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.