Raras
Buscar doenças, sintomas, genes...
Síndrome antifosfolípido catastrófico
ORPHA:464343CID-10 · D68.6CID-11 · 4A45DOENÇA RARA

Doença autoimune sistémica rara caracterizada por início agudo de tromboses potencialmente fatais em três ou mais órgãos, simultaneamente ou em menos de uma semana, na presença de anticorpos antifosfolipídicos séricos (como anticoagulante lúpico, anticorpos anticardiolipina e anticorpos anti‐beta‐glicoproteína 1) e com confirmação histopatológica de oclusão de pequenos vasos em pelo menos um órgão afetado. A situação geralmente é precipitada por infeção, trauma ou cirurgia.

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Introdução

O que você precisa saber de cara

📋

A Síndrome Antifosfolipíde Catastrófica (CAPS), também conhecida como síndrome de Asherson, é uma doença autoimune rara na qual a coagulação intravascular generalizada causa falência de múltiplos órgãos. A síndrome é causada por anticorpos antifosfolípides que têm como alvo um grupo de proteínas no corpo que estão associadas a fosfolipídios. Esses anticorpos ativam células endoteliais, plaquetas e células imunes, causando, em última análise, uma grande resposta imune inflamatória e coagulação generalizada. A CAPS foi descrita pela primeira vez por Ronald Asherson em 1992. A síndrome exibe microangiopatia trombótica, tromboses de múltiplos órgãos e, em alguns casos, necrose tecidual, sendo considerada uma variante extrema ou catastrófica da síndrome antifosfolípide.

Publicações científicas
699 artigos
Último publicado: 2026 Mar

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D68.6
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
9 sintomas
❤️
Coração
7 sintomas
🧠
Neurológico
3 sintomas
🫁
Pulmão
2 sintomas
🧬
Pele e cabelo
1 sintomas
🫃
Digestivo
1 sintomas

+ 26 sintomas em outras categorias

Características mais comuns

100%prev.
Positividade do anticorpo antifosfolipídeo
90%prev.
Trombose anormal
Muito frequente (99-80%)
55%prev.
Trombose venosa profunda
Frequente (79-30%)
55%prev.
Anemia hemolítica Coombs-positiva
Frequente (79-30%)
55%prev.
Trombose arterial
Frequente (79-30%)
55%prev.
Anormalidade do nível sérico de citocinas
Frequente (79-30%)
52sintomas
Muito frequente (2)
Frequente (11)
Ocasional (26)
Muito raro (13)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 52 características clínicas mais associadas, ordenadas por frequência.

Positividade do anticorpo antifosfolipídeoAntiphospholipid antibody positivity
Muito frequente100%
Trombose anormalAbnormal thrombosis
Muito frequente (99-80%)90%
Trombose venosa profundaDeep venous thrombosis
Frequente (79-30%)55%
Anemia hemolítica Coombs-positivaCoombs-positive hemolytic anemia
Frequente (79-30%)55%
Trombose arterialArterial thrombosis
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico699PubMed
Últimos 10 anos200publicações
Pico202443 papers
Linha do tempo
2026Hoje · 2026🧪 2009Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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2Fase 21
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome antifosfolípido catastrófico

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

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Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

2 ensaios clínicos encontrados.

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Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
361 papers (10 anos)
#1

Catastrophic Antiphospholipid Syndrome: A Rare but Life-Threatening Thrombotic Storm-A Case Report and Literature Review.

Case reports in rheumatology2026

Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening variant of antiphospholipid syndrome (APS) characterized by rapid, widespread thrombosis leading to multiorgan failure. Affecting less than 1% of APS patients, CAPS is associated with a high mortality rate of 30%-50%, necessitating prompt diagnosis and aggressive treatment. The mainstay of management includes anticoagulation, high-dose glucocorticoids, and plasma exchange or intravenous immunoglobulins, with biologic therapies such as rituximab and eculizumab reserved for refractory cases. We report a case of a 60-year-old male with a history of triple-antibody-positive APS complicated by recurrent diffuse alveolar hemorrhage (DAH), adrenal hemorrhage, chronic kidney disease, and superficial vein thrombosis. His condition progressed to CAPS approximately 5 years prior with a course complicated by heparin-induced thrombocytopenia. His condition stabilized with high-dose corticosteroids and rituximab therapy with sustained symptomatic improvement after 10 months of rituximab. This case highlights the complexity of CAPS diagnosis and management, in the context of DAH, emphasizing the importance of early recognition, multidisciplinary care, and individualized treatment strategies. Our patient's prolonged disease stabilization with rituximab underscores its potential role in long-term CAPS management. Further research is needed to refine treatment protocols and improve outcomes for this rare but life-threatening condition.

#2

Imaging features of catastrophic antiphospholipid syndrome : Massive saddle pulmonary embolism with subsequent complete resolution.

