Vasculite crônica, recidivante e multissistêmica caracterizada por lesões mucocutâneas, bem como manifestações articulares, vasculares, oculares e do sistema nervoso central.
Introdução
O que você precisa saber de cara
Vasculite crônica, recidivante e multissistêmica caracterizada por lesões mucocutâneas, bem como manifestações articulares, vasculares, oculares e do sistema nervoso central.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 49 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 95 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
15 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.
Antigen-presenting major histocompatibility complex class I (MHCI) molecule. In complex with B2M/beta 2 microglobulin displays primarily viral and tumor-derived peptides on antigen-presenting cells for recognition by alpha-beta T cell receptor (TCR) on HLA-B-restricted CD8-positive T cells, guiding antigen-specific T cell immune response to eliminate infected or transformed cells (PubMed:23209413, PubMed:25808313, PubMed:29531227, PubMed:9620674). May also present self-peptides derived from the
Cell membraneEndoplasmic reticulum membrane
Stevens-Johnson syndrome
A rare blistering mucocutaneous disease that share clinical and histopathologic features with toxic epidermal necrolysis. Both disorders are characterized by high fever, malaise, and a rapidly developing blistering exanthema of macules and target-like lesions accompanied by mucosal involvement. Stevens-Johnson syndrome is a milder disease characterized by destruction and detachment of the skin epithelium and mucous membranes involving less than 10% of the body surface area. Ocular symptoms include ulcerative conjunctivitis, keratitis, iritis, uveitis and sometimes blindness. It can be caused by a severe adverse reaction to particular types of medication, although Mycoplasma infections may induce some cases.
Involved in the regulation of innate immunity and the inflammatory response in response to IFNG/IFN-gamma (PubMed:10807793, PubMed:11468188, PubMed:16037825, PubMed:16785446, PubMed:17431422, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923, PubMed:26347139, PubMed:27030597, PubMed:28835462). Organizes autophagic machinery by serving as a platform for the assembly of ULK1, Beclin 1/BECN1, ATG16L1, and ATG8 family members and recognizes specific autophagy targets, thus coordinat
Cytoplasm, cytoskeletonCell projection, ruffleCell projection, lamellipodiumNucleusCytoplasmCytoplasmic vesicle, autophagosome
Familial Mediterranean fever, autosomal recessive
A hereditary periodic fever syndrome characterized by recurrent episodic fever, serosal inflammation and pain in the abdomen, chest or joints. It is frequently complicated by reactive amyloidosis, which leads to renal failure and can be prophylactically treated with colchicine.
Transmembrane receptor that functions as a pattern recognition receptor recognizing pathogen- and damage-associated molecular patterns (PAMPs and DAMPs) to induce innate immune responses via downstream signaling pathways (PubMed:10835634, PubMed:15809303, PubMed:16622205, PubMed:17292937, PubMed:17478729, PubMed:20037584, PubMed:20711192, PubMed:23880187, PubMed:27022195, PubMed:29038465, PubMed:17803912, PubMed:15852007). At the plasma membrane, cooperates with LY96 to mediate the innate immune
Cell membraneEarly endosomeCell projection, ruffle
Transcriptional regulator mainly expressed in hematopoietic cells that plays a critical role in cellular growth, differentiation and immune response (PubMed:10961885, PubMed:37256972, PubMed:8943379). Plays a key role in the differentiation of T-helper 1 cells and the production of interferon-gamma (PubMed:12213961, PubMed:35614130). Also participates in multiple neutrophil functions including chemotaxis and production of the neutrophil extracellular traps (By similarity). After IL12 binding to
CytoplasmNucleus
Systemic lupus erythematosus 11
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Receptor for TNFSF6/FASLG. The adapter molecule FADD recruits caspase CASP8 to the activated receptor. The resulting death-inducing signaling complex (DISC) performs CASP8 proteolytic activation which initiates the subsequent cascade of caspases (aspartate-specific cysteine proteases) mediating apoptosis. FAS-mediated apoptosis may have a role in the induction of peripheral tolerance, in the antigen-stimulated suicide of mature T-cells, or both. The secreted isoforms 2 to 6 block apoptosis (in v
Cell membraneMembrane raftSecreted
Autoimmune lymphoproliferative syndrome 1A
A disorder of apoptosis that manifests in early childhood and results in the accumulation of autoreactive lymphocytes. It is characterized by non-malignant lymphadenopathy with hepatosplenomegaly, and autoimmune hemolytic anemia, thrombocytopenia and neutropenia.
Heterodimerizes with IL12B to form the IL-12 cytokine or with EBI3/IL27B to form the IL-35 cytokine (PubMed:8605935, PubMed:8943050). IL-12 is primarily produced by professional antigen-presenting cells (APCs) such as B-cells and dendritic cells (DCs) as well as macrophages and granulocytes and regulates T-cell and natural killer-cell responses, induces the production of interferon-gamma (IFN-gamma), favors the differentiation of T-helper 1 (Th1) cells and is an important link between innate res
Secreted
Restricts trafficking of FAF2 from the endoplasmic reticulum to lipid droplets (PubMed:23297223). In association with LMBR1L and E3 ubiquitin-protein ligase AMFR, negatively regulates the canonical Wnt signaling pathway in the lymphocytes by promoting the ubiquitin-mediated degradation of CTNNB1 and Wnt receptors FZD6 and LRP6 (PubMed:31073040)
Endoplasmic reticulum membrane
May play a role as a receptor for the recognition of MHC class I HLA-E molecules by NK cells
Membrane
Precursor of non-enzymatic components of the classical, lectin and GZMK complement pathways, which consist in a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system Non-enzymatic component of C3 and C5 convertases (PubMed:8538770). Generated following cleavage by complement proteases (C1S, MASP2 or GZMK, depending on the complement pathway), it covalently attaches to the surface of pathogens, where it acts as an opson
SecretedSynapseCell projection, axonCell projection, dendriteCell surface
Complement component 4A deficiency
A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.
