Raras
Buscar doenças, sintomas, genes...
Doença de Buerger
ORPHA:36258CID-10 · I73.1CID-11 · 4A44.8OMIM 211480DOENÇA RARA

Doença vascular inflamatória não necrosante rara que afeta as artérias e veias de pequeno e médio calibre das extremidades superiores e inferiores, caracterizada por endarterite e vaso-oclusão devido ao desenvolvimento de trombo oclusivo. O desenvolvimento e progressão da doença estão consistentemente associados à exposição ao tabaco.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Doença vascular inflamatória não necrosante rara que afeta as artérias e veias de pequeno e médio calibre das extremidades superiores e inferiores, caracterizada por endarterite e vaso-oclusão devido ao desenvolvimento de trombo oclusivo. O desenvolvimento e progressão da doença estão consistentemente associados à exposição ao tabaco.

Pesquisas ativas
3 ensaios
19 total registrados no ClinicalTrials.gov
Publicações científicas
187 artigos
Último publicado: 2026 Feb 27

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
16.0
Worldwide
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I73.1
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

🩸
Sangue
2 sintomas
🦴
Ossos e articulações
2 sintomas
🧬
Pele e cabelo
1 sintomas
🧠
Neurológico
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

90%prev.
Trombose arterial
Muito frequente (99-80%)
90%prev.
Úlcera cutânea
Muito frequente (99-80%)
90%prev.
Gangrena
Muito frequente (99-80%)
90%prev.
Vasculite
Muito frequente (99-80%)
90%prev.
Neuropatia sensorial
Muito frequente (99-80%)
55%prev.
Claudicação intermitente
Frequente (79-30%)
20sintomas
Muito frequente (5)
Frequente (7)
Ocasional (4)
Muito raro (1)
Sem dados (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 20 características clínicas mais associadas, ordenadas por frequência.

Trombose arterialArterial thrombosis
Muito frequente (99-80%)90%
Úlcera cutâneaSkin ulcer
Muito frequente (99-80%)90%
GangrenaGangrene
Muito frequente (99-80%)90%
VasculiteVasculitis
Muito frequente (99-80%)90%
Neuropatia sensorialSensory neuropathy
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico187PubMed
Últimos 10 anos52publicações
Pico20248 papers
Linha do tempo
2026Hoje · 2026🧪 2003Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 31
2Fase 24
1Fase 11
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 9 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença de Buerger

🗺️

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.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

19 ensaios clínicos encontrados, 3 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
52 papers (10 anos)
#1

The Diagnostic Pitfall of Ankle-Brachial Index in Buerger Disease: Can We Trust Pressure Measurements in the Presence of Corkscrew Collaterals?

Journal of vascular and interventional radiology : JVIR2026 Feb 27

To evaluate the diagnostic performance of the ankle-brachial index (ABI) in patients with Buerger disease and to assess the impact of collateral circulation on ankle pressure measurements in the presence of angiographically confirmed infrapopliteal arterial occlusions. This retrospective study included 66 lower limbs from 45 male patients (mean age, 45.4 years [SD ± 9.7]) diagnosed with Buerger disease. All patients underwent digital subtraction angiography (DSA) and ABI measurement prior to endovascular intervention. Limbs were categorized according to the angiographic patency of the anterior and posterior tibial arteries. ABI diagnostic performance was evaluated using DSA as the reference standard, with emphasis on the prevalence of normal ABI values (≥0.90) in limbs with complete tibial artery occlusion. Angiography demonstrated complete occlusion of both tibial arteries in 50 of 66 limbs (75.7%). Despite severe infrapopliteal disease, 21 of these 50 limbs (42.0%) exhibited normal ABI values, indicating a high false-negative rate. The mean ABI in limbs with complete tibial occlusion was 0.82 (SD ± 0.20). Doppler-derived ankle pressure measurements were obtainable in 94%-98% of occluded tibial arteries due to extensive collateral circulation. ABI values did not differ significantly between limbs with isolated tibial occlusion and those with combined popliteal and tibial artery involvement (P > .05). ABI frequently underestimates disease severity in Buerger disease because collateral networks preserve ankle pressures despite critical infrapopliteal occlusions. ABI should not be used as a standalone screening tool, and adjunct physiologic testing or imaging is required for accurate vascular assessment.

#2

A systematic review and meta-analysis of early and long-term outcomes after endovascular interventions in patients with thromboangiitis obliterans and critical limb ischemia with GRADE assessment.

