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Clinical presentation and treatment outcomes of arterial involvement in Behçet’s disease: a single-centre experience

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Abstract

Arterial involvement, although rare, accounts for significant mortality and morbidity in patients of Behçet’s disease (BD). There is paucity of data on arterial BD. The objective of this 5-year retrospective cohort study was to examine the clinical presentation, pattern of arterial involvement, and treatment outcome in Indian arterial BD patients. Data on demography, clinical presentation, radiology, instituted therapy, vascular interventions and treatment outcomes were recorded and analyzed. Ten (16.9%) out of 59 patients with BD had arterial involvement in 13 vascular territories [mean age 30 (8) years, 9 (90%) males]. Pulmonary artery was most commonly involved (46%), followed by abdominal aorta (15%), femoral artery (15%), descending thoracic aorta (8%), common iliac (8%), and dorsalis pedis artery (8%). Two patients had multi-territory involvement. The median interval between disease onset and development of arterial aneurysms was 3 years (3 months–12 years). Concomitant deep vein thrombosis was seen in 60% cases. Prednisolone and cyclophosphamide were the most common immunosuppressive therapy used; one patient who relapsed on cyclophosphamide responded to infliximab. Five surgical or endovascular interventions were performed. Four patients (40%) died due to aneurysm rupture—all had a delayed diagnosis, and three had pulmonary artery involvement, with death due to massive hemoptysis. Based on the present study, we concluded that arterial involvement in BD is seen predominantly in males and has a high mortality. Early detection and aggressive treatment with immunosuppression and surgical or endovascular interventions are essential for good outcomes.

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All data generated or analysed during this study are included in this published article.

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Funding

No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.

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Author notes

  1. Arghya Chattopadhyay and Siddharth Jain contributed equally as first authors.

Authors and Affiliations

  1. Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Arghya Chattopadhyay, Siddharth Jain, G. S. R. S. N. K. Naidu, Varun Dhir, Nupoor Acharya, Sanjay Jain & Aman Sharma

  2. Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Bharath Chhabria & Valliappan Muthu

  3. Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Manphool Singhal

  4. Department of General Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Ajay Savlania

  5. Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Rajesh Vijayvergiya

Authors
  1. Arghya Chattopadhyay
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  2. Siddharth Jain
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  3. G. S. R. S. N. K. Naidu
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  4. Varun Dhir
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  10. Rajesh Vijayvergiya
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  11. Sanjay Jain
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  12. Aman Sharma
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Contributions

AC and AmS conceptualized the study. AC, SiJ, BC and NA collected, analysed and interpreted the data. AC, SiJ and AmS drafted the manuscript. GN, VD, VM, AjS, RV and SaJ provided critical, intellectual inputs on the same. MS interpreted and analysed the radiological data. AjS was involved in surgical management, while RV performed percutaneous interventions. All co-authors approved the final submitted version of the manuscript. All co-authors take full responsibility for all aspects of the work and meet all four criteria for authorship recommended by the ICMJE.

Corresponding author

Correspondence to Aman Sharma.

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Conflict of interest

The authors declare no conflicts of interest.

Ethics approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Institutional Ethics Committee of Postgraduate Institute of Medical Education and Research, Chandigarh, India (INT/IEC/2021/SPL-126, dated 22.01.2021).

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Informed consent was obtained from all individual participants included in the study.

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Chattopadhyay, A., Jain, S., Naidu, G.S.R.S.N.K. et al. Clinical presentation and treatment outcomes of arterial involvement in Behçet’s disease: a single-centre experience. Rheumatol Int 42, 115–120 (2022). https://doi.org/10.1007/s00296-021-05022-y

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  • DOI: https://doi.org/10.1007/s00296-021-05022-y

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