Radiology case reports2026 Apr

Catastrophic antiphospholipid syndrome (CAPS) is a rare thrombotic disorder that can present with distinctive imaging features. Recognition of these imaging patterns is critical for timely diagnosis and management. We report the imaging findings in a 27-year-old woman presenting with acute dyspnea. Computed tomography pulmonary angiography (CTPA) revealed a large saddle embolus at the main pulmonary artery bifurcation with bilateral extension, accompanied by right ventricular dysfunction on echocardiography. The constellation of imaging findings-massive central thrombus burden, right heart dysfunction indices (RV/LV ratio 1.3, TAPSE 13mm), and absence of deep venous thrombosis-prompted consideration of an underlying thrombophilic disorder. Subsequent workup confirmed CAPS with multiorgan involvement. Following treatment with thrombolysis, corticosteroids, and plasmapheresis, follow-up CTPA at three months demonstrated complete thrombus resolution with normalization of cardiac parameters. This case illustrates the characteristic imaging appearance of CAPS-related massive pulmonary embolism and emphasizes the need to recognize high-risk features that suggest underlying systemic thrombophilia. Multimodality imaging is essential for diagnosis, risk stratification, treatment monitoring, and documenting complete resolution.

#3

Catastrophic antiphospholipid syndrome with eosinophilia mimicking hypereosinophilic syndromes with disseminated intravascular coagulation: a case report.

Journal of medical case reports2026 Feb 03

Catastrophic antiphospholipid syndrome is a rare variant of antiphospholipid syndrome where the presence of antiphospholipid antibodies and systemic inflammation leads to the rapid onset of multifocal thrombosis. Eosinophilia is known to accompany conditions such as allergy, parasite infection, malignancy, or autoimmune diseases; however, catastrophic antiphospholipid syndrome with eosinophilia has not been reported and its clinical influence remains unknown. We describe the first case of probable catastrophic antiphospholipid syndrome with eosinophilia, which mimicked eosinophil-associated disorders such as hypereosinophilic syndromes or eosinophilic granulomatosis with polyangiitis with disseminated intravascular coagulation, adding a new aspect of differential diagnosis of eosinophilia. A 46-year-old previously healthy Japanese man presenting with fever, abdominal pain, and skin lesions with pruritus showed marked eosinophilia, thrombocytopenia, and coagulopathy. A dynamic contrast-enhanced computed tomography scan of the abdomen showed some nonenhancing lesions in both lobes of the liver and the portal vein thrombosis. Upper gastrointestinal endoscopy showed gastric erosions, and lower gastrointestinal endoscopy revealed transverse colon and cecum ulcers. Common causes of eosinophilia including allergy, infection, and medication were not detected. He was initially suspected with hypereosinophilic syndromes or vasculitis such as eosinophilic granulomatosis with polyangiitis complicated by disseminated intravascular coagulation, and was treated with prednisone and thrombomodulin from hospital day 4 after bone marrow examination and the biopsies of the skin, stomach, and colon; however, these examinations excluded neoplastic hypereosinophilic syndromes and vasculitis. Later examination revealed positive antiphospholipid antibodies including lupus anticoagulant, anticardiolipin antibodies immunoglobulin G, and anticardiolipin β2-glycoprotein 1 complex antibodies. He was complicated by colonic perforation and bilateral adrenal hemorrhage on day 10. Histopathology of the resected colon and liver biopsy confirmed arterial and venous small-vessel thrombosis and microthrombi, leading to the diagnosis of probable catastrophic antiphospholipid syndrome. He was successfully treated with plasma exchange and rituximab. He has been in remission of catastrophic antiphospholipid syndrome for 4 years, and his antiphospholipid antibodies have been negative post-rituximab treatment. His eosinophil count has been between normal to slightly increased, possibly due to the chronic adrenal insufficiency. Our case shows that eosinophilia can accompany catastrophic antiphospholipid syndrome, and this can mimic eosinophil-associated disorders with disseminated intravascular coagulation. Identifying antiphospholipid antibodies is important for differential diagnosis when treating unexplained eosinophilia, thrombocytopenia, and thrombosis.

#4

Navigating clinical overlap: A case of polyarteritis nodosa in the setting of antiphospholipid syndrome.

Modern rheumatology case reports2026 Jan 06

Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis of medium-sized arteries, often challenging to differentiate from other autoimmune and thrombotic disorders due to overlapping clinical features. This case-based literature review describes a 56-year-old male with a prior diagnosis of catastrophic antiphospholipid syndrome who presented with digital ischemia, myalgias, and a progressive cutaneous rash. Despite initial treatment with anticoagulation, symptoms persisted, and a skin biopsy ultimately revealed medium-vessel vasculitis consistent with PAN. Muscle biopsy was inconclusive, underscoring the difficulty of diagnosis in the setting of coexisting vasculopathies. This case illustrates the diagnostic complexity at the intersection of inflammatory and thrombotic syndromes and highlights the potential for concurrent or misclassified disease. A review of literature reveals rare but documented associations between PAN and antiphospholipid syndrome, yet no diagnostic tool for differentiation exists. Early recognition and accurate differentiation are critical to optimise outcomes in such overlapping syndromes. Future studies should evaluate a possible differentiation score or explore further diagnostics to help differentiate these inflammatory and thrombotic syndromes to better recognise and treat these conditions.

#5

Catastrophic antiphospholipid syndrome: Clinical characteristics and outcomes in a non-caucasian population - A five-year experience from a single centre.