BMC surgery2026 Feb 16

Thromboangiitis obliterans (TAO) is a rare inflammatory occlusive vascular disorder that affects small and medium-sized vessels and often progresses to critical limb ischemia (CLI). Although various treatments have been used in advanced stages, endovascular interventions have increasingly gained attention as a limb-saving therapy. Due to limited and varied evidence in published studies, this systematic review and meta-analysis evaluates the early and long-term outcomes of endovascular treatments in patients with TAO-related CLI. This systematic review and meta-analysis was conducted according to PRISMA guidelines and registered on PROSPERO (CRD420251089226). PubMed, Scopus, WOS, Embase, and Google Scholar databases were searched with relevant keywords up to July 2025. A total of 27 studies involving 1,217 patients were included. Pooled outcomes were estimated using a generalized linear mixed model (GLMM). Heterogeneity was assessed using the I² statistic, and potential publication bias was examined via funnel plots and Egger’s test. The pooled technical success rate for endovascular procedures was 89%, with limb salvage rates of 94% at 6 months and 97% at 12 months. Primary patency was 56% at 6 months, 73% at 12 months, and 61% at 36 months; these findings highlight temporal variability, and cross-time comparisons should be interpreted with caution. Secondary patency was initially higher but also decreased over long-term follow-up. Restenosis rates were 15% early and 34% late. Major and minor amputation rates were 10% and 13%, respectively. No in-hospital mortality was reported. Endovascular therapy represents a safe and effective option for treating TAO-related CLI, especially in patients who are not candidates for surgery. While early results are promising, the loss of patency and rising restenosis over time highlight the importance of careful follow-up and proper patient selection. Our findings support a personalized, multidisciplinary approach to managing TAO and emphasize the vital role of smoking cessation in improving clinical outcomes. [Image: see text] The online version contains supplementary material available at 10.1186/s12893-026-03563-2.

#3

Seltoplasmid promotes ulcer healing versus placebo for treating patients with chronic limb-threatening ischemia: HOPE CLTI-2 trial.

Molecular therapy : the journal of the American Society of Gene Therapy2025 Jul 02

Intramuscular injection of donaperminogene seltoplasmid (recombinant human hepatocyte growth factor plasmids) represents a gene therapy that treats patients with chronic limb-threatening ischemia (CLTI). The HOPE CLTI-2 trial was a phase 3, multicenter, double-blind, placebo-controlled study aimed to evaluate the efficacy and safety of seltoplasmid in patients with Rutherford class 5 CLTI. This study did not require participants to be ineligible for revascularization, allowing enrollment of patients with CLTI caused by either atherosclerosis (ASO) or Buerger disease (or thromboangiitis obliterans [TAO]). The primary endpoint was the complete ulcer healing rate at 6 months. A total of 242 participants (53.3% ASO versus 46.7% TAO) were enrolled, with 161 receiving seltoplasmid and 81 receiving placebo. Complete ulcer healing was achieved in 70 patients in the seltoplasmid group compared to 15 patients in the placebo group, resulting in an adjusted healing rate difference of 26.1% (95% confidence interval [CI]: 15.1%-37.0%; p < 0.001). The hazard ratio for healing was 2.31 (95% CI: 1.32-4.05; p = 0.004). The benefits of seltoplasmid on ulcer healing persisted in both TAO and ASO subgroups. Serious adverse events were rare. Our study demonstrated that seltoplasmid significantly improved ulcer healing rates in patients with Rutherford class 5 CLTI compared to placebo.

#4

Treatment Options for Buerger Disease: A Systematic Review and Meta-Analysis of Outcomes.

The Journal of surgical research2025 Feb

Uncertainties exist regarding the optimal management strategy for patients with thromboangiitis obliterans (TAOs). The aim of this study was to investigate the safety and effectiveness of common interventions used for treating patients with TAO. Endovascular treatment, revascularization, sympathectomy, stem cell therapy (SCT), and nonsurgical interventions were selected for inclusion in the study. Changes in mean visual analogue scale score and ankle brachial index (ABI) values were evaluated. In addition, the rate of ulcer healing, postintervention amputation, and overall complication rates were compared across interventions. A total of 1262 TAO patients (1159 males, 84 females), with a mean age of 38.4 ± 7.8 ys, were included in this systematic review. With an amputation rate of 16.6%, the endovascular treatment group showed statistically significant improvements in mean ABI and visual analogue scale scores (P < 0.05). There was a greater increase in ABI postoperatively with endovascular treatment compared to SCT (P < 0.05), and also a greater increase in ABI postoperatively with revascularization compared to SCT (P < 0.05). Meta-regression showed that both endovascular treatment and open surgical revascularization were superior to stem cell treatment for postoperative mean ABI improvement (P < 0.05). Interestingly, the rate of postoperative amputation was lower in the SCT group compared with the other two interventions. Our results indicated that all three interventions may be a reasonable therapeutic option for TAO. Endovascular intervention and open revascularization demonstrated superior outcomes.

