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OP0088 Childhood sarcoidosis: experience in a paediatric rheumatology centre
  1. A Gedalia,
  2. AK Shetty,
  3. LR Espinoza
  1. Pediatrics and Medicine, LSU Health Sciences Center/Children’s Hospital, New Orleans, USA

Abstract

Background Sarcoidosis is a multisystemic granulomatous disease of unknown aetiology, most commonly affecting young and middle-aged adults. It is relatively uncommon in children.

Objectives To report our experience on the diagnosis, management and outcome of childhood sarcoidosis in a Paediatric Rheumatology settings.

Methods A retrospective chart review to detect patients with sarcoidosis seen by the Rheumatology Service from 1993 to 1998. All patients had initial evaluation that included a complete history and physical examination and an eye evaluation by an Ophthalmologist. Laboratory evaluation at initial and follow-up visits included CBC, ESR, renal and liver function tests, urinalysis, serum calcium, serum immunoglobulins, and Angiotensin converting enzyme (ACE) and chest radiograph. Clinical response to therapy was measured by clinical severity score that we devised. These data were analysed at diagnosis and follow-up visits.

Results Sixteen patients with sarcoidosis were identified. Thirteen were African-Americans. There were nine females and seven males with mean age at onset of 8 (range, 1–15) years; mean age at diagnosis of 10 (range, 2–16) years. Four patients had onset before age 4 years and were classified as early-onset sarcoidosis. Common manifestations included uveitis (69%), pulmonary disease (44%), hilar adenopathy (44%), arthritis (31%), generalised lymphadenopathy (25%), and skin rash (25%). Unusual associated features included abdominal aortic aneurysm (1 case), nephrocalcinosis (1), membranous nephropathy (1), and marrow disease (3). The diagnosis was confirmed by demonstration of noncaseating granuloma on a biopsy specimen associated with negative stains for mycobacteria and fungi in 12 patients. The tissue biopsy sites included peripheral lymph node (1), skin (1), kidney (1), lung (3), conjunctival nodule (1), lacrimal gland (1), bone (2), bone marrow (1), and tonsil (1). Laboratory abnormalities included elevated ACE serum levels (82%), hyper IgG (82%), anaemia (75%), elevated ESR (70%), and hypercalcemia (12%). Treatment with oral prednisone (1–2 mg/kg/day) was initiated in all 15 symptomatic patients with significant improvement. Prednisone was tapered and successfully discontinued within a mean duration of one year in four patients. Low-dose methotrexate (MTX) in oral dose of 10–15 mg/m2/week was administered in 11 patients. On oral MTX therapy steroid dose was successfully tapered and discontinued within a mean duration of 2.6 years in 6 patients, and was tapered from 1.2 mg/kg/day to 0.2 mg/kg/day within 3.5 years in four. One patient received cytoxan. Two patients relapsed 6 months after discontinuation of MTX. No deaths were noted. Morbidity included persistent iritis (3 patients), restrictive lung disease (1) and joint sequelae (1).

Conclusion Childhood sarcoidosis is prevalent in the State of Louisiana. This enigmatic disorder may involve a broad spectrum of body systems and may present in unusual ways in children. Low-dose MTX seems to be effective, steroid-sparing, and safe adjunct to treat sarcoidosis with multisystem involvement. Long-term follow-up is critical for better understanding of the guide of therapy and prognosis in this disease.

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