Lupus2026 Jan 28

BackgroundCatastrophic Antiphospholipid Syndrome (CAPS) is a rare but highly severe manifestation of antiphospholipid syndrome (APS), occurring in fewer than 1% of APS patients, but is associated with mortality of around 25-37%, despite treatment. It is characterized by rapid onset of widespread thrombotic events leading to microangiopathy, intravascular thrombosis with multiorgan failure, involving three or more organs within one week. Most published data originate from European or multinational registries like the CAPS Registry. However, data on CAPS in Latin American populations, who may differ in genetic background, socioeconomic status, and healthcare access, remain scarce.MethodsThe objective was to describe the clinical characteristics, treatment strategies, and outcomes of patients diagnosed with CAPS at a tertiary referral center in Mexico City over 5 years. We conducted a retrospective cohort study of patients who fulfilled the CAPS classification criteria between January 2019 and December 2023.ResultsNineteen patients were included (74% female; median age 38.4 years). Six had primary APS, while 11 had concomitant systemic lupus erythematosus (SLE). Based on the 2019 EULAR aPL titers classification, seven patients were classified as low-risk and 12 as high-risk. In 47%, CAPS was the first manifestation of APS. Precipitating factors were identified in 94.7% of cases, most commonly infections and anticoagulant withdrawal. The most frequently affected were the arterial and venous circulation (74% and 68%, respectively), hematologic manifestations (47%), kidneys (42%), lungs (42%), and the heart (32%). Treatment strategies included anticoagulation (84%), corticosteroids (79%), plasma exchange (79%), triple therapy (74%), and, in selected cases, immunosuppressants. Overall mortality was 42%; among survivors, 75% achieved full recovery with no relapses.ConclusionThis study represents one of the largest single-center series of CAPS in a non-Caucasian population. Our findings reveal a predominance of high-risk, secondary APS-mainly SLE-associated-with thrombocytopenia. Nearly half presented with CAPS as the initial APS manifestation. Limited access to full triple therapy significantly impacted mortality, which decreased from 100% with partial therapy to 21% with complete regimens. Early recognition, prompt management, and multidisciplinary care were associated with favourable outcomes, underscoring the need for adaptable therapeutic strategies in resource-limited settings.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC502 artigos no totalmostrando 198

2026

Catastrophic Antiphospholipid Syndrome: A Rare but Life-Threatening Thrombotic Storm-A Case Report and Literature Review.

Case reports in rheumatology
2026

Catastrophic antiphospholipid syndrome in a 39-year-old woman.

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2026

Imaging features of catastrophic antiphospholipid syndrome : Massive saddle pulmonary embolism with subsequent complete resolution.

Radiology case reports
2026

Catastrophic antiphospholipid syndrome with eosinophilia mimicking hypereosinophilic syndromes with disseminated intravascular coagulation: a case report.

Journal of medical case reports
2026

Navigating clinical overlap: A case of polyarteritis nodosa in the setting of antiphospholipid syndrome.

Modern rheumatology case reports
2026

Catastrophic antiphospholipid syndrome: Clinical characteristics and outcomes in a non-caucasian population - A five-year experience from a single centre.

Lupus
2026

Catastrophic Antiphospholipid Syndrome.

Seminars in thrombosis and hemostasis
2025

Thrombotic Storm: A Challenging Case of Catastrophic Antiphospholipid Syndrome.

Cureus
2026

Antiphospholipid Syndrome Mimicking Acute Exacerbation of Interstitial Pneumonia: A Case Report and Literature Review.

Respirology case reports
2026

Diffuse alveolar haemorrhage in antiphospholipid syndrome: a French series of 26 patients.

Rheumatology (Oxford, England)
2025

Catastrophic antiphospholipid syndrome following surgery for Libman-Sacks endocarditis: A case report and literature review.

Journal of cardiothoracic surgery
2025

Intensive care unit admission and mortality in rheumatic diseases.

Rheumatology international
2025

A new tip for early warning of catastrophic antiphospholipid syndrome and hemolysis, elevated liver enzymes, and low platelet count syndrome: a case report.

Journal of medical case reports
2025

Case Report: A Challenging Case of Seronegative Catastrophic Antiphospholipid Syndrome.

International journal of rheumatic diseases
2025

Genetic and epigenetic dysregulation of CR1 is associated with catastrophic antiphospholipid syndrome.

Annals of the rheumatic diseases
2025

Catastrophic antiphospholipid syndrome in a young patient: a case report of severe multi-organ and cardiovascular involvement.

Oxford medical case reports
2025

A Near Catastrophe: Antiphospholipid Syndrome and Catastrophic Antiphospholipid Syndrome Complicating Emergency Department Care.

The Journal of emergency medicine
2025

Case Report: Failure of eculizumab to block complement to prevent relapse of anti-phospholipid syndrome in kidney transplant recipient.

Frontiers in nephrology
2025

Choroidal Ischemia in patients with Catastrophic Antiphospholipid Syndrome: a case series.

Retinal cases &amp; brief reports
2025

Catastrophic Antiphospholipid Syndrome Following Treated Antineutrophilic Cytoplasmic Antibody (ANCA) Vasculitis: Unusual Case Presentation.

Cureus
2025

Complex presentation of probable catastrophic antiphospholipid syndrome: diagnostic dilemmas and treatment strategies.

BMJ case reports
2025

Genetic and Epigenetic Dysregulation of CR1 is Associated with Catastrophic Antiphospholipid Syndrome (CAPS).

medRxiv : the preprint server for health sciences
2025

Pathological Study of a Case of Catastrophic Antiphospholipid Syndrome in a Patient With Gastrointestinal Bleeding.