#5

ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis and Buerger Disease.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases2025 Apr 01

Publicações recentes

Ver todas no PubMed

📚 EuropePMC111 artigos no totalmostrando 51

2026

The Diagnostic Pitfall of Ankle-Brachial Index in Buerger Disease: Can We Trust Pressure Measurements in the Presence of Corkscrew Collaterals?

Journal of vascular and interventional radiology : JVIR
2026

A systematic review and meta-analysis of early and long-term outcomes after endovascular interventions in patients with thromboangiitis obliterans and critical limb ischemia with GRADE assessment.

BMC surgery
2025

Seltoplasmid promotes ulcer healing versus placebo for treating patients with chronic limb-threatening ischemia: HOPE CLTI-2 trial.

Molecular therapy : the journal of the American Society of Gene Therapy
2025

ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis and Buerger Disease.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2025

Treatment Options for Buerger Disease: A Systematic Review and Meta-Analysis of Outcomes.

The Journal of surgical research
2024

Ultrasonographic Study of the Corkscrew Arterial Image in Buerger Disease Patients.

Annals of vascular diseases
2024

Thromboangiitis obliterans (Buerger disease).

Vascular medicine (London, England)
2024

Spontaneous Intercostal Artery Bleeding in a Patient With Buerger's Disease: A Case Report.

Cureus
2024

Low-intensity pulsed ultrasound improves symptoms in patients with Buerger disease: a double-blinded, randomized, and placebo-controlled study.

Scientific reports
2024

Vasoactive and Antifibrotic Properties of Cannabinoids and Applications to Vasospastic/Vaso-Occlusive Disorders: A Systematic Review.

Annals of plastic surgery
2024

Low-intensity pulsed ultrasound for 'no-option' chronic/critical limb-threatening ischaemia in a patient with Buerger disease: a case report.

European heart journal. Case reports
2024

True leukonychia as the presenting sign of early thromboangiitis obliterans.

JAAD case reports
2024

The effect of different taping techniques on transtibial amputation walking parameters: A case report.

Prosthetics and orthotics international
2023

Off-the-shelf percutaneous deep vein arterialization for no-option chronic limb-threatening ischemia related to Buerger disease.

Journal of vascular surgery cases and innovative techniques
2023

The Effect of Smoking Cessation on the Technical Success of Endovascular Treatment for Thromboangiitis Obliterans.

Journal of vascular and interventional radiology : JVIR
2023

Do Patients With Arterial Occlusive Disease of Different Etiologies Benefit Equally From Cilostazol?

Texas Heart Institute journal
2023

A systematic review and meta-analysis of early and late outcomes after endovascular angioplasty among patients with thromboangiitis obliterans and chronic limb ischemia.

Journal of vascular surgery
2022

Buerger Disease: Pathological Changes in Elderly Patients.

Annals of vascular diseases
2022

Nails Involvement in Winiwarter-Buerger Disease.

Skin appendage disorders
2022

Estimate and Temporal Trends of Buerger Disease Hospitalizations in the United States.

The American journal of cardiology
2021

The historical differential diagnosis of the disease that afflicted Aleijadinho, the famous 18th century Brazilian sculptor.

Arquivos de neuro-psiquiatria
2021

Recent Updates and Advances in Winiwarter-Buerger Disease (Thromboangiitis Obliterans): Biomolecular Mechanisms, Diagnostics and Clinical Consequences.

Diagnostics (Basel, Switzerland)
2021

Pearls & Oy-sters: Primary Cerebral Buerger Disease: A Rare Differential Diagnosis of Stroke in Young Adults.

Neurology
2021

Pathological and immunological differences of arterial thrombi and wall caused by three different periodontal bacterial injections in rat models and proposals on the pathogeneses of vascular diseases.

Clinical and experimental dental research
2021

Left Carotid Artery Thrombosis Due to Thromboangiitis Obliterans.

The American journal of forensic medicine and pathology
2021

Endovascular Treatment of Critical Limb Ischemia in Buerger Disease (Thromboangiitis Obliterans) With Midterm Follow-Up: A Viable Option When Bypass Surgery Is Not Feasible.

AJR. American journal of roentgenology
2020

The Imbalance among Oxidative Biomarkers and Antioxidant Defense Systems in Thromboangiitis Obliterans (Winiwarter-Buerger Disease).

Journal of clinical medicine
2020

Long-Term Clinical Outcomes of Autologous Bone Marrow Mononuclear Cell Implantation in Patients With Severe Thromboangiitis Obliterans.

Circulation journal : official journal of the Japanese Circulation Society
2019

D-dimer levels in patients with thromboangiitis obliterans.

