Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS Registry”

Berman, Horacio; Rodríguez‐Pintó, Ignasi; Cervera, Ricard; Gregory, Simone; Meis, Ernesto de; Rodrigues, Carlos Ewerton Maia; Aikawa, Nádia E.; Carvalho, Jozélio Freire de; Springer, Janusz; Niedźwiecki, Maciej; Espinosa, Gerard

doi:10.1016/j.autrev.2013.10.004

Cited by 87 publications

(57 citation statements)

References 11 publications

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“…Similar to adults, mortality in this group was high (27%). None of the patients who received only partial treatment with anticoagulants, corticosteroids, plasma exchange, with or without intravenous immunoglobulins (IVIG), survived the catastrophic event 45. Three paediatric CAPS patients reported in a case series received combination treatment (heparinisation with high-dose corticosteroids and IVIG, in one patient additional rituximab).…”

Section: Resultsmentioning

confidence: 99%

European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative

et al. 2017

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Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young adults with rheumatic diseases. Here, we developed evidence-based recommendations for diagnosis and treatment of paediatric APS. Evidence-based recommendations were developed using the European League Against Rheumatism standard operating procedure. Following a detailed systematic review of the literature, a committee of paediatric rheumatologists and representation of paediatric haematology with expertise in paediatric APS developed recommendations. The literature review yielded 1473 articles, of which 15 were valid and relevant. In total, four recommendations for diagnosis and eight for treatment of paediatric APS (including paediatric Catastrophic Antiphospholipid Syndrome) were accepted. Additionally, two recommendations for children born to mothers with APS were accepted. It was agreed that new classification criteria for paediatric APS are necessary, and APS in association with childhood-onset systemic lupus erythematosus should be identified by performing antiphospholipid antibody screening. Treatment recommendations included prevention of thrombotic events, and treatment recommendations for venous and/or arterial thrombotic events. Notably, due to the paucity of studies on paediatric APS, level of evidence and strength of the recommendations is relatively low. The SHARE initiative provides international, evidence-based recommendations for diagnosis and treatment for paediatric APS, facilitating improvement and uniformity of care.

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Section: Resultsmentioning

confidence: 99%

European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative

et al. 2017

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“…Severe cutaneous necrosis was the first manifestation in our patient. In 2/3 of patients with primary CAPS there is a precipitating event, most frequently an infection [11]. In our case, the upper respiratory tract infection may be speculated as the precipitating factor by history.…”

Section: Discussionmentioning

confidence: 84%

“…Pediatric APS registry also investigated single nucleotide polymorphisms of inflammatory mediators for the role of development of APS in pediatric patients which ended without any firm conclusion [14]. Different options of treatment including steroids, anticoagulation, antiaggregants, plasma exchange, intravenous immunoglobulins, cyclophosphamide, rituximab, prostacyclin, ancrod and defibrotide [11,15,16] reflect the vast variety of etiology and organ involvement. Our patient who had received most of these agents completed 1 year follow-up period without any new thrombotic event.…”

Section: Discussionmentioning

confidence: 99%

Primary catastrophic antiphospholipid syndrome in an 8 year-old girl

Barış¹,

Limon²,

Vural³

et al. 2016

MMJ

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Antiphospholipid syndrome (APS) is a disease characterized by recurrent arterial and venous thromboses. Rapidly progressive multiple thromboses leading to multiorgan failure occur in less than 1% of patients and named as catastrophic antiphospholipid syndrome (CAPS). We, hereby, describe an 8 year-old-girl with erythematous skin lesions progressing into purpura fulminans. The patient developed CAPS with the findings including proteinuria, microangiopathic hemolytic anemia, thrombocytopenia, arterial and venous thromboses demonstrated on skin biopsies. She was admitted to intensive care unit and received empirical antibiotics, anticoagulants, antiaggregants, steroids and intravenous immunoglobulins. The diagnosis of APS was confirmed by positive lupus anticoagulants, elevated anti beta-2 glycoprotein IgG and antiphospholipid IgG titers. Moreover, other than MTHFR-A1298C, MTHFR-C677T, factor V H1299R, beta fibrinogen-455 G>A heterozygosity indicating low risk for thrombophilia, no infectious, rheumatological or malignant etiologies were identified. Family history revealed Raynaud's phenomenon in a sister, interstitial lung disease, proteinuria and hematuria in paternal grandmother in addition to lupus anticoagulant positivity in father and 2 elder sisters. Her treatment included debridement of necrotic skin tissue, grefting and local mesenchymal stem cell application to upper thigh and lower leg region following oral azathioprine administration.

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“…Pediatric patients exhibit a similar clinical phenotype as adults, but with a higher prevalence of thrombotic events involving the peripheral vasculature. 8 Almost half of all patients with catastrophic APS, and .85% of pediatric patients, present with the catastrophic event as their first manifestation of APS. 8,9 Patients with catastrophic APS frequently also manifest thrombocytopenia and hemolytic anemia.…”

Section: Introductionmentioning

confidence: 99%

“…8 Almost half of all patients with catastrophic APS, and .85% of pediatric patients, present with the catastrophic event as their first manifestation of APS. 8,9 Patients with catastrophic APS frequently also manifest thrombocytopenia and hemolytic anemia. 10 For patients without a prior diagnosis of APS or systemic lupus erythematosus (SLE), distinguishing catastrophic APS from a non-APS-related disorder can be difficult.…”

Section: Introductionmentioning

confidence: 99%

How I treat catastrophic thrombotic syndromes

Ortel

Erkan

Kitchens

2015

Blood

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Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thrombocytopenic purpura (TTP) or heparin-induced thrombocytopenia (HIT), and Trousseau syndrome, but some patients present with multiple thrombotic events in the absence of associated prothrombotic disorders. Diagnostic workup must rapidly determine which, if any, of these syndromes are present because therapeutic management is driven by the underlying disorder. With the exception of atypical presentations of TTP, which are treated with plasma exchange, anticoagulation is the most important therapeutic intervention in these patients. Effective anticoagulation may require laboratory confirmation with anti–factor Xa levels in patients treated with heparin, especially if the baseline (pretreatment) activated partial thromboplastin time is prolonged. Patients with catastrophic APS also benefit from immunosuppressive therapy and/or plasma exchange, whereas patients with HIT need an alternative anticoagulant to replace heparin. Progressive thrombotic events despite therapeutic anticoagulation may necessitate an alternative therapeutic strategy. If the thrombotic process can be controlled, these patients can recover, but indefinite anticoagulant therapy may be appropriate to prevent recurrent events.

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Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS Registry”

Cited by 87 publications

References 11 publications

European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative

European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative

Primary catastrophic antiphospholipid syndrome in an 8 year-old girl

How I treat catastrophic thrombotic syndromes

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