Cureus
2025

Primary Catastrophic Antiphospholipid Syndrome With Multiple Infarcts in the Kidney, Liver, and Spleen in a Healthy Young Man: A Case Report.

Cureus
2025

Case Report: Primary adrenal insufficiency due to bilateral adrenal infarction and antiphospholipid syndrome in Covid19 - A complicate case of cardiogenic shock.

Frontiers in endocrinology
2025

Multi-organ Involvement in Catastrophic Antiphospholipid Syndrome: A Challenging Case with a Fatal Outcome.

European journal of rheumatology
2025

Coronavirus disease 2019 and catastrophic antiphospholipid syndrome: Case report.

Medicine
2025

Catastrophic antiphospholipid syndrome presenting with a stroke as a first presentation: case report.

Oxford medical case reports
2025

Surgical outcomes of cardiac surgery in patients with antiphospholipid syndrome and systemic lupus erythematosus: A systematic review.

Asian cardiovascular &amp; thoracic annals
2025

An atypical presentation of catastrophic antiphospholipid syndrome with refractoriness to treatment.

Blood coagulation &amp; fibrinolysis : an international journal in haemostasis and thrombosis
2025

Complications of Pulmonary Embolism in a Pediatric Patient.

JACC. Case reports
2025

Antiphospholipid Syndrome: A Comprehensive Clinical Review.

Journal of clinical medicine
2024

Case Report: Catastrophic antiphospholipid syndrome in a pediatric patient after percutaneous treatment of aortic re-coarctation.

Frontiers in pediatrics
2024

Intravenous Unfractionated Heparin vs. Therapeutic Plasma Exchange in Patients with Autoimmune Disease with Acute Thrombotic Events: Sampling in a Case of Catastrophic Antiphospholipid Syndrome.

Journal of Nippon Medical School = Nippon Ika Daigaku zasshi
2025

Systemic lupus erythematosus-associated autoantibodies in sickle cell disease: Spontaneous emergence in a patient and in transgenic sickle mice.

British journal of haematology
2024

Case Report: Primary catastrophic antiphospholipid syndrome in a pediatric patient with cerebral venous sinus thrombosis as the first manifestation.

Frontiers in pediatrics
2025

Positivity of antiphosphatidylserine/prothrombin antibodies identifies a subgroup of more severe antiphospholipid syndrome patients.

Clinical and experimental rheumatology
2024

Catastrophic antiphospholipid syndrome of pregnancy with acute massive cerebral infarction: A case report.

Medicine
2024

Catastrophic antiphospholipid syndrome: a CAPS-tivating hematologic disease.

Hematology. American Society of Hematology. Education Program
2024

Seronegative Catastrophic Antiphospholipid Syndrome: A Case Report of a Deadly Diagnosis.

Cureus
2024

Dramatic devastative complications in a patient with catastrophic antiphospholipid syndrome: A Case report and literature review.

SAGE open medical case reports
2024

Catastrophic Antiphospholipid Syndrome: A Review of Current Evidence and Future Management Practices.

Cureus
2024

Catastrophic Antiphospholipid Syndrome in a Lupus Patient With Severe Recurrent Life-Threatening Clinical Manifestations.

Journal of medical cases
2024

Case report: Timing of eculizumab treatment in catastrophic antiphospholipid syndrome.

Frontiers in immunology
2024

Catastrophic antiphospholipid syndrome: a case report and literature review.

Journal of wound care
2024

Antiphospholipid Syndrome and Catastrophic Antiphospholipid Syndrome: A Comprehensive Review of Pathogenesis, Clinical Features, and Management Strategies.

Cureus
2024

Catastrophic antiphospholipid syndrome with massive cerebral venous sinus thrombosis.

Clinical and experimental rheumatology
2024

Myocardial Involvement in Catastrophic Antiphospholipid Syndrome during Pregnancy or Puerperium: A Case of a Young Breastfeeding Woman and Literature Review.

Journal of clinical medicine
2024

Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry".

Medicina clinica
2024

Pediatric antiphospholipid syndrome: is it the same as an adult?

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
2025

Catastrophic Thrombosis: A Narrative Review.

Seminars in thrombosis and hemostasis
2024

Antiphospholipid syndrome in children.

Best practice &amp; research. Clinical rheumatology
2024

Catastrophic Antiphospholipid Syndrome: A Life-Threatening Condition.

Cureus
2024

Hematological features and alternate diagnoses in critically ill thrombotic antiphospholipid syndrome patients.

Clinical rheumatology
2024

Systemic Embolism Following Mechanical Thrombectomy for Acute Ischemic Stroke: A Case of Suspected Catastrophic Antiphospholipid Syndrome.

Journal of neuroendovascular therapy
2024

AngioJet thrombectomy with extracorporeal membrane oxygenation support for an acute large-scale pulmonary embolism with bilateral atrial thrombosis: a case report of catastrophic antiphospholipid syndrome.

Frontiers in cardiovascular medicine
2024

Antiphospholipid Syndrome and Patent Foramen Ovale: A Case Report and Literature Review.

Cureus
2024

The Relentless Recurrence of Diffuse Alveolar Hemorrhage in Catastrophic Antiphospholipid Syndrome and Lupus: A Therapeutic Challenge.

Cureus
2024

Catastrophic antiphospholipid syndrome: challenging case and importance of multidisciplinary evaluation and management.

