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Exploring clinical features and therapeutic outcomes in Indian children with mixed connective tissue disease: A
Suparna Guha1, Deepti Suri2, Suma Balan3
1Pediatric Rheumatology, VIMS & RKMSP, Kolkata, India.
Insights
This study details juvenile-onset mixed connective tissue disease (jMCTD) in India, highlighting common symptoms like arthritis and rash. Findings aim to improve understanding and care for this rare pediatric condition.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Rare Pediatric Conditions
Background:
- Juvenile-onset mixed connective tissue disease (jMCTD) is a rare autoimmune disorder in children.
- Limited data exists on jMCTD, particularly from Southeast Asia.
- Understanding jMCTD's characteristics is crucial for early diagnosis and management.
Purpose of the Study:
- To characterize the clinical and laboratory features of jMCTD in Indian children.
- To provide insights into the presentation and progression of jMCTD.
- To establish a foundation for improved patient care strategies.
Main Methods:
- A multi-center study involving 11 pediatric rheumatology centers across India.
- Data collected using a pre-designed case proforma.
- Clinical and laboratory data of diagnosed jMCTD patients were compiled and analyzed.
Main Results:
- Thirty-one patients (27 females, 4 males) with jMCTD were analyzed.
- Common manifestations included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%).
- At a mean follow-up of 43 months, 45% achieved remission; two deaths occurred due to macrophage activation syndrome and sepsis.
Conclusions:
- This represents the largest multicenter experience of jMCTD from the Indian subcontinent.
- The findings offer critical insights into jMCTD's complexities.
- This study serves as a vital step towards enhancing jMCTD patient care and management.
Introduction:
Mixed 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.
Objectives:
To describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India.
Methods:
A 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.
Results:
Thirty-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.
Conclusion:
We 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.
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