Sarcoidosis (with PubMed collection on neurosarcoidosis)

Fretzayas A, et al. The puzzling clinical spectrum and course of juvenile sarcoidosis. World J Pediatr. 2011 May;7(2):103-10.

Sarcoidosis in childhood presents with two age dependent, distinct forms. In younger children it is clinically evident before the age of four years and characterized by the triad of rash, arthritis and uveitis. In their older counterparts, the juvenile late onset sarcoidosis involves several organs and its clinical appearance resembles the adult type of the disease, with the respiratory system being most frequently affected (hilar lymphadenopathy, pulmonary infiltrations). Steroid is the main agent of treatment whereas methotrexate is also used for beneficial steroid sparing effects. New, novel therapies may change the outcome of the disease especially in difficult morbid cases.

Cavalcanti A, et al. Sarcoidosis in childhood: report of three cases. Clin Exp Rheumatol. 2010 Jul-Aug;28(4):586.

“Diagnosis of sarcoidosis must be suspected in cases with skin granuloma, synovitis, lung involvement and lymphadenomegaly
(third case), or in the presence of rash, arthritis and uveitis as in Blau syndrome or in sporadic form (first and second case) which should be confirmed through genetic testing (5). Corticosteroids, methotrexate and
anti-TNF agents have been used as treatment (6).”

Shetty AK, Gedalia A. Childhood sarcoidosis: A rare but fascinating disorder. Pediatr Rheumatol Online J. 2008 Sep 23;6:16.

The clinical presentation can vary greatly depending upon the organs involved and age of the patient. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestations, with frequent hilar lymphadenopathy and pulmonary infiltrations. Early-onset sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in children presenting before four years of age. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. Other granulmatous diseases should be reasonably excluded.

Wiegand JA, Brutsche MH. Sarcoidosis is a multisystem disorder with variable prognosis–information for treating physicians. Swiss Med Wkly. 2006 Apr 1;136(13-14):203-9.

Sarcoidosis is a chronic granulomatous inflammatory disorder of unknown origin with heterogeneous outcome. In most cases the disease is self-limited, others progress or die from organ involvement, which is often associated with extensive scarring. Although often the primarily affected sites are the lungs and thoracic lymph nodes patients with sarcoidosis must be staged for multiorgan involvement. Modern treatment strategies appraise a critical awareness for the side-effect-ratio of long-term medication. A main interest of research is to identify those patients with unfavourable outcome.

Fauroux B, Clément A. Paediatric sarcoidosis. Paediatr Respir Rev. 2005 Jun;6(2):128-33.

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, most commonly affecting young adults. Divergent prevalence rates and clinical appearances in different races indicate the existence of predisposing genes, with probably a major effect of the major histocompatibility complex. The diagnosis is relatively rare in children. Lesions can occur in almost any tissue or organ but the lungs, lymph nodes, eyes, skin and liver are the most commonly involved. Clinical symptoms are non-specific and often minor. The diagnosis can only be supported by typical histopathological findings with non-caseating epithelioid-cell granulomas. The prognosis seems to be more severe in younger children and in cases of multi-organ involvement. Corticosteroids are the therapeutic agents most commonly used and are indicated in cases of significant lung or eye lesions, cardiac, neurological, or multiorgan involvement. Close monitoring is mandatory during and after treatment because relapses are common.

Hoffmann AL, Milman N, Byg KE. Childhood sarcoidosis in Denmark 1979-1994: incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr. 2004 Jan;93(1):30-6.

The incidence of sarcoidosis in Danish children is low and increases with age. Sarcoidosis in young children may present clinical features that are different from the appearance of those in older children and often constitute a diagnostic challenge. In older children, the clinical appearance has many features in common with the presentation in adults.

More PubMed results on neurosarcoidosis in pediatric patients.

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