Chemokine receptor that plays a crucial role in regulating immune cell migration, inflammation, and immune responses (PubMed:14991608). Contributes to the inflammatory response by recruiting immune cells, such as monocytes, macrophages, T-cells, and dendritic cells, to sites of inflammation for the clearance of pathogens and the resolution of tissue damage. When activated by its ligands including CCL3, CCL5-9, CCL13-16 and CCL23, triggers a signaling cascade within immune cells, leading to their
Cell membrane
Associates with IL12RB1 to form the interleukin-23 receptor. Binds IL23 and mediates T-cells, NK cells and possibly certain macrophage/myeloid cells stimulation probably through activation of the Jak-Stat signaling cascade. IL23 functions in innate and adaptive immunity and may participate in acute response to infection in peripheral tissues. IL23 may be responsible for autoimmune inflammatory diseases and be important for tumorigenesis
Cell membrane
Inflammatory bowel disease 17
A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.
Major immune regulatory cytokine that acts on many cells of the immune system where it has profound anti-inflammatory functions, limiting excessive tissue disruption caused by inflammation. Mechanistically, IL10 binds to its heterotetrameric receptor comprising IL10RA and IL10RB leading to JAK1 and STAT2-mediated phosphorylation of STAT3 (PubMed:16982608). In turn, STAT3 translocates to the nucleus where it drives expression of anti-inflammatory mediators (PubMed:18025162). Targets antigen-prese
Secreted
Receptor subunit for interferon gamma/INFG that plays crucial roles in antimicrobial, antiviral, and antitumor responses by activating effector immune cells and enhancing antigen presentation (PubMed:20015550). Associates with transmembrane accessory factor IFNGR2 to form a functional receptor (PubMed:10986460, PubMed:2971451, PubMed:7615558, PubMed:7617032, PubMed:7673114). Upon ligand binding, the intracellular domain of IFNGR1 opens out to allow association of downstream signaling components
Cell membrane
Immunodeficiency 27A
A form of Mendelian susceptibility to mycobacterial disease, a rare condition caused by impairment of interferon-gamma mediated immunity. It is characterized by predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine, environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. Clinical outcome severity depends on the degree of impairment of interferon-gamma mediated immunity. Some patients die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas.
Aminopeptidase that plays a central role in peptide trimming, a step required for the generation of most HLA class I-binding peptides. Peptide trimming is essential to customize longer precursor peptides to fit them to the correct length required for presentation on MHC class I molecules. Strongly prefers substrates 9-16 residues long. Rapidly degrades 13-mer to a 9-mer and then stops. Preferentially hydrolyzes the residue Leu and peptides with a hydrophobic C-terminus, while it has weak activit
Endoplasmic reticulum membrane
Variantes genéticas (ClinVar)
264 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 19 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
58 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença de Behçet
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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
Ensaios em destaque
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Outros ensaios clínicos
24 ensaios clínicos encontrados, 5 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 901
Multiple cerebral venous thrombosis as the first presentation of Behçet syndrome: case report and literature review.
Cerebral venous thrombosis as the first manifestation of Behçet's Syndrome (BS) is very rare. We describe a case of BS in which all four major cerebral venous sinuses, including the bilateral sigmoid sinuses, transverse sinuses, and superior sagittal sinuses, exhibited severe cerebral venous thrombosis. An 18-year-old man was brought to our hospital with a three-month-old, wide, throbbing headache in both hemispheres and double, blurred vision while looking to the right for the last week. Magnetic Resonance Imaging (MRI) showed extensive thrombosis involving the bilateral sigmoid sinuses, transverse sinuses, and superior sagittal sinus. Collagen tissue diseases and systemic thrombophilia were ruled out. The patient, who had a history of erythema nodosum and genital and oral aphthae, was evaluated as 3/6 (+) positive by the pathergy test, leading to the diagnosis of BS. Prednisolone 48 mg/day and azathioprine 2 × 50 mg/day were started, and the prednisolone dosage was lowered to be stopped in three to four months. For nine months, anticoagulant medicine was administered. Partial recanalization was seen on control MRI venography. It may be life-saving to consider Behçet syndrome as a differential diagnosis in patients with multiple cerebral venous thrombosis.
Effectiveness and Tolerability of Anti-Tumor Necrosis Factor Alpha Therapy in Refractory Intestinal Behçet's Disease: A Large Single-Center Study.
