Exploring clinical features and therapeutic outcomes in Indian children with mixed connective tissue disease: A multicenter study

Author:

Guha Suparna1,Suri Deepti2,Balan Suma3,Janarthanan Mahesh4,Agarwal Manjari5,Viswanathan Vijay6,Gupta Aman7ORCID,Hazarika Rashna Dass8,Gummadi Anjani9,Sudhakar Murugan10,Pal Samar Ranjan11,Raghuram Jyothi12,Rao Anand Prahalad13ORCID,Singh Neha14ORCID,Aggarwal Amita15ORCID,Bhattad Sagar14ORCID

Affiliation:

1. Pediatric Rheumatology VIMS & RKMSP Kolkata India

2. Department of Pediatrics, Pediatric Allergy Immunology Unit PGIMER Chandigarh India

3. Department of Rheumatology AIMS Kochi India

4. Department of Clinical Immunology & Rheumatology Sri Ramachandra Institute of Higher Education and Research Chennai India

5. Paediatric Rheumatology Sir Ganga Ram Hospital New Delhi India

6. Paediatric Rheumatology Jupiter Hospital Thane India

7. Pediatric Rheumatology, Immunology & Allergy MEDENS Hospital Panchkula India

8. Department of Paediatric Disciplines Health City Hospital Guwahati India

9. Pediatric Immunology and Rheumatology Ankura Hospitals for Women and Children Hyderabad India

10. Division of Pediatric Rheumatology, Department of Pediatrics Christian Medical College Vellore India

11. Department of Rheumatology RKMSP & VIMS Kolkata India

12. Pediatrics and Pediatric Rheumatology Aster Women and Children's Bangalore India

13. Pediatric Rheumatology Clinics Manipal Hospital Bangalore India

14. Pediatric Immunology and Rheumatology, Department of Pediatrics Aster CMI Hospital Bangalore India

15. Department of Clinical Immunology & Rheumatology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow India

Abstract

AbstractIntroductionMixed connective tissue disease (MCTD) is a rare entity in children. There is a paucity of studies on juvenile‐onset MCTD (jMCTD) worldwide especially from Southeast Asia.ObjectivesTo describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India.MethodsA predesigned detailed case proforma in an excel format was prepared and was sent to all the Pediatric Rheumatology centers in India. Eleven centers provided the clinical and laboratory data of their jMCTD patients, which was then compiled and analyzed in detail.ResultsThirty‐one jMCTD patients from 11 centers were included in the study. Our cohort had 27 females and four male patients over 12 months (August 2021 to July 2022). The median age at presentation was 12 years (range 5–18 years) and the median duration of symptoms was 24 months at diagnosis (range 2–96 months). The common features included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%). At a mean follow‐up of 43 months (range 1–168 months), 45% of them were in remission. There were two deaths reported, due to macrophage activation syndrome and sepsis respectively.ConclusionWe present the largest multicenter experience on jMCTD from the Indian subcontinent. The study's findings serve as a crucial stepping stone toward unraveling the complexities of jMCTD and improving patient care and management strategies.

Publisher

Wiley

Reference24 articles.

1. Mixed connective tissue disease-an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA)

2. Mixed connective tissue disease: A clinicopathologic study of 20 cases

3. Long-term outcome in mixed connective tissue disease: Longitudinal clinical and serologic findings

4. Clinical features of patients with juvenile onset mixed connective tissue disease: analysis of data collected in a nationwide collaborative study in Japan;Kotajima L;J Rheumatol,1996

5. Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland;Pelkonen PM;J Rheumatol,1994

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Juvenile‐onset mixed connective tissue disease from a different perspective;International Journal of Rheumatic Diseases;2024-08

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