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Pediatric sarcoidosis in India
1Calcutta Hospital, Sarcoidosis Clinic, Institute For Respiratory Diseases, Kolkata, India.
Insights
Pediatric sarcoidosis in India presents with fever, constitutional symptoms, and organomegaly, often misdiagnosed. Oral steroids or chloroquine with NSAIDs offer effective treatment for this rare childhood granulomatous disease.
Area of Science:
- Pediatric Pulmonology
- Rheumatology
- Immunology
Background:
- Pediatric sarcoidosis is a rare granulomatous disease with limited reported cases in Indian literature since 1957.
- Initial diagnosis is often delayed due to non-specific symptoms and misdiagnosis, frequently as tuberculosis.
Observation:
- Cases predominantly affect girls (9-12 years) and present with universal fever, constitutional symptoms, weight loss, hepatomegaly, splenomegaly, and lymphadenopathy.
- Pulmonary findings are often subtle, contributing to diagnostic challenges.
Findings:
- Treatment with oral steroids or chloroquine combined with NSAIDs (with or without inhaled steroids) showed comparable efficacy.
- Long-term follow-up data is limited, with approximately 66% of cases experiencing relapses.
- One case developed a superinfection with acid-fast bacilli.
Implications:
- Early recognition and appropriate management are crucial for pediatric sarcoidosis.
- Further research is needed to understand long-term outcomes and optimize treatment strategies.
- Awareness among clinicians can reduce diagnostic delays in suspected cases of pediatric sarcoidosis.
Objective:
Since 1957, when the first pediatric case of sarcoidosis was reported, 11 more cases have been traced in the Indian literature.
Methods:
Nine of them were reported from general wards of hospitals (while the remaining 3 were from pediatric unit of AIIMS, New Delhi). Failure of initial treatment with anti-tuberculosis drugs for some months, necessitated search for an alternative diagnosis. Considerable delay (several months to years) occurred due to several parent- or physician-dependant factors.
Results:
Affection more in girls (9 to 12), universal fever and constitutional symptoms, loss of weight, scanty lung features, hepatomegaly, often with massive splenomegaly, frequent lymphadenopathy etc. caused initial confusion.
Conclusion:
Treatment with oral steroid or with chloquine and NSAIDS with or without steroid MDI gave equally good results. Long follow-up was done in a few cases only, showing relapses in nearly 66%. One case had a superinfection with acid-fast bacilli.
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