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. 2015 Mar;17(3):18 Table 1: Clinical features Table 2: Investigations ankurjindal11@gmail.com