Intestinal Behçet's disease (BD) is a rare, chronic intestinal vascular disorder often refractory to conventional therapy. We aimed to assess the effectiveness and tolerability of anti-tumor necrosis factor alpha (anti-TNF-α) therapy in patients with moderate to severe refractory intestinal BD. Clinical remission, clinical response, and biological response rates at 4, 12, and 24 months, as well as the adverse effects of anti-TNF-α therapy were investigated at the Inflammatory Bowel Disease Center of Severance Hospital, Seoul, Korea. We also examined the relapse rates and predictive factors for disease relapse. Of the 119 patients, 15 (12.6%) were bio-exposed, 68 (57.1%) received concomitant immunomodulators, and 56 (47.1%) received concomitant corticosteroids at anti-TNF-α treatment induction. At 4, 12, and 24 months, clinical remission rates were 23.5%, 40.3%, and 42.0%; clinical response rates were 84.0%, 62.2%, and 62.2%; and biological response rates were 61.3%, 68.9%, and 58.8%, respectively. Sixty-three patients (52.9%) relapsed, with a mean relapse time of 2.8 years. Higher initial C-reactive protein levels (hazards ratio [HR], 1.013; 95% confidence interval [CI], 1.008 to 1.018; p<0.001), history of previous intestinal surgery (HR, 4.282; 95% CI, 2.379 to 7.709; p<0.001), concomitant immunomodulator use (HR, 0.455; 95% CI, 0.267 to 0.775; p=0.004), and clinical response at 4 months (HR, 0.353; 95% CI, 0.181 to 0.687; p=0.002) were independent factors associated with the disease relapse. No mortality was observed during the study period; 26 (21.8%) and three patients (2.5%) experienced mild infection and infusion reactions, respectively. Anti-TNF-α therapy could be an effective and tolerable option for refractory intestinal BD.
CD39 in the pathogenesis of neuro-Behçet disease: is it an immune regulator marker?
Neuro-Behçet's disease (NBD) is a severe neurological manifestation of Behçet's disease (BD), a multisystemic inflammatory vasculitis affecting multiple organs. NBD presents complex immunopathological features involving both innate and adaptive immune responses. CD39 (ectonucleoside triphosphate diphosphohydrolase-1, ENTPD1), an ectoenzyme expressed on various immune cells, has emerged as a critical regulator of inflammation through its role in hydrolyzing extracellular ATP to immunosuppressive adenosine. This mini-review explores the role of CD39 in modulating neuroinflammation in NBD, focusing on its function in immune cell subsets, its dual role in inflammation and regulation, and its potential as a therapeutic target. It synthesizes the role of CD39 in neuroinflammatory disorders, with a specific focus on Neuro-Behçet's Disease (NBD). We examine the dual role of CD39 expression in driving inflammation and immune regulation, and highlighting its potential as a therapeutic target.
Hughes-Stovin syndrome without peripheral venous thrombosis: Early recognition and successful immunosuppressive management-A case report and brief literature review.
Hughes-Stovin syndrome (HSS) is an exceptionally rare and potentially life-threatening systemic vasculitis characterized by the coexistence of pulmonary artery aneurysms and venous thrombosis. Its striking clinical overlap with Behçet disease has led to ongoing debate regarding whether HSS constitutes a distinct disease entity or instead represents a vascular variant within the Behçet spectrum. In the absence of standardized diagnostic criteria, diagnosis relies primarily on clinical suspicion supported by radiologic evidence. Early identification is crucial, as rupture of pulmonary aneurysms is frequently fatal. We report a young male patient diagnosed with HSS who demonstrated favorable clinical and radiologic improvement following treatment with corticosteroids and mycophenolate mofetil. This case highlights the importance of considering HSS in young individuals presenting with unexplained pulmonary artery aneurysms, even in the absence of venous thrombosis. Prompt diagnosis, early initiation of immunosuppressive therapy, and coordinated multidisciplinary management are essential to mitigate the risk of catastrophic complications. This case is unusual in that HSS occurred in the absence of peripheral venous thrombosis and responded favorably to combined corticosteroid and mycophenolate mofetil therapy.
Safety of Immune Checkpoint Inhibitors in Cancer Patients With Preexisting Autoimmune Vasculitis.
Immune checkpoint inhibitors (ICIs) are effective cancer therapies but often cause serious immune-related adverse events (irAEs). Patients with preexisting autoimmune diseases, including vasculitis, are excluded from trials. We aimed to evaluate the frequency, severity, and outcomes of vasculitis flares and irAEs in this population. We performed a retrospective review of cancer patients with prior vasculitis treated with ICIs at our institution and conducted a literature search for additional cases. Data included baseline features, vasculitis flares, irAEs, treatments, and tumor response. Twenty-five patients were identified (16 from our institution and 9 from the literature). Median age was 71 years; 13 (52%) were female. Vasculitis types included giant cell arteritis (GCA, n = 9), granulomatosis with polyangiitis (GPA, n = 8), eosinophilic granulomatosis with polyangiitis (EGPA, n = 2), leukocytoclastic vasculitis (n = 2), other cutaneous vasculitis (n = 2), and Henoch-Schönlein purpura and Behçet disease (1 each). Cancer types were diverse. Most patients received anti-programmed death-1 monotherapy (n = 17). Eight patients (31%) experienced a vasculitis flare after ICI initiation (2/9 GCA, 4/8 GPA, 2/4 cutaneous vasculitis). Flares occurred after a median of seven weeks. Treatment included glucocorticoids in seven patients, combined with biologic or cytotoxic agents depending on vasculitis type. Seven of eight flares resolved, and four patients continued ICI therapy. Three additional patients developed de novo irAEs: severe hepatitis (n = 1), grade 3 colitis (n = 1), and grade 3 autoimmune hemolytic anemia (n = 1), all improving with treatment. One patient with GCA died due to a flare; no deaths were attributed to de novo irAEs. Overall, more than one-third of patients achieved a favorable tumor response. Nearly one-third of patients with preexisting vasculitis experienced a disease flare during ICI therapy, with one fatal case. For most, outcomes were favorable with effective flare management. Preexisting vasculitis should not be considered an absolute contraindication for cancer immunotherapy with ICI.
Publicações recentes
Recurrent Stroke Presentation in Neuro-Behcet Disease: Clinical Insights from a Case Report.