Clinical case reports
2024

Extensive Thrombosis in Catastrophic Antiphospholipid Syndrome in a Newly Diagnosed Systemic Lupus Erythematosus: A Case Report.

Cureus
2024

Triumph Over Adversity: A Comprehensive Case Series on Successful Pregnancy Outcomes in Antiphospholipid Antibody (APLA)-Positive Patients.

Cureus
2024

Catastrophic antiphospholipid syndrome after pregnancy complicated by hemolysis, elevated liver enzymes and low platelets syndrome.

Obstetric medicine
2024

Pediatric catastrophic antiphospholipid syndrome with extensive skin involvement.

Pediatric dermatology
2024

First presentation of Graves' thyroid storm complicated by catastrophic antiphospholipid antibody syndrome: A case report.

SAGE open medical case reports
2024

Management of Anticoagulation and Antifibrinolytics in Catastrophic Antiphospholipid Syndrome.

Seminars in cardiothoracic and vascular anesthesia
2024

Balloon pulmonary angioplasty under awake veno-arterial extracorporeal membrane oxygenation in a patient with class III obesity with chronic thromboembolic pulmonary hypertension complicated with multiple serious comorbidities.

Pulmonary circulation
2024

Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?

Current rheumatology reports
2024

Primary catastrophic antiphospholipid syndrome in children with midbrain infarction: a case report.

Frontiers in pediatrics
2024

Therapeutic plasma exchange in catastrophic antiphospholipid syndrome: a rare case with concomitant systemic lupus erythematosus and infection.

Polish archives of internal medicine
2024

Cardiac involvement in the catastrophic antiphospholipid syndrome (CAPS): Lessons from the "CAPS registry".

Seminars in arthritis and rheumatism
2024

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.

Antibodies (Basel, Switzerland)
2024

Eculizumab therapy and complement regulation in a case of resistant catastrophic antiphospholipid syndrome.

BMJ case reports
2024

What we know and what we don't know about catastrophic antiphospholipid syndrome.

Rheumatology (Oxford, England)
2024

A Case of Catastrophic Antiphospholipid Syndrome with Acute Multiorgan Thrombosis and Concerns for Re-Emergence.

The American journal of case reports
2024

Connective tissue disease as a challenge in heart failure: Three case reports.

Medicine
2023

Antiphospholipid syndrome in rural, remote, and First Nations peoples in the Top End of the Northern Territory, Australia.

Research and practice in thrombosis and haemostasis
2023

Cardiac arrest in the setting of probable catastrophic antiphospholipid syndrome in young patient with a history of COVID infection and polyglandular disorder-Case report.

SAGE open medical case reports
2023

Unveiling Complexity: A Case Report of Catastrophic Antiphospholipid Syndrome With Coronary Occlusion and a Concomitant Patent Foramen Ovale Shunt.

Cureus
2023

Complement biomarkers in the antiphospholipid syndrome - Approaches to quantification and implications for clinical management.

Clinical immunology (Orlando, Fla.)
2023

A Rare Case of Refractory Catastrophic Antiphospholipid Syndrome Successfully Treated With Rituximab and Plasma Exchange.

Journal of community hospital internal medicine perspectives
2023

Pulmonary involvement in catastrophic antiphospholipid syndrome: A descriptive analysis from the "CAPS Registry".

Seminars in arthritis and rheumatism
2023

French National Diagnostic and Care Protocol for antiphospholipid syndrome in adults and children.

La Revue de medecine interne
2023

Perioperative management of patients with antiphospholipid and catastrophic antiphospholipid syndrome undergoing urgent neurosurgery.

Frontiers in pharmacology
2023

Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus.

Cureus
2025

Antiphospholipid Syndrome: Thrombotic and Vascular Complications.

Cardiology in review
2023

Catastrophic Antiphospholipid Syndrome: A Rare Cause of Acute Heart Failure.

Cureus
2025

Antiphospholipid Syndrome: State of the Art of Clinical Management.

Cardiovascular drugs and therapy
2023

A Rare Rheumatologic Case of Catastrophic Antiphospholipid Syndrome.

Cureus
2024

Oligosymptomatic infection by SARS-Cov-2 in catastrophic antiphospholipid syndrome, a singular coincidence: a case report in an Ecuadorian hospital.

Hematology, transfusion and cell therapy
2023

Penile Glans Necrosis Associated With Antiphospholipid Syndrome: A Rare Complication.

Cureus
2023

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review.

In vivo (Athens, Greece)
2024

Choroidal involvement in patients with catastrophic anti-phospholipid syndrome: Two case reports.

European journal of ophthalmology
2023

Catastrophic Antiphospholipid Syndrome and Renal Failure: An Unexpected Recovery After Three Years on Dialysis.

Cureus
2023

Early HELLP Syndrome or Catastrophic Antiphospholipid Syndrome? A Diagnostic Dilemma.

Cureus
2023

Catastrophic antiphospholipid syndrome in Mexico. Literature review.

Reumatologia clinica
2023

Unusual presentation of acquired thrombotic thrombocytopenic purpura (TTP) versus catastrophic antiphospholipid syndrome in a patient with Moya-Moya disease, case report, and literature review.

Clinical case reports
2023

Catastrophic Antiphospholipid Syndrome: A Case Series.

The Indian journal of radiology &amp; imaging
2023

Catastrophic antiphospholipid syndrome immediately after coronary angiography: the unexpected complication.

