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Paediatric Rheumatology

 

Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. ankurjindal11@gmail.com
  2. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  3. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  4. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  5. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  6. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  7. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  8. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  9. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  10. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  11. Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

CER13582
2021 Vol.39, N°4 ,Suppl.131
PI 0149, PF 0156
Paediatric Rheumatology

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PMID: 34251299 [PubMed]

Received: 18/05/2020
Accepted : 21/10/2020
In Press: 06/07/2021
Published: 28/07/2021

Abstract

OBJECTIVES:
To describe the clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma (JSSc) from a tertiary care referral hospital in North-West India.
METHODS:
A review of case records was performed and children with JSSc (disease onset <14 years of age) were analysed. Diagnosis was based on the Paediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc.
RESULTS:
Forty patients (28 girls and 12 boys; F:M ratio= 2.3:1) were diagnosed with JSSc (including 22 children with overlap) in the last 25 years. Mean age at symptom onset was 7.75±3.19 years with a mean delay in diagnosis of 2.275±2.09 years. Raynaud’s phenomenon was seen in 26/40 (65%) patients at presentation. Lung involvement was noted in 40% patients. Methotrexate was the most commonly used therapy, followed by oral prednisolone. Patients without overlap had higher incidence of cutaneous ulcers as compared to patients with overlap (55% vs. 18%; p-value: 0.01). Patients with overlap required significantly higher oral prednisolone (81% vs. 22%), methotrexate (72% vs. 38%) and hydroxychloroquine (54% vs. 5%) while cyclophosphamide (13% vs. 44%) and azathioprine (9% vs. 44%) were used relatively less in this group. Mortality was 15% at a mean follow-up of 51.75 months. Infections were noted to be the most common cause of death. There was no significant difference in the mortality between patients with and without lung disease or patients with or without overlap.
CONCLUSIONS:
We describe the largest single-centre cohort with longest follow-up of juvenile systemic scleroderma from India.

Rheumatology Article