Definition
• Sarcoidosis, which is a rare multisystemic disease in childhood, has 2 different forms.
• One of them is the adult-like form, which starts in late childhood and progresses with fever, weight loss, malaise, lung involvement and lymphadenopathy.
• The other is the early-onset form, which is seen especially under the age of 4 and progresses with the typical triad (rash, uveitis and polyarthritis).
• Infantile form sarcoidosis and Blau syndrome have similar clinical genetic features.
pathogenesis
• Noncaseating epithelioid granulomatous lesions are the cardinal pathological feature of the disease.
Clinical Findings
• In the late-onset form, hilar lymphadenopathy in the isolated bilateral lung is the most common finding.
• Extrathoracic lymphadenopathy, hepatomegaly, splenomegaly, skin lesions (erythema nodosum, lupus pernio, red-brown nodular skin lesions, etc.), arthritis and uveitis are other important clinical findings.
Diagnosis
• The type is determined by showing noncaseating granulomatous lesions in the biopsy of the affected ratio.
• Laboratory findings may include anemia, leukopenia and eosinophilia. Serum calcium may be high.
• The level of angiotensin converting enzyme (ACE) synthesized from granulomas may be high in the blood, but it has no diagnostic sensitivity or specificity.
Treatment
• The main drug in the treatment of acute and chronic signs of the disease is corticosteroids.
• Methotrexate, leflonomide, and infliximab may also be effective in steroid-dependent patients.