Blood coagulation &amp; fibrinolysis : an international journal in haemostasis and thrombosis
2023

Catastrophic antiphospholipid syndrome accompanied by complement regulatory gene mutation.

The Turkish journal of pediatrics
2023

Catastrophic antiphospholipid syndrome in an immune thrombocytopenia patient treated with avatrombopag.

Research and practice in thrombosis and haemostasis
2023

Catastrophic Antiphospholipid Syndrome as a Complication of COVID-19 Infection.

Journal of investigative medicine high impact case reports
2023

An Unusual Case of Catastrophic Antiphospholipid Syndrome in an Elderly Man.

Cureus
2023

Catastrophic antiphospholipid syndrome in lupus-associated immune thrombocytopenia treated with eltrombopag A case series and literature review.

Medicine
2023

Thrombotic storm and cardiovascular manifestations of probable catastrophic antiphospholipid syndrome: the usefulness of multimodal imaging.

European heart journal. Case reports
2023

Corticosteroids, Plasmapheresis, Argatroban, Rituximab, and Sirolimus Provided Clinical Benefit for Catastrophic Antiphospholipid Syndrome in a Patient with a History of Heparin-Induced Thrombocytopenia.

Case reports in rheumatology
2023

Precipitating factors of catastrophic antiphospholipid syndrome: the role of anticoagulant treatment in a series of 112 patients.

Journal of thrombosis and haemostasis : JTH
2022

[Selected severe &#8222;haematological&#8220; syndromes in adult intensive care patients].

Vnitrni lekarstvi
2022

A case report of acute heart failure and cardiogenic shock caused by catastrophic antiphospholipid syndrome and lupus myocarditis.

European heart journal. Case reports
2023

Cutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients.

JAMA dermatology
2022

Subacute parkinsonism due to systemic lupus erythematosus and catastrophic antiphospholipid syndrome.

Lancet (London, England)
2023

Differentiating and Managing Rare Thrombotic Microangiopathies During Pregnancy and Postpartum.

Obstetrics and gynecology
2023

Catastrophic antiphospholipid syndrome presenting initially as severe abdominal pain.

Polish archives of internal medicine
2022

Antiphospholipid Antibody Syndrome-Associated Increased Surface Expression of VLA4 Integrin on Human Monocytes.

Biomedicines
2022

Rapidly evolving necrotic skin lesions of the face.

JAAD case reports
2022

Emerging Therapies in Antiphospholipid Syndrome.

Transfusion medicine reviews
2023

Determination of four homogeneous subgroups of patients with antiphospholipid syndrome: a cluster analysis based on 509 cases.

Rheumatology (Oxford, England)
2022

Prevalence, characteristics and outcome of cardiac manifestations in critically-ill antiphospholipid syndrome patients.

Journal of autoimmunity
2022

[Heart involvement in systemic lupus erythematosus and antiphospholipid syndrome].

La Revue de medecine interne
2023

Early recognition of catastrophic antiphospholipid syndrome in patients with antiphospholipid syndrome based on a Chinese cohort study.

Clinical and experimental rheumatology
2022

Suspected COVID-19 Immunization-Induced Probable Catastrophic Antiphospholipid Syndrome.

Cureus
2022

Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome.

Current neurology and neuroscience reports
2023

Bilateral adrenal hemorrhage: A rare presentation of catastrophic anti-phospholipid syndrome.

The American journal of the medical sciences
2022

Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review.

The Brown journal of hospital medicine
2022

Catastrophic antiphospholipid syndrome with Epstein-Barr virus-associated hemophagocytosis: A clinicopathological conference.

Lupus
2022

Pontine Infarct as Initial Presentation of Catastrophic Antiphospholipid Syndrome in Systemic Lupus Erythematous.

Acta medica Indonesiana
2022

COVID-19 Vaccines and Autoimmune Hematologic Disorders.

Vaccines
2023

Severe Libman-Sacks endocarditis complicating antiphospholipid syndrome: a retrospective analysis of 23 operated cases.

Rheumatology (Oxford, England)
2022

The clinical significance of low complement levels in patients with catastrophic antiphospholipid syndrome: A descriptive analysis of 73 patients from the "Catastrophic antiphospholipid syndrome registry".

Lupus
2022

Cardiovascular complications of catastrophic antiphospholipid syndrome: a case report and review of literature.

European heart journal. Case reports
2022

Catastrophic Antiphospholipid Syndrome Presenting as a Stroke in an 11-Year-Old with Lupus.

Case reports in pediatrics
2022

Catastrophic Antiphospholipid Syndrome after Orthotopic Liver Transplant.

Case reports in transplantation
2022

Small intestine necrosis in catastrophic antiphospholipid syndrome: A rare and severe case.

Lupus
2022

Aortitis is an under-recognized manifestation of antiphospholipid syndrome: A case report and literature review.

Lupus
2022

Severe infectious acute respiratory failure mimicking COVID-19 in a healthy adolescent.

Respirology case reports
2022

All catastrophes are not catastrophic antiphospholipid syndrome.

American journal of hematology
2022

Clinical Management of Thrombotic Antiphospholipid Syndrome.

Journal of clinical medicine
2022

Digital ischemia as the initial presentation of catastrophic antiphospholipid syndrome.

Reumatologia clinica
2022

Eculizumab use in catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis from the "CAPS Registry".

