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Published on: February 8, 2019
Current status of Takayasu arteritis in India
S Jain1, S Kumari, N K Ganguly
1Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Insights
This study documents 106 Takayasu arteritis (TA) patients, finding hypertension common. It also reveals protein kinase C (PKC) pathway activation in TA pathogenesis, suggesting a new therapeutic target.
Area of Science:
- Cardiovascular Medicine
- Immunology
- Genetics
Background:
- Takayasu arteritis (TA) is a rare, chronic inflammatory disease affecting large arteries, primarily the aorta and its branches.
- Understanding the clinical spectrum and pathogenesis of TA is crucial for effective management.
Purpose of the Study:
- To document the clinical features, disease classification, treatment modalities, and pathogenetic insights of Takayasu arteritis (TA) in a cohort of 106 patients.
- To investigate the role of protein kinase C (PKC) and intracellular calcium signaling in TA pathogenesis.
Main Methods:
- Retrospective analysis of clinical data from 106 TA patients over 16 years.
- Classification of TA based on aortography findings (Types I-V).
- Assessment of T cell signaling pathways, including protein kinase C (PKC), intracellular calcium, and inositol 1,4,5 triphosphate levels.
Main Results:
- Hypertension was the most frequent presentation (51.3%), with 77.4% of patients diagnosed at presentation.
- Type V TA (involving multiple aortic segments) was most common (55.7%).
- Elevated protein kinase C (PKC) activity, intracellular calcium, and inositol 1,4,5 triphosphate were observed in T cells of TA patients, suggesting pathway activation.
Conclusions:
- Takayasu arteritis (TA) presents with diverse clinical features, hypertension being predominant.
- The study provides evidence for the activation of the PKC-calcium pathway in the pathogenesis of TA.
- These findings may offer novel therapeutic targets for managing Takayasu arteritis.
Abstract:
The clinical features of 106 patients of Takayasu arteritis (TA) seen over a period of 16 years are documented (65 females and 41 males). The mean age was 27.3 +/- 9.2 years. Hypertension was the commonest mode of presentation (51.3%) and was detected in 82 patients (77.4%) at the time of presentation. Vascular bruits were heard in 72 patients (67.9%) and 13 patients (12.3%) were in congestive heart failure. Aortography was performed in 95 patients. Based on the extent of involvement, Type I (branches of aortic arch) was seen in 7 (6.6%) patients, Type II (aortic arch, its branches and descending thoracic aorta) in 7 (6.6%) patients, Type III (descending thoracic aorta and abdominal aorta) in 4 (3.8%) patients, Type IV (abdominal aorta only) in 29 (27.3%) patients and Type V (aortic arch, descending thoracic aorta and abdominal aorta) in 59 (55.7%) patients. Therapeutic modalities included antihypertensive drug therapy in 81 patients, antitubercular drugs in 8 patients, steroids in 16 patients and cyclophosphamide in one patient. Response to steroids was satisfactory in 5 of these 16 patients while the lesions of vasculitis healed in the patient who was treated with cyclophosphamide. Surgical interventions included nephrectomy and autotransplantation of kidney in 3 patients each and revascularization in 4 patients and angioplasty in 4 patients. In the area of pathogenesis of this disease, a high activity of protein kinase C(PKC), an increased intracellular calcium and inositol 1,4,5 triphosphate in both unstimulated and stimulated T cells of TA was observed. These findings suggest an activation of PKC-calcium pathway in TA.
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