The true incidence and prevalence of sarcoidosis in children are unknown because of the rarity of the disease and the small number of reported cases in childhood. Most reported childhood cases have occurred in patients aged 13-15 years, but the disease has also been reported in very young children.
Two distinct forms of childhood sarcoidosis appear to exist. Older children usually present with a multisystem disease similar to the adult manifestation, with frequent lymphadenopathy and pulmonary involvement. They also show generalized signs and symptoms, such as fever and malaise. In contrast, early-onset childhood sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis, usually in patients who are younger than 4 years.
Pulmonary disease and abnormal findings on chest radiography are more common in children with sarcoidosis who are aged 8-15 years; these findings occur in 94%-100% of patients in that age group, compared with 22% of those younger than 4 years. Bilateral hilar adenopathy is the most common finding on chest radiography in children; it occurs in almost all cases. It is typically symmetrical, although it may be unilateral in rare instances. Pulmonary parenchymal involvement is common and predominantly appears radiographically as an interstitial pattern; however, nodular, alveolar, and fibrotic patterns have also been described.
Other uncommon manifestations include pleural effusion, pneumothorax, pleural thickening, calcification, atelectasis, and cor pulmonale. Nearly half of all children with sarcoidosis demonstrate restrictive lung disease on static and dynamic pulmonary function tests, including a reduction in total lung capacity, forced vital capacity, functional residual capacity, and transfer factor. These changes are believed to be secondary to early alveolitis progressing to fibrosis. An obstructive ventilatory pattern has been reported in approximately 15% of children with sarcoidosis. Airway obstruction may be secondary to airway hyperactivity, intrabronchial sarcoid granuloma, hilar or mediastinal lymph node compression of the airways, or bronchiectasis.[2,3]
The serum ACE level is increased in 30%-80% of patients with sarcoidosis and may be a surrogate marker of the total granuloma burden. False-positive findings are noted in fewer than 20% of patients with other pulmonary disorders; however, the serum ACE level may be normal in patients with active disease.
About 40%-70% of children with sarcoidosis have palpable peripheral lymph glands. The lymph nodes typically are firm, nontender, discrete, and freely movable. They do not ulcerate and do not form draining sinuses. The most frequently involved glands are the cervical, axillary, epitrochlear, and inguinal glands. In the neck, the posterior triangle nodes are affected more commonly than the nodes in the anterior triangle. The enlarged peripheral lymph nodes are the most accessible tissue for biopsy, providing a high diagnostic yield.
Hepatosplenomegaly occurs in as many as 43% of children with sarcoidosis at some point in their clinical course; however, clinically significant hepatic dysfunction is rare. Mild elevation in biochemical liver function test values is common (as it was in this patient), but severe liver involvement is unusual in children.
Ocular involvement is very common in children with sarcoidosis, and a complete ophthalmologic evaluation, including a slit-lamp examination, is crucial (particularly in young children). Any part of the eye or orbit may be affected. Anterior uveitis (also known as "iritis" or "iridocyclitis") is the most frequently observed lesion, occurring in about 58%-90% of early-onset sarcoidoses, compared with 24%-54% of sarcoidosis cases in older children.
Sarcoid-associated uveitis can be acute or chronic and may vary from an isolated iridocyclitis to a bilateral panuveitis syndrome. If left untreated, the disease may result in synechiae, corneal opacities, glaucoma, and, eventually, blindness.
Conjunctival granulomas are the second most common ocular manifestation in sarcoidosis. Other forms of ocular lesions include interstitial keratitis; band keratopathy (from calcium deposition); dacryocystitis; retinal vasculitis; lacrimal gland enlargement; choroiditis; and orbital infiltration, which may result in proptosis.
Cutaneous involvement occurs in about 24%-40% of older children with sarcoidosis and in 77% of young children with sarcoidosis. Various cutaneous eruptions are frequently found on the face, but the trunk, extremities, and buttocks may also be involved. Skin lesions of sarcoidosis may include macules, papules, nodules, hyperpigmented lesions, hypopigmented lesions, ulcers, subcutaneous tumors, and erythema nodosum. Rarely, "scar sarcoidosis," the infiltration of old scars with granuloma, may present in children.
Renal involvement is not well characterized in published series of childhood sarcoidosis. Only 32 isolated cases of sarcoidosis with renal involvement have been reported in children since 1941. Histopathologic studies have revealed epithelioid granuloma formation, interstitial infiltration by mononuclear cells, interstitial fibrosis, tubulitis, tubular atrophy, mesangial hyperplasia, glomerular fibrosis, membranous nephropathy, and vascular involvement.[3,4]
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