Granulomatous nephritis: A rare presentation of juvenile-onset sarcoidosis.

Sarcoidosis is rare in children. Incidence and prevalence of sarcoidosis in India are not known. Renal involvement in childhood sarcoidosis is further rare with no clear data about prevalence. Here we report a case of a 13-year-old girl who presented with sarcoidosis with multi-system involvement including renal sarcoidosis. She initially presented with pyrexia of unknown origin and cervical lymphadenopathy - evaluation of which led to diagnosis of sarcoidosis. Later, after development of pulmonary involvement, she was treated with oral prednisolone and azathioprine. She again defaulted on medicines and later presented with renal failure and was diagnosed with a renal sarcoidosis. She was treated with oral prednisolone and mycophenolate