Autoimmunity reviews
2022

Cost-effectiveness analysis of treatments for the first episode of catastrophic antiphospholipid syndrome: A study based on the catastrophic antiphospholipid syndrome registry.

Lupus
2022

Rare yet catastrophic presentation of undiagnosed antiphospholipid syndrome.

BMJ case reports
2022

A Middle-Aged Woman Presented with Catastrophic Anti-phospholipid Syndrome in Nephrology Department of BSMMU.

Mymensingh medical journal : MMJ
2021

Commentary: Managing catastrophic antiphospholipid syndrome-do we have a way out?

JTCVS techniques
2021

Catastrophic antiphospholipid syndrome in the setting of mitral valve repair.

JTCVS techniques
2021

Catastrophic anti-phospholipid syndrome with Libman-Sacks endocarditis following eltrombopag therapy for immune thrombocytopenic purpura: A case report.

Lupus
2021

Catastrophic antiphospholipid syndrome complicated with essential thrombocythaemia after COVID-19 vaccination: in search of the underlying mechanism.

Rheumatology advances in practice
2022

Ferritin - from iron, through inflammation and autoimmunity, to COVID-19.

Journal of autoimmunity
2022

Catastrophic antiphospholipid syndrome triggered by mRNA COVID-19 vaccine.

Clinical and experimental rheumatology
2023

Secondary vasculopathy due to catastrophic antiphospholipid syndrome.

Hematology, transfusion and cell therapy
2021

A Diagnostic Dilemma: Catastrophic or Seronegative Antiphospholipid Syndrome.

Cureus
2021

Concurrent arterial and venous thrombosis in a patient with catastrophic antiphospholipid syndrome.

Caspian journal of internal medicine
2021

Report on effective treatment and genetic predisposition in two children with refractory probable catastrophic antiphospholipid syndrome.

Thrombosis research
2021

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome.

Frontiers in cardiovascular medicine
2021

A Case Report of COVID-Associated Catastrophic Antiphospholipid Syndrome Successfully Treated with Eculizumab.

Journal of blood medicine
2021

Catastrophic Antiphospholipid Syndrome Associated With Systemic Lupus Erythematosus Flare With Multiorgan Failure: A Chronicle of Cardiogenic Shock, Renal Failure, Vasculitis-Like Lesions, and Disseminated Intravascular Coagulation.

Cureus
2022

COVID-19 and adult-onset Still's disease as part of hyperferritinemic syndromes.

Modern rheumatology case reports
2022

Catastrophic Antiphospholipid Syndrome Following Lower Extremity Arterial Bypass Surgery: Case Report and Evidence Review.

Vascular and endovascular surgery
2021

Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature.

Medicina (Kaunas, Lithuania)
2021

Case 294: Catastrophic Antiphospholipid Syndrome.

Radiology
2021

Phenytoin Pharmacokinetics During Venoarterial Extracorporeal Membrane Oxygenation and Plasma Exchange.

Cureus
2021

A Difficult and Rare Case of Warfarin Refractory Antiphospholipid Syndrome Presenting With Catastrophic Antiphospholipid Syndrome Complicated by Gastrointestinal Bleeding.

Cureus
2021

Disseminated Mycobacterium chelonae infection with Hughes-Stovin syndrome.

Proceedings (Baylor University. Medical Center)
2021

[Severe organ manifestations in rheumatic diseases].

Deutsche medizinische Wochenschrift (1946)
2022

Complement and the prothrombotic state.

Blood
2021

Neovascular Glaucoma as a Presenting Sign of Catastrophic Antiphospholipid Syndrome with a "Catastrophic" Heart Valve Finding.

Case reports in ophthalmology
2022

Early use of eculizumab for catastrophic antiphospholipid syndrome.

British journal of haematology
2021

Catastrophic antiphospholipid syndrome during pregnancy.

European journal of obstetrics, gynecology, and reproductive biology
2021

Pathogenic lipid-binding antiphospholipid antibodies are associated with severity of COVID-19.

Journal of thrombosis and haemostasis : JTH
2021

Small Bowel Necrosis Associated with Catastrophic Antiphospholipid Syndrome: A Case Report.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2021

Expert Perspective: Management of Microvascular and Catastrophic Antiphospholipid Syndrome.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2022

Characteristics of purified anti-β2GPI IgG N-glycosylation associate with thrombotic, obstetric and catastrophic antiphospholipid syndrome.

Rheumatology (Oxford, England)
2021

Ferritin, fever, and frequent visits: Hyperferritinemic syndromes in the emergency department.

The American journal of emergency medicine
2021

Primary Refractory Catastrophic Antiphospholipid Syndrome Masquerading as Buerger's Disease.

Cureus
2021

Erythrocytosis, embolism in a woman with antiphospholipid antibodies: A diagnostic challenge.

Clinical case reports
2021

Catastrophic antiphospholipid syndrome presenting with aortic barrage: case report and review of the literature.

Lupus
2021

Abnormal immunothrombosis and lupus anticoagulant in a catastrophic COVID-19 recalling Asherson's syndrome.

Journal of thrombosis and thrombolysis
2020

Catastrophic Antiphospholipid Syndrome in Immune Thrombocytopenic Purpura - Beyond Tenuous Concomitance!

Indian journal of nephrology
2021

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME AND POSTERIOR OCULAR INVOLVEMENT: Case Series of 11 Patients and Literature Review.

