: Discussionmentioning

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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Section: Discussionmentioning

confidence: 99%

Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

Anjo

Fortuna

Reis

et al. 2019

CRIM

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“…The acute manifestations of CAPS are usually the result of an acute thrombotic microangiopathy. The differential diagnosis of CAPS in pregnancy and puerperium includes: disseminated intravascular coagulation (DIC), thrombotic thrombocytopaenic purpura (TTP), haemolytic-uraemic syndrome (HUS), heparin-induced thrombocytopaenia (HIT)7 and HELLP (haemolysis, elevated liver enzymes, low platelet count) syndrome, all of which can form part of the manifestations of CAPS. TTP and HUS are both characterised by thrombocytopaenia, macroangiopathic haemolytic anaemia and ischaemic organ damage.…”

Section: Discussionmentioning

confidence: 99%

Catastrophic antiphospholipid syndrome in pregnancy: a life-threatening condition

Collict¹,

Buhagiar

Mercieca

et al. 2019

BMJ Case Rep

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Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially life-threatening variant of the antiphospholipid syndrome which is characterised by multiple small vessel thrombosis which can lead to multiorgan failure. CAPS is a clinical emergency which all clinicians need to be aware of because early diagnosis and treatment may improve maternal and fetal outcome. Here, we report a case of CAPS in pregnancy in a 31-year-old female patient who presented at 28 weeks of gestation. A literature review of CAPS in pregnancy and the puerperium is also included.

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“…Since triple combination therapy with anticoagulants, steroids and TPE and/or IVIG was first proposed in 2001, the prognosis of patients with CAPS has improved significantly. In fact, a recent report from the CAPS Registry confirmed that triple therapy is independently associated with a higher survival rate (mortality rate 28%) (Rodríguez‐Pintó et al , ). Patients who received both TPE and IVIG showed mortality rates similar to those who only received either (27·3% vs. 29%).…”

Section: Tpe In Autoimmune Haematological Disordersmentioning

confidence: 98%

The role of plasma exchange in the management of autoimmune disorders

Zanatta

Cozzi²,

Marson

et al. 2019

Br J Haematol

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Summary Therapeutic plasma exchange (TPE) has been mainly used in the treatment of autoimmune diseases. The main mechanisms of action of TPE include the removal of circulating autoantibodies, immune complexes, complement components, cytokines and adhesion molecules, along with sensitization of antibody‐producing cells to immunosuppressant agents. TPE is useful in autoimmune haematological, renal, rheumatic and neurological diseases, and is recommended for acute disorders, together with relapsed or worsened chronic diseases that are often unresponsive to conventional treatments. The American Society for Apheresis and the British Society of Haematology have published guidelines on the clinical use of apheresis procedures, indicating the different levels of efficacy of TPE. Based on the evidence from current literature and our personal experience, this review discusses the indications and the suggested regimens for TPE in autoimmune haematological and non‐haematological disorders.

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The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients

Abstract: Triple therapy is independently associated with a higher survival rate among patients with CAPS.

Cited by 83 publications

References 10 publications

Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

Catastrophic antiphospholipid syndrome in pregnancy: a life-threatening condition

The role of plasma exchange in the management of autoimmune disorders

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