Childhood sarcoidosis: Louisiana experience

Area of Science:

• Pediatric Rheumatology
• Immunology
• Pulmonology

Background:

• Sarcoidosis is a multisystem inflammatory disease of unknown etiology.
• Childhood sarcoidosis is less common than adult-onset disease and can present with varied manifestations.
• Understanding the prevalence and clinical course of pediatric sarcoidosis is crucial for timely diagnosis and management.

Purpose of the Study:

• To identify and characterize patients with sarcoidosis treated by a pediatric rheumatology service.
• To describe the clinical manifestations, diagnostic findings, and treatment outcomes in childhood sarcoidosis.
• To evaluate the efficacy and safety of low-dose methotrexate as a steroid-sparing agent.

Main Methods:

• Retrospective chart review of 27 patients diagnosed with sarcoidosis between 1992 and 2013.
• Analysis of patient demographics, symptom duration, clinical manifestations, diagnostic tests (biopsy, ACE levels), and treatments.
• Comparison of early-onset (<5 years) versus later-onset sarcoidosis.

Main Results:

• The average symptom duration before diagnosis was 5 months; 5 patients had early-onset sarcoidosis.
• Common manifestations included constitutional symptoms, ocular (uveitis/iritis), hilar adenopathy, arthritis, and lung disease; 70% had multiorgan involvement.
• Noncaseating granulomas were found in 77% of biopsies; elevated angiotensin-converting enzyme (ACE) levels in 74%. Low-dose methotrexate (MTX) was effective and steroid-sparing in 14 patients.

Conclusions:

• Childhood sarcoidosis is prevalent in Louisiana, often presenting as a multisystem disease with adult-like manifestations.
• Early-onset sarcoidosis is associated with increased morbidity and poorer prognosis.
• Low-dose MTX appears to be an effective, safe, and steroid-sparing adjunctive therapy for pediatric sarcoidosis with multiorgan involvement.