The National medical journal of India
2019

Successful Fat Grafting in a Patient With Thromboangiitis Obliterans.

Advances in skin &amp; wound care
2019

Major lower extremity amputation: a contemporary analysis from an academic tertiary referral centre in a developing community.

BMC surgery
2019

Medical adjunctive therapy for patients with chronic limb-threatening ischemia: a systematic review.

The Journal of cardiovascular surgery
2019

Case report of two pregnancies and deliveries by a woman with Buerger disease.

The journal of obstetrics and gynaecology research
2018

Performance of noninvasive laser Doppler flowmetry and laser speckle contrast imaging methods in diagnosis of Buerger disease: A case report.

Medicine
2018

Three cases of dorsal metatarsal artery bypass in patients with Buerger disease.

Journal of vascular surgery cases and innovative techniques
2018

[Digital ischemia revealing multiple myeloma].

Journal de medecine vasculaire
2018

Immediate and late outcomes of endovascular therapy for lower extremity arteries in Buerger disease.

Journal of vascular surgery
2018

Four-extremity salvage with long vein grafts in buerger disease.

Archives of plastic surgery
2017

Endothelial dysfunction in patients with Buerger disease.

Vascular health and risk management
2017

Progressive fingertip necrosis after nail avulsion injury in a patient with ulnar artery hypoplasia: A case report.

Medicine
2016

An Experimental Model of Peripheral Vascular Disease Involving the Intravenous Injection of Oral Bacteria.

Annals of vascular diseases
2016

Efficacy of bosentan in patients with refractory thromboangiitis obliterans (Buerger disease): A case series and review of the literature.

Medicine
2016

Vascular Function and Intima-media Thickness of a Leg Artery in Peripheral Artery Disease: A Comparison of Buerger Disease and Atherosclerotic Peripheral Artery Disease.

Journal of atherosclerosis and thrombosis
2016

Investigation of the Etiology of Anemia in Thromboangiitis Obliterans.

The International journal of angiology : official publication of the International College of Angiology, Inc
2016

Corkscrew Collateral Vessels in Buerger Disease: Vasa Vasorum or Vasa Nervorum.

Journal of vascular and interventional radiology : JVIR
2016

Smoking, Periodontitis, and Buerger Disease.

Annals of vascular surgery
2015

Effect of a Low-Intensity Pulsed Ultrasound Device, SX-1001, on Clinical Symptoms in Buerger Disease With Limb Ischemia.

International heart journal
2016

The Epidemiologic and Clinical Findings of Patients with Buerger Disease.

Annals of vascular surgery
2015

Ocular Inflammation in the Setting of Concomitant Systemic Autoimmune Conditions in an Older Male Population.

Cornea
2015

Refractory leg ulcers associated with Klinefelter syndrome.

Journal of Nippon Medical School = Nippon Ika Daigaku zasshi
2014

Buerger disease (thromboangiitis obliterans).

Techniques in vascular and interventional radiology
Ver todos os 111 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Doença de Buerger

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. The Diagnostic Pitfall of Ankle-Brachial Index in Buerger Disease: Can We Trust Pressure Measurements in the Presence of Corkscrew Collaterals?
    Journal of vascular and interventional radiology : JVIR· 2026· PMID 41765080mais citado
  2. A systematic review and meta-analysis of early and long-term outcomes after endovascular interventions in patients with thromboangiitis obliterans and critical limb ischemia with GRADE assessment.
    BMC surgery· 2026· PMID 41699570mais citado
  3. Seltoplasmid promotes ulcer healing versus placebo for treating patients with chronic limb-threatening ischemia: HOPE CLTI-2 trial.
    Molecular therapy : the journal of the American Society of Gene Therapy· 2025· PMID 40221835mais citado
  4. Treatment Options for Buerger Disease: A Systematic Review and Meta-Analysis of Outcomes.
    The Journal of surgical research· 2025· PMID 39847853mais citado
  5. ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis and Buerger Disease.
    Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases· 2025· PMID 39890210mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:36258(Orphanet)
  2. OMIM OMIM:211480(OMIM)
  3. MONDO:0008889(MONDO)
  4. GARD:5969(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q746001(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

Doença de Buerger
Compêndio · Raras BR

Doença de Buerger

ORPHA:36258 · MONDO:0008889
Prevalência
1-5 / 10 000
Herança
Not applicable
CID-10
I73.1 · Tromboangeíte obliterante [doença de Buerger]
CID-11
Ensaios
3 ativos
Início
Adult
Prevalência
16.0 (Worldwide)
MedGen
UMLS
C0040021
EuropePMC
Wikidata
Wikipedia
Papers 10a
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