CARD8 variant-mediated inflammasome activation in a case of refractory Behçet disease: from PFAPA to atypical neurovascular involvement.
Age-Related Differences in the Etiology and Clinical Characteristics of Uveitis in Japan: A Retrospective Comparative Study.
Stroke in autoimmune rheumatic diseases.
Five children with haploinsufficiency of A20 caused by heterozygous mutations in the TNFAIP3 gene.
📚 EuropePMC1.158 artigos no totalmostrando 196
CD39 in the pathogenesis of neuro-Behçet disease: is it an immune regulator marker?
Frontiers in immunologyHughes-Stovin syndrome without peripheral venous thrombosis: Early recognition and successful immunosuppressive management-A case report and brief literature review.
Respiratory medicine case reportsSafety of Immune Checkpoint Inhibitors in Cancer Patients With Preexisting Autoimmune Vasculitis.
ACR open rheumatologyIncidence and Prevalence of Non-Infectious Uveitis: A Systematic Review and Meta-Analysis.
Retina (Philadelphia, Pa.)A Case of Concurrent Behçet Disease and Erythema Multiforme in a Young Adult.
CureusExploratory whole-exome sequencing identifies candidate DNA variants in Ocular Behcet disease: a pilot study from a Pakistani cohort.
Ophthalmic geneticsAssociation between Behçet disease and pregnancy-related outcomes: A systematic review and meta-analysis.
Journal of reproductive immunologyUstekinumab-associated morphoea: systematic review of the literature and a real-world case.
Drugs in contextUnmasking Hemophagocytic Lymphohistiocytosis After Minimal Tumor Necrosis Factor-Alpha (TNF-α) Blockade: An Interplay of Contributing Elements.
CureusA case of intestinal Behçet disease in a deceased donor kidney transplantation recipient.
Clinical transplantation and researchThe Impact of Vitreoretinal Surgery in Patients with Uveitis: Current Strategies and Emerging Perspectives.
Diagnostics (Basel, Switzerland)Behçet-like Syndromes: A Comprehensive Review.
Dermatopathology (Basel, Switzerland)Multiple cerebral venous thrombosis as the first presentation of Behçet syndrome: case report and literature review.
Irish journal of medical scienceLactate dehydrogenase to albumin ratio as a convenient parameter for predicting the severity of Behçet disease: a cross-sectional study.
BMC immunologyManagement of Aortic Aneurysms Associated with Behçet Disease: A Single-Center Experience with Endovascular Repair.
Vascular specialist internationalSingle-Cell Genetic Mapping of Gasdermin Expression Across Immune-Mediated Inflammatory Diseases.
FASEB journal : official publication of the Federation of American Societies for Experimental BiologyClinical Implications of Vasculitis Associated with Familial Mediterranean Fever: A Comparative Study in Childhood.
Modern rheumatology[Role of the SII and SIRI in risk prediction, disease activity assessment, and prog-nostic evaluation of Behçet disease uveitis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesAseptic pyomyositis in Behçet's disease: a case report and narrative review of muscle involvement phenotypes.
Neuromuscular disorders : NMDMonitoring Behçet uveitis.
Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological SocietyBehçet disease: A unique systemic vasculitis with a distinct form of ocular involvement.
Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological SocietyBehçet Disease and Cognitive Impairment: A Case Study of an Overlooked Symptom.
The American journal of case reportsEffectiveness and Tolerability of Anti-Tumor Necrosis Factor Alpha Therapy in Refractory Intestinal Behçet's Disease: A Large Single-Center Study.
Gut and liverRapidly progressive glucocorticoid-resistant childhood-primary angiitis of the central nervous system positive for HLA-B51 and A26: a case report.
Modern rheumatology case reportsThe MAGIC (Mouth and Genital Ulcers With Inflamed Cartilage) of VEXAS: A Case of Overlap Between Behçet Disease and VEXAS Syndrome.
The Journal of rheumatologyCase Report: An endogenous endophthalmitis case caused by Haemophilus influenzae.
Frontiers in medicinePhenotypes of patients with more than one autoinflammatory disease-associated gene variant: overlapping and mixed autoinflammatory disorders.
Rheumatology (Oxford, England)Evaluation of predictors and clinical outcome after tapering of chronic systemic corticosteroids in Behçet's disease patients.
Clinical rheumatologyProton Pump Inhibitor Use and Its Significant Association With an Increased Incidence of Intestinal Behçet Disease.
The Journal of rheumatologyAssociation Between Autoimmune Diseases, Treatments, and Dementia Risk: A Population-Based Case-Control Study From Taiwan.
The Journal of clinical psychiatry[Clinical characteristics of Behçet syndrome in 45 children].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsChallenges in Diagnosing Non-Nasal Natural Killer/T-Cell Lymphoma Mimicking Behçet Disease: A Case Report.
The American journal of case reportsA case of paracentral acute middle maculopathy and retinal vein occlusion: idiopathic occlusive retinal vasculitis or incomplete Behçet's disease?
BMC ophthalmologyA case of Behçet disease complicated with multiple false aneurysms of the aorta.
Journal of vascular surgeryBehçet Disease Uveitis: Insights into Epigenetic and Environmental Factors, Retinal Imaging, and Treatment.
Ocular immunology and inflammationRare presentation of Behçet's disease in a 22-year-old male with combined retinal artery and vein occlusions.
American journal of ophthalmology case reportsThe relationship between NETosis findings and disease activity in Behçet's disease: an exploratory study.
