Skip to main content

Advertisement

SpringerLink
Log in

Juvenile systemic sclerosis: experience from a tertiary care center from India

  • Epidemiology of RMD
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms). Controlled: ability to perform ADL and absence of B features for at least 6 months. Partly controlled: inability to perform ADL or any of the B features. Non-responsive/progressive disease: presence of both A and any of B features. Thirty-two children (21, girls) diagnosed as systemic sclerosis for whom follow-up of more than 6 months was available were included for this retrospective analysis. Mean (SD) age at presentation was 112.79 (30.05) months, while the median (IQR) delay in diagnosis was 28.5 (9–47.25) months. Of the 32 children 17 (53.12%) had diffuse systemic sclerosis (dSSc), 5 (15.62%) had limited systemic sclerosis (lSSc) and 10 (31.25%) had sclerosis with overlap syndrome. The common clinical features apart from sclerosis/induration proximal to metacarpophalangeal joint were Raynauds phenomenon (n = 22, 68.7%), skin rash (n = 20, 62%), arthritis or arthralgia (n = 16, 50%), and muscular weakness (n = 10, 31.2%). Among those for whom data regarding investigations were available; ANA was positive in 50% (12/24), whereas Anti Scl70 was positive in one out three cases. Treatment regimen included naproxen, methotrexate, calcium channel blockers with or without steroids. HCQ was added in children with skin rash or in children with partial control. Median (IQR) follow-up period was 19.75 (12–31.75) months. With the above treatment protocol, 19 (59.3%) children achieved disease control on treatment, 8 (26.6%) had partial control while 5 (16.6%) showed no response or progressive disease. Esophageal dysmotility and intertitial lung disease (ILD) were documented in three children each. Complication (cataract and herpes zoster) related to immunosuppressive therapy were observed in two children. There was no mortality during the study period. Juvenile Sclerosis though rare is associated with significant morbidities and lacks a curative treatment but a reasonable quality of life to perform daily activities can be achieved using methotrexate and steroid-based immuosuppressive therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Hashkes PJ (2016) 50 years ago in the journal of pediatrics: scleroderma in childhood. J Pediatr 169:35

    Article  PubMed  Google Scholar 

  2. Vancheeswaran R, Black CM, David J, Hasson N, Harper J, Atherton D et al (1996) Childhood-onset scleroderma: is it different from adult-onset disease. Arthritis Rheum 39(6):1041–1049

    Article  CAS  PubMed  Google Scholar 

  3. Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino AV et al (2006) Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol 33(5):1004–1013

    PubMed  Google Scholar 

  4. Foeldvari I, Zhavania M, Birdi N, Cuttica RJ, de Oliveira SH, Dent PB et al (2000) Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatol Oxf Engl 39(5):556–559

    Article  CAS  Google Scholar 

  5. Misra R, Singh G, Aggarwal P, Aggarwal A (2007) Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. Clin Rheumatol 26(8):1259–1262

    Article  PubMed  Google Scholar 

  6. Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23(5):581–590

    Article  Google Scholar 

  7. Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA, Lehman TJA et al (2007) The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum 57(2):203–212

    Article  PubMed  Google Scholar 

  8. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205

    CAS  PubMed  Google Scholar 

  9. Bennett RM, O’Connell DJ (1980) Mixed connective tissue disease: a clinicopathologic study of 20 cases. Semin Arthritis Rheum 10(1):25–51

    Article  CAS  PubMed  Google Scholar 

  10. Krishnamurthy V, Porkodi R, Ramakrishnan S, Rajendran CP, Madhavan R, Achuthan K et al (1991) Progressive systemic sclerosis in south India. J Assoc Phys India 39(3):254–257

    CAS  Google Scholar 

  11. Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG et al (2006) Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatol Oxf Engl 45(5):614–620

    Article  CAS  Google Scholar 

  12. Jablonska S, Blaszczyk M (1997) Childhood-onset scleroderma from a dermatologist’s perspective: comment on the article by Vancheeswaran et al. Arthritis Rheum 40(6):1183–1184

    Article  CAS  PubMed  Google Scholar 

  13. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A et al (2006) Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum 54(12):3971–3978

    Article  CAS  PubMed  Google Scholar 

  14. Foeldvari I, Nihtyanova SI, Wierk A, Denton CP (2010) Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort. J Rheumatol 37(11):2422–2426

    Article  PubMed  Google Scholar 

  15. Torok KS (2012) Pediatric scleroderma—systemic and localized forms. Pediatr Clin North Am 59(2):381–405

    Article  PubMed  PubMed Central  Google Scholar 

  16. Zulian F (2005) Scleroderma in children. Pediatr Clin North Am 52(2):521–545, vii

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India

    Narendra Kumar Bagri, Dinesh Raj, Jasmeet Kaur, Harish Punia, Isha Saini, Rakesh Lodha & S. K. Kabra

Authors
  1. Narendra Kumar Bagri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dinesh Raj
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jasmeet Kaur
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Harish Punia
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Isha Saini
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rakesh Lodha
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. S. K. Kabra
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

NKB: Data collection, analysis, and drafted the manuscript. DR: Data collection and drafted manuscript. JK: Data collection and drafted manuscript. HP, IS: Data collection and analysis. RL: Reviewed the manuscript for intellectual content. SKK: Concept, intellectual critical review and guarantor of the study

Corresponding author

Correspondence to Narendra Kumar Bagri.

Ethics declarations

Conflict of interest

No potential conflict of interests.

Ethical approval

As this is a retrospective chart review of our follow-up clinic patients, we did not sought ethical approval. In addition, the chart review does not reveal the identity of any of the study subjects. For these reasons, we did not seek ethical approval and informed consent. No procedures were performed in the study (only a retrospective chart review is presented).

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bagri, N.K., Raj, D., Kaur, J. et al. Juvenile systemic sclerosis: experience from a tertiary care center from India. Rheumatol Int 37, 1687–1691 (2017). https://doi.org/10.1007/s00296-017-3793-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-017-3793-3

Keywords

Search

Navigation