1. Fig 1: Skin thickening and sclerodactyly Fig 2: Digital tip ulcers
A Single Centre Experience of Pediatric Systemic Sclerosis from North India
Ankur Kumar, Sagar Bhattad, Anju Gupta, Amit Rawat, Deepti Suri, Biman Saikia#, Ranjana W. Minz#, Surjit Singh
Pediatric Allergy and Immunology Unit, Dept of Pediatrics, #Department of Immunopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Introduction
Results
Fig 1: Skin thickening and sclerodactyly
A total of 15 children (5 boys and 10 girls) were diagnosed as SSc between 1990 and 2015
All of them had diffuse cutaneous systemic sclerosis
Mean age at symptom onset – 7.7 years
(range 4-14 years)
Mean age at diagnosis – 10.2 years
(range 6-15 years)
Maximum mRSS (median) in our cohort
– 18 (range 6 – 40)
Mean J4S at the time of enrolment - 7
(range 3- 12)
Skin biopsy - 9 patients
Suggestive of sclerodermatous skin changes in all
Capillaroscopy - 3 patients
Suggestive of capillary drop outs and tortuous loops
HRCT chest - interstitial lung disease (ILD) in 7/10 patients with ground glass opacity and honeycombing being common abnormalities
One patient developed ILD after 2 years of initial diagnosis of SSc
One patient showed regression of ILD on 6 years of follow-up
Systemic sclerosis (SSc) is an inflammatory disorder with multisystem involvement characterized by progressive fibrosis affecting skin and various internal organs
Methods
Fig 2: Digital tip ulcers
Diagnosis of SSc was based on Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for Juvenile Systemic sclerosis
Retrospective review of case records
Modified Rodnan skin score (mRSS) was used to quantify the dermatological involvement
Juvenile systemic scleroderma severity score (J4S) was used to assess the severity
Fig 3: HRCT chest – ILD
Treatment
PFT -14
Mild restriction -5
Mod/severe restriction - 6
HRCT chest - 10
ILD - 1
ILD - 6
Outcome
Total patient-months of follow–up: 546
Mean follow-up: 36.4 months
(6-120 months)
No mortality, though 7 of these patients were not under regular follow-up
Feature % RP
73%
RP as the sole presenting manifestation
20%
Skin tightening at presentation
80%
Arthritis
13%
Calcinosis
Dyspnoea/Symptomatic ILD
Dysphagia
33%
Skin pigmentation
Digital tip ulcers
50%
Telengiectesia
Acro-osteolysis 6%
Investigation
Result
PFT 11 03
Restrictive
Normal
HRCT chest
ILD in 7/10 patients
Echocardiography
PAH in 1/13 patients
ANA
Speckled
Nucleolar
Mixed
Diffuse
11/15 4 2 1
RA factor
3/15
Urine examination
normal
Conclusions
Systemic sclerosis is a rare pediatric rheumatological disorder with poorly defined treatment protocols
Though a significant proportion of children with SSc have lung involvement on imaging, symptomatic ILD is uncommon
Suggested reading
RP – Raynaud phenomenon, ILD – interstitial lung disease
PFT- Pulmonary function test, ILD – Interstitial lung disease, PAH - Pulmonary hypertension
Foeldvari I. Update on juvenile systemic sclerosis. Curr Rheumatol Rep Mar;17(3):18
Table 1: Clinical features
Table 2: Investigations