Clinical and experimental rheumatologyClinical trials and quasi-experimental studies in the treatment of noninfectious retinal vasculitis: A systematic review from the International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) - Report 4.
Survey of ophthalmologyThe cascade sign in aquaporin-4-IgG positive (AQP4-IgG+) NMOSD.
Multiple sclerosis (Houndmills, Basingstoke, England)Canadian Rheumatology Association/Canadian Research Group of Rheumatology in Immuno-Oncology Living Guidelines for Baseline Immunosuppression in Individuals With Preexisting Rheumatic Diseases Initiating Immune Checkpoint Inhibitors. Part 2: Preexisting Systemic Autoimmune Rheumatic Diseases.
The Journal of rheumatologyComparative Real-World Effectiveness of Immunomodulatory Therapies for Prevention of Uveitis Relapse in Behçet Disease.
Arthritis & rheumatology (Hoboken, N.J.)Integrated stress response in Behçet disease: expression analyses in peripheral blood and synovial monocytes.
Rheumatology internationalVasculitis as an indicator of disease severity in familial Mediterranean fever.
Frontiers in immunologyNSAID overuse leading to cardiac aneurysm: Unveiling a missed diagnosis of Behçet's disease: A case report.
MedicineMyelodysplastic Syndrome With Trisomy 8 and Behçet Disease-Like Intestinal and Upper Gastrointestinal Involvement.
Clinical case reportsAtypical Presentation of Behçet Disease Unmasked by Acute Coronary Syndrome.
JACC. Case reportsEpidemiology, Clinical Data, and Management of Aseptic Abscess Syndrome: Review of Published Cases Outside France.
Epidemiologia (Basel, Switzerland)Clinical Images: Syphilis as the great imitator; Behçet disease mimic.
Arthritis & rheumatology (Hoboken, N.J.)Predictors of uveitic macular edema and functional prognostic outcomes: real-life data from the international AIDA Network uveitis registry.
Frontiers in medicineInflammatory hematological ratios and subclinical microvascular changes of fundus in Behçet disease.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle OphthalmologieInvestigating the mediating effect of plasma metabolites on the gut microbiome in influencing Behçet disease: A multi-omics validated Mendelian randomization study.
MedicineFunnel-Shaped Vitreous Extension of Retinal Infiltrates in Behcet Disease.
Ophthalmology. RetinaRenal artery thrombosis in Behçet's disease: Case report and literature review.
MedicineStent-Assisted Coil Embolization of Multiple Intracranial Aneurysms Associated With Behçet Disease.
The Journal of craniofacial surgeryExamining Sirtuin-1 Levels and Inflammation Markers in Behcet's Disease: A Pilot Study.
Archives of rheumatologyClinical characteristics of behçet's disease in palestine, a retrospective cohort study.
BMC rheumatologyASSESSMENT OF CLINICAL FEATURES AND PROGNOSIS IN BEHÇET DISEASE PATIENTS WITH OCULAR MANIFESTATIONS: A Focus on Optical Coherence Tomography and Angiography.
Retina (Philadelphia, Pa.)Clinical Images: Appendix vasculitis in a patient with intestinal Behçet disease.
Arthritis & rheumatology (Hoboken, N.J.)Acute Cardiogenic Shock Secondary to Left Main Occlusion in a Patient with Behçet Disease.
JACC. Case reportsPulmonary Artery Involvement and Parenchymal Lung Changes in Behçet Disease: A Comparative Cohort Thoracic Computed Tomography Imaging Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesA life-threatening discovery: How hematemesis unveiled a splenic artery aneurysm in Behçet's disease.
Vascular diseases (Paris, France)Giant Aortic Aneurysm in Refractory Behçet's Disease Shrank with Tocilizumab Treatment: A Case Report.
ACR open rheumatologyOCTA measurements in Behcet's disease across different stages of the disease activity: A systematic review and meta-analysis.
PloS oneThe Immunopathogenesis of Adamantiades-Behçet Uveitis: Advances in Understanding of Genetics and Molecular Mechanisms.
International ophthalmology clinicsINVESTIGATE THE CHANGE OF CHOROIDAL THICKNESS IN PATIENTS WITH COMMON SYSTEMIC AUTOIMMUNE DISEASES: A Review.
Retina (Philadelphia, Pa.)Uncommon Non-MS Demyelinating Disorders of the Central Nervous System.
Current neurology and neuroscience reportsNeurological Behcet's disease with transverse myelitis in an adolescent: A case report.
SAGE open medical case reportsThermal Imaging-Guided Surgical Unroofing of a Myocardial Bridge in a Patient With Refractory Angina.
JACC. Case reportsClinicodemographic Data of Patients with Behçet's Disease: Data from a Tertiary Center in Saudi Arabia.
Current rheumatology reviewsSleep and circadian parameters in Behçet's syndrome: a comparative analysis using actigraphy and questionnaires.
Rheumatology (Oxford, England)Serum immunoreactivity to neurofilament-medium shows high sensitivity and specificity in patients with Behçet disease.
RMD openA 30-year experience in neuro-Behçet disease.
Journal of neuroimmunologySeeking Clarity in the Muddy Waters of Vascular Behçet Management.
European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular SurgeryLong Term Outcomes of Open or Endovascular Treatment for Arterial Manifestations in Behçet Disease.
European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular SurgerySelective immunoglobulin E deficiency and its association with autoimmune and autoinflammatory diseases.
Allergy and asthma proceedingsExpression of miR-127 and miR-29 in Egyptian patients with Behçet disease and its clinical significance and relationship with disease activity.
Clinical rheumatologyLow IL-35 expression in CSF is associated with Neuro-Behcet Disease: Comparative analysis between parenchymal and Non-parenchymal NBD.
Immunology lettersClinical features and differential diagnosis in symptomatic localized terminal ileitis or ulcer.
MedicineIdentification of markers predicting clinical course in patients with Behcet disease by combination of machine learning and unbiased clustering analysis.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle OphthalmologieRisk of Autoimmune Rheumatic Diseases in Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder: A Nationwide Cohort Study in South Korea.
Mayo Clinic proceedingsOphthalmic Posterior Segment OCTA Metrics as Potential Biomarkers for Systemic Involvement in Systemic Sclerosis, Systemic Lupus Erythematosus, and Behçet Disease: A Systematic Review.
Open access rheumatology : research and reviewsSerum levels of miR-34, miR-182 and miR-378 as novel diagnostic biomarkers in Behçet patients and their relation to disease activity and severity.
Archives of physiology and biochemistrySevere Alveolar Bone Loss as a Rare Manifestation of Suspected Intestinal Behçet Disease: A Case Report.
Mayo Clinic proceedings. Innovations, quality & outcomesChanges in the metabolome, lipidomein, and gut microbiota in Behçet's disease.
Frontiers in cell and developmental biologyPatients with vasculitis present a poor oral health: results of the online cross-sectional survey from Canada (VASC-TOOTH Survey).
Rheumatology internationalHerpetiform ulceration in Behçet disease.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneEarly atypical MRI findings in a pediatric patient with Neuro-Behçet's disease.
BMC neurologyIntracardiac Thrombus Resulting in Multiple Cardiovascular Complications as a Manifestation of Behçet Disease: A Case Report.
CJC openHighlights from the breakout session: management of special situations in systemic vasculitis.
Rheumatology (Oxford, England)Endomyocardial Fibrosis Mimicking a Heart Tumor and Dysplastic Tricuspid Valve in a Teenager: A Rare Cardiac Manifestation of Behçet Disease.
Circulation. Cardiovascular imagingTurmeric-Induced Liver Injury.
Journal of Brown hospital medicineFrosted branch angiitis post COVID vaccine: A presumptive association with Behçet's disease.
European journal of ophthalmologyTherapeutic Potential of Janus Kinase Inhibitors for Treating Refractory Behçet Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesGlobal demographic and etiological variations of retinal vasculitis: A systematic review and meta-analysis: International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) Report 1.
Survey of ophthalmologyBehcet syndrome: A rare cause of recurrent genital ulceration.
Indian journal of sexually transmitted diseases and AIDSImaging of Pulmonary Vasculitis.
Radiologic clinics of North AmericaCase report: Adult case of A20 haploinsufficiency suspected as neuro-Behçet disease.
Frontiers in immunologyNeuro-Behcet's Masquerading as Status Epilepticus and Meningoencephalitis in the Emergency Department.
The Journal of emergency medicineAccuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes: A Discovery and Validation Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAcute macular neuroretinopathy occurrence in a Behçet disease patient: a case report.
Journal of ophthalmic inflammation and infectionTeaching NeuroImage: Atypical Presentation of HLA-B51 Positive Neuro-Behçet Disease in a 27-Year-Old Patient: The Waterfall Sign.
Neurology[Clinical significance of antiphospholipid antibodies in Behcet disease with thrombosis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesComparative Study of Adalimumab, Infliximab and Certolizumab Pegol in the Treatment of Cystoid Macular Edema Due to Behçet's Disease.
Journal of clinical medicineBehcet disease presenting as large and medium vessel occlusive disease.
Indian journal of thoracic and cardiovascular surgeryContraceptives or Hormone Replacement Therapy and Associations with Autoimmune Conditions: Exploring Effects of Estrogen Analog Supplementation.
ACR open rheumatologyUveitis in paediatric Behçet disease: a large multicentric Italian cohort.
Therapeutic advances in musculoskeletal diseaseFunctional and anatomic outcomes of pars plana vitrectomy for macular holes in ocular Behçet's disease.
Journal francais d'ophtalmologieNeuro-Behçet leading to coma: A case report.
Radiology case reportsLong-Term Risk of Autoimmune and Autoinflammatory Connective Tissue Disorders Following COVID-19.
JAMA dermatologyVasculo-Behçet disease with venous thromboembolism successfully treated with direct oral anticoagulants: A literature review.
The Journal of dermatologyIntestinal vascular diseases misdiagnosed as Crohn disease: analysis of 13 cases.
Pathology"Lupus Myelitis" Revisited: A Retrospective Single-Center Study of Myelitis Associated With Rheumatologic Disease.
Neurology(R) neuroimmunology & neuroinflammationRheumatic diseases detected in patients presenting with uveitis.
Northern clinics of IstanbulAn Australian Shepherd with possible Behçet-like disease characterized by cutaneous and intestinal vasculitis leading to septic abdomen.
Journal of the American Veterinary Medical AssociationThe Current and Novel Imaging Modalities for Ocular Vasculitis in Behcet's Disease: A Review.
CureusInternal Jugular Vein Thrombosis as the Initial Presentation of Antiphospholipid Antibody-positive Vascular Behçet Disease: A Clinical Image.
Journal of community hospital internal medicine perspectivesEnigmatic odyssey: A case of Behcet disease with atypical thrombotic complications.
International journal of surgery case reportsCytokine Signature Differences in Major Phenotypic Groups of Behçet Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesSuccessful treatment of Sweet syndrome complicated by Behcet disease with adalimumab.
Pediatrics international : official journal of the Japan Pediatric SocietyNeuro-Behçet Disease Presenting as a Bulbar Lesion: A Case Report.
CureusMultiple Conjunctival Ulcers in Behçet Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesThe association between Behçet disease activity and elevated systemic immune-inflammation index: A retrospective observational study in a tertiary care hospital.
The National medical journal of IndiaSevere sepsis induced by zoledronic acid: A case report.
MedicineEvaluation of right ventricular function in patients with Behcet's disease by four-dimensional echocardiography.
Echocardiography (Mount Kisco, N.Y.)Severe Occlusive Retinal Vasculitis in an Immunocompetent Patient with Chronic CMV Anterior Uveitis.
Ocular immunology and inflammationBehçet disease: epidemiology, classification criteria and treatment modalities.
Expert review of clinical immunologyAssociation Between Proton Pump Inhibitors and the Risk of Intestinal Behçet Disease.
The Journal of rheumatologyBiology of HLA class I associated inflammatory diseases.
Best practice & research. Clinical rheumatologySaliva assay: a call for methodological standardization.
Journal of periodontal & implant scienceThe clinical manifestations and treatment outcomes of Behçet's disease: A single-center experience.
Health science reportsTreatment of Behçet disease with oral roflumilast: an observational study.
Clinical and experimental dermatologySuccessful Intrasaccular Microcoil Embolization of a Lower Segment Pulmonary Artery Aneurysm in a Patient With Behcet's Disease.
CureusIntestinal ultrasound for intestinal Behçet disease reflects endoscopic activity and histopathological findings.
Intestinal researchApplication of Ophthalmic Electrophysiology in Inflammatory Disorders of Retina and Optic Nerve.
Journal of clinical medicineExploring Headaches in Pediatric Behçet Disease: Prevalence, Clinical Impact, and Management.
Journal of clinical medicineSurgical Intervention for Giant Pulmonary Artery Aneurysm in Behcet Disease: A Case Report.
The American journal of case reportsWhole phenotype of patients with systemic sclerosis and sicca manifestations: Comparison with sicca manifestations from other causes.
Seminars in arthritis and rheumatismRecurrent Oral Ulcers: Are They Horses or Zebras?
CureusIncreased Risk of Preterm Delivery Subtypes and Hypertensive Disorders of Pregnancy in First Deliveries of Patients With Systemic Vasculitis.
ACR open rheumatologyRole of Optical Coherence Tomography in Vasculitis-Associated Pulmonary Hypertension and Chronic Thromboembolic Pulmonary Hypertension.
Circulation journal : official journal of the Japanese Circulation SocietyNeuro-Behçet's disease: A review.
La Revue de medecine interneRecurring ST-Elevation Myocardial Infarction With No-Reflow Caused by Hypercoagulable State: A Behcet Disease Case Report.
Journal of clinical medicine researchThe Spectrum of Papillophlebitis.
Ocular immunology and inflammationExploring Reproductive Experiences With Women Enrolled in the International Vasculitis Pregnancy Registry.
The Journal of rheumatologyAssociation of high disease activity and serum IL-6 levels with the incidence of inflammatory major organ events in Behçet disease: a prospective registry study.
Frontiers in immunologyNeuro-Behcet's Disease and Its Association With Cerebral Aneurysms and Subarachnoid Hemorrhage: A Case Report.
CureusImmune Ataxias: The Continuum of Latent Ataxia, Primary Ataxia and Clinical Ataxia.
Journal of integrative neuroscienceIncreased IL-22 in cerebrospinal fluid of neuro-behçet's disease patients.
CytokineValue of Serum Asymmetric Dimethylarginine (ADMA) As a Novel Biomarker for Uveitis in Behçet's Disease.
Ocular immunology and inflammationThe Frequency of Intraocular Pressure Elevation, Incidence of Secondary Glaucoma, and Surgical Treatment With Postoperative Complications in Pediatric and Adult Patients With Uveitis.
CureusThe clinical and radiological features and prevalence of Neuro-Behçet's Disease: A retrospective cohort multicenter study in Saudi Arabia.
Multiple sclerosis and related disordersA Rare but Fatal Behçet Variant: The Hughes-Stovin Syndrome-Successful Case Report and New Evidence from Literature Review.
Aorta (Stamford, Conn.)A Case Report of Neuro-Behçet Syndrome: Frequent Neurological Manifestations Concurrent With Life-Threatening Illnesses.
CureusFolliculitis might be associated with pathergy-positivity in patients with Behçet syndrome.
MedicineFrosted branch angiitis in Behcet disease.
QJM : monthly journal of the Association of PhysiciansMortality landscape of Behçet disease.
The British journal of dermatologyRevealing Academic Evolution and Frontier Pattern in the Field of Uveitis Using Bibliometric Analysis, Natural Language Processing, and Machine Learning.
Ocular immunology and inflammationPediatric Neutrophilic Dermatoses.
Dermatologic clinicsCharacterization and Management of Amicrobial Pustulosis of the Folds, Aseptic Abscess Syndrome, Behçet Disease, Neutrophilic Eccrine Hidradenitis, and Pyostomatitis Vegetans-Pyodermatitis Vegetans.
Dermatologic clinicsBehçet Disease Periaortic Pseudoaneurysms Connect with Pulmonary Artery.
RadiologyMaternal and fetal outcomes of pregnancy in women with primary systemic vasculitis: A single-center cohort study of 20 patients and 30 pregnancies.
Seminars in arthritis and rheumatismHLA-B*51 Frequency in Transplant Patients and Donors.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ TransplantationEffect of adalimumab on choroidal thickness and choroidal vascularity index in eyes with non-infectious uveitis using enhanced-depth imaging optical coherence tomography.
Eye (London, England)Neuro-Behçet Disease Presenting with Wall-Eyed Bilateral Internuclear Ophthalmoplegia Syndrome: A Case Report.
Korean journal of ophthalmology : KJOBehcet disease: A case report on the utilization of T1-weighted black blood fat-suppressed sequences for the detection of venous vasculitis.
Clinical case reportsInnominate artery aneurysm in behcet disease; report of one case.
International journal of surgery case reportsAll-cause and cause-specific mortality in patients with Behçet disease versus the general population.
The British journal of dermatologyClinical and anamnestic features of patients with systemic vasculitis: a single-center retrospective study.
Rheumatology internationalIL-38 in Behçet's disease: Gene expression in bronchoalveolar lavage from patients having pulmonary involvement.
Immunology lettersUlcus Vulvae Acutum Lipschütz-UVAL: case series at a Swiss university hospital emergency room.
Frontiers in reproductive healthHughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis.
Mediterranean journal of rheumatology[Translated aticle] Topical and Oral Roflumilast in Dermatology: A Narrative Review.
Actas dermo-sifiliograficas[Diagnostic modalities of a Behçet uveitis].
Revue medicale de LiegeAssociation of endomyocardial fibrosis and minor myocarditis sequelae with intracardiac thrombus and Ebstein like valvulopathy in a patient with Behçet disease: a case report.
European heart journal. Case reportsAmyloid Goiter Secondary to Behcet's Disease: A Case Report and Literature Review.
CureusPulmonary Artery Aneurysm in Behcet Disease: Medical, Endovascular or Surgical Intervention.
CureusRecurrent Stroke as a First Presentation in Behçet Disease: A Case Report.
CureusRecent Advances in Immune-Mediated Cerebellar Ataxias: Pathogenesis, Diagnostic Approaches, Therapies, and Future Challenges-Editorial.
Brain sciencesLife-Threatening Involvement of Bilateral Pulmonary Arteries in Behçet Disease on FDG PET.
Clinical nuclear medicineThoughts and Experiences of Behçet Disease From Participants on a Reddit Subforum: Qualitative Online Community Analysis.
JMIR formative researchOsteonecrosis as a rare musculoskeletal complication in Behcet's disease- the largest case series with literature review.
BMC rheumatologyResuming anti-TNF therapy after development of miliary tuberculosis in Behcet's disease-related uveitis: a case report.
Journal of ophthalmic inflammation and infectionOcular involvement in pediatric Behçet's disease: is it different than in adults? (a short case series and mini review).
BMC ophthalmologyAssociation of IL-23R and IL-10 variations with Behçet disease: a genetic analysis study.
Immunologic researchRefractory Intestinal Behçet-Like Disease Associated with Trisomy 8 Myelodysplastic Syndrome Resolved by Parenteral Nutrition.
Case reports in gastroenterologyEpidemiology of Pediatric Uveitis.
Ocular immunology and inflammationSurgical intervention and management of Behcet disease with perianal abscess: A case report.
Asian journal of surgeryNeuro-Behçet With a Thalamic Lesion: A Case Report.
CureusCentral retinal artery occlusion after spinal surgery: Case report and literature review.
European journal of ophthalmologyIleocecal involvement in intestinal Behçet's disease and Crohn's disease: comparison of clinicopathological and immunophenotypic features.
Journal of digestive diseasesNeurologic manifestations of Behçet disease: rheumatology experience.
Zeitschrift fur RheumatologieIndications for Magnetic Resonance Imaging in Patients With Behcet Uveitis.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology SocietyInferior Vena Cava Thrombosis and Pulmonary Embolism in a Patient With Behcet Disease: A Rare Presentation.
CureusDiagnosis and management of Neuro-Behçet disease with isolated intracranial hypertension: a case report and literature review.
BMC neurologyChoroidal vascularity index and submacular choroidal thickness in patients with Behçet disease assessed with enhanced-depth OCT.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologieHLA Alleles in a Behçet Disease Multiethnic Population With and Without Ophthalmic Manifestations.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAssociações
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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.
- Multiple cerebral venous thrombosis as the first presentation of Behçet syndrome: case report and literature review.
- Effectiveness and Tolerability of Anti-Tumor Necrosis Factor Alpha Therapy in Refractory Intestinal Behçet's Disease: A Large Single-Center Study.
- CD39 in the pathogenesis of neuro-Behçet disease: is it an immune regulator marker?
- Hughes-Stovin syndrome without peripheral venous thrombosis: Early recognition and successful immunosuppressive management-A case report and brief literature review.
- Safety of Immune Checkpoint Inhibitors in Cancer Patients With Preexisting Autoimmune Vasculitis.
- Recurrent Stroke Presentation in Neuro-Behcet Disease: Clinical Insights from a Case Report.
- CARD8 variant-mediated inflammasome activation in a case of refractory Behçet disease: from PFAPA to atypical neurovascular involvement.
- Age-Related Differences in the Etiology and Clinical Characteristics of Uveitis in Japan: A Retrospective Comparative Study.
- Stroke in autoimmune rheumatic diseases.
- Five children with haploinsufficiency of A20 caused by heterozygous mutations in the TNFAIP3 gene.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:117(Orphanet)
- OMIM OMIM:109650(OMIM)
- MONDO:0007191(MONDO)
- Doenca de Behcet(PCDT · Ministério da Saúde)
- GARD:848(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q911427(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