Retina (Philadelphia, Pa.)
2021

Increased levels of ferritin on admission predicts intensive care unit mortality in patients with COVID-19.

Medicina clinica (English ed.)
2021

[Spontaneous adrenal hematomas. Retrospective analysis of 20 cases from a tertiary center].

La Revue de medecine interne
2021

Catastrophic antiphospholipid syndrome presented as ruptured papillary muscle during puerperium in a patient with systemic lupus erythematosus.

Lupus
2021

Markers of complement activation in plasma during quiescent phases in patients with catastrophic antiphospholipid syndrome.

Blood
2022

A rare case of catastrophic antiphospholipid syndrome triggered by estrogen-containing oral contraceptives in a patient with double heterozygous factor V Leiden and prothrombin G20210A mutations.

American journal of hematology
2021

Immunosuppressive Treatment in Antiphospholipid Syndrome: Is It Worth It?

Biomedicines
2021

[New aspects of therapeutic plasma exchange in critical care medicine].

Deutsche medizinische Wochenschrift (1946)
2021

Update on the Use of Intravenous Immunoglobulin in Pregnancy.

NeoReviews
2022

Catastrophic antiphospholipid syndrome post-Epstein-Barr virus infection: a case report.

Hematology, transfusion and cell therapy
2020

COVID-19, Antiphospholipid Antibodies, and Catastrophic Antiphospholipid Syndrome: A Possible Association?

Clinical medicine insights. Arthritis and musculoskeletal disorders
2020

Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy.

BMC ophthalmology
2020

Hematologic Chaos in Lupus Flare: A Case of Fulminant and Simultaneous Antiphospholipid, Anti-ADAMTS13, and Red Blood Cell Autoantibodies.

Case reports in rheumatology
2020

Severe mesenteric ischemia with multiple organ failure in a patient previously treated with a humanized monoclonal antibody against programmed death receptor-1 (pembrolizumab), a case of pembrolizumab associated catastrophic antiphospholipid syndrome?

SAGE open medical case reports
2021

Successful outcome with eculizumab treatment in a patient with antiphospholipid syndrome presenting with an unusual thrombotic storm.

Journal of thrombosis and thrombolysis
2021

Hematological Manifestations among Patients with Rheumatic Diseases.

Acta haematologica
2020

Conservative Management of Catastrophic Antiphospholipid Syndrome-Related Skin Necrosis: A Case Report.

Advances in skin &amp; wound care
2022

Catastrophic Antiphospholipid Syndrome: Skin, Eye and Brain Involvement.

Ocular immunology and inflammation
2021

Pediatric Catastrophic Antiphospholipid Syndrome Successfully Treated with Eculizumab.

American journal of respiratory and critical care medicine
2021

Catastrophic antiphospholipid syndrome associated with systemic lupus erythematosus: a case-based review.

Future cardiology
2021

Pediatric catastrophic antiphospholipid syndrome patient evolving to systemic lupus erythematosus.

Lupus
2020

16th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome.

Lupus
Ver todos os 502 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Catastrophic Antiphospholipid Syndrome: A Rare but Life-Threatening Thrombotic Storm-A Case Report and Literature Review.
    Case reports in rheumatology· 2026· PMID 41777560mais citado
  2. Imaging features of catastrophic antiphospholipid syndrome : Massive saddle pulmonary embolism with subsequent complete resolution.
    Radiology case reports· 2026· PMID 41647748mais citado
  3. Catastrophic antiphospholipid syndrome with eosinophilia mimicking hypereosinophilic syndromes with disseminated intravascular coagulation: a case report.
    Journal of medical case reports· 2026· PMID 41634880mais citado
  4. Navigating clinical overlap: A case of polyarteritis nodosa in the setting of antiphospholipid syndrome.
    Modern rheumatology case reports· 2026· PMID 41615024mais citado
  5. Catastrophic antiphospholipid syndrome: Clinical characteristics and outcomes in a non-caucasian population - A five-year experience from a single centre.
    Lupus· 2026· PMID 41606985mais citado
  6. Ultrastructural Injury of Renal Podocytes in Catastrophic Antiphospholipid Syndrome.
    Kidney Med· 2026· PMID 41993685recente
  7. Bartonella henselae infection associated with a multisystemic thrombo-inflammatory syndrome in an immunocompetent adult.
    Infection· 2026· PMID 41986819recente
  8. [Management of emergencies in rheumatology].
    Med Klin Intensivmed Notfmed· 2026· PMID 41979866recente
  9. From hemoptysis to thrombosis: a comprehensive case study of catastrophic antiphospholipid syndrome in a young female.
    J Cardiothorac Surg· 2026· PMID 41965772recente
  10. Severe thrombocytopenia in antiphospholipid syndrome: a retrospective study of 432 patients.
    RMD Open· 2026· PMID 41956709recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:464343(Orphanet)
  2. MONDO:0018737(MONDO)
  3. GARD:9820(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q5051580(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome antifosfolípido catastrófico
Compêndio · Raras BR

Síndrome antifosfolípido catastrófico

ORPHA:464343 · MONDO:0018737
Prevalência
Unknown
Herança
Not applicable
CID-10
D68.6 · Outros defeitos da coagulação
CID-11
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C3662487
EuropePMC
Wikidata
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