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A rare inflammatory large-vessel vasculitis primarily affecting the aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm.
Features include very common findings: Arteritis, Abnormal heart valve (abnormal heart valve morphology), Weight loss, and Fever and others; and common findings: Hypertension, Seizure, Muscle weakness, and Joint inflammation (arthritis) and others. 54 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Heart and blood vessels | 13 | Abnormal heart valve (abnormal heart valve morphology), Hypertensive crisis, Hypertension |
Biomarker and diagnostic research for Takayasu arteritis has been reported in the published literature.
No approved treatments are currently available for Takayasu arteritis. An additional 2 compounds hold orphan drug designation.
While no drugs are FDA-approved specifically for Takayasu arteritis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for Takayasu arteritis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
Phenotype severity distribution: 9 very common features, 24 common features.
Estimated prevalence: 1-9 in 100,000 (Uncommon).
32 clinical trials registered, 19 recruiting. Interventions under study include other interventions, drug therapy, medical devices, and biologic therapy. Pipeline includes 7 PHASE4, 1 PHASE3, 2 PHASE2. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT02967068](https://clinicaltrials.gov/study/NCT02967068) |
Data assembled from 7 of 12 sources · Last updated Sep 19, 2026, 8:57 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Brain and nerves | 8 | Fatigue, Seizure, Migraine |
Blood and immune system | 2 | Vasculitis, Low red blood cell count (anemia) |
Muscles | 2 | Muscle weakness, Myalgia |
Bones and joints | 2 | Joint inflammation (arthritis), Arthralgia |
Kidneys and urinary system | 2 | Renal artery stenosis, Ascending tubular aorta aneurysm |
Digestive system | 2 | Anorexia, Gastrointestinal infarctions |
Lungs and breathing | 2 | High blood pressure in lung arteries (pulmonary arterial hypertension), Dyspnea |
Skin | 2 | Skin ulcer, Erythema nodosum |
Eyes | 2 | Damage to the retina (retinopathy), Visual impairment |
Growth and development | 1 | Weight loss |
Metabolism | 1 | Fever |
Ears | 1 | Vertigo |
Lab test results | 1 | Elevated CRP (inflammation marker) (elevated circulating c-reactive protein concentration) |
Designated
Exclusivity End |
|---|
Designation Status |
|---|
tocilizumab | tocilizumab | ODDIFACT SAS | 2025 | — | Designated |
adalimumab | adalimumab | ODDIFACT SAS | 2025 | — | Designated |
32 trials found
VCRC Tissue Repository |
— |
University of Pennsylvania |
RECRUITING |
[NCT05102448](https://clinicaltrials.gov/study/NCT05102448) | Comparison of Tofacitinib and Methotrexate in Takayasu's Arteritis | PHASE4 | Shanghai Zhongshan Hospital | RECRUITING |
[NCT03372980](https://clinicaltrials.gov/study/NCT03372980) | A Registry Study on Biomarkers of Takayasu's Arteritis (ARSBTA) | — | Beijing Institute of Heart, Lung and Blood Vessel Diseases | RECRUITING |
[NCT06887062](https://clinicaltrials.gov/study/NCT06887062) | Dapagliflozin and Endothelin Receptor Antagonism in Large Vessel Vasculitis (DERAIL-LVV) | PHASE2 | University of Edinburgh | RECRUITING |
[NCT07184814](https://clinicaltrials.gov/study/NCT07184814) | Clinical Features and Prognosis of Takayasu's Arteritis With Pulmonary Arteries Involvement | — | China-Japan Friendship Hospital | RECRUITING |
325 publications have been identified in PubMed for Takayasu arteritis. Kisho has analyzed 220 by research type. Research spans Review / Meta-Analysis (25%), Case Report / Case Series (19%), and Epidemiology / Natural History (19%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 54 | 25% |
Patient case studies | 42 | 19% |
Disease patterns and progression | 42 | 19% |
Clinical study results | 26 | 12% |
Testing and diagnosis research | 25 | 11% |
Laboratory research | 23 | 10% |
Other research | 8 | 4% |
Moore KE (2026). [PMID: 41582886](https://pubmed.ncbi.nlm.nih.gov/41582886/). *Mod Rheumatol Case Rep*. [Case Report / Case Series]
Shirai T (2026). [PMID: 41692437](https://pubmed.ncbi.nlm.nih.gov/41692437/). *Circ J*. [Review / Meta-Analysis]
Freitas de Aguiar M (2026). [PMID: 41923366](https://pubmed.ncbi.nlm.nih.gov/41923366/). *Rheumatology (Oxford)*. [Other]
Avcu A (2026). [PMID: 40974164](https://pubmed.ncbi.nlm.nih.gov/40974164/). *Rheumatology (Oxford)*. [Epidemiology / Natural History]
Kwon OC (2026). [PMID: 41560383](https://pubmed.ncbi.nlm.nih.gov/41560383/). *Yonsei Med J*. [Basic Science / Preclinical]
Sugiyama T (2026). [PMID: 40931925](https://pubmed.ncbi.nlm.nih.gov/40931925/). *Immunol Med*. [Diagnostic / Biomarker]
Espinosa-Bautista F (2026). [PMID: 42079604](https://pubmed.ncbi.nlm.nih.gov/42079604/). *Front Immunol*. [Epidemiology / Natural History]
Bulut Gökten D (2026). [PMID: 41872661](https://pubmed.ncbi.nlm.nih.gov/41872661/). *Clin Rheumatol*. [Review / Meta-Analysis]
Armstrong A (2026). [PMID: 41587271](https://pubmed.ncbi.nlm.nih.gov/41587271/). *Circulation*. [Clinical Trial Publication]
Tomelleri A (2026). [PMID: 41702323](https://pubmed.ncbi.nlm.nih.gov/41702323/). *Semin Arthritis Rheum*. [Epidemiology / Natural History]
AI-curated news mentioning Takayasu arteritis
Updated Sep 15, 2026
A case report highlights severe aortic regurgitation as the initial manifestation of Takayasu arteritis in a young woman. This finding may enhance understanding of the disease's presentation and aid in early diagnosis.
A recent case report highlights a rare instance of refractory Kawasaki disease in infants, complicated by giant coronary artery aneurysms and Takayasu arteritis. This literature review provides insights into the complexities of managing such cases.
A recent case report details a four-decade follow-up of a patient with childhood-onset Takayasu arteritis, highlighting extensive vascular involvement. This long-term observation provides valuable insights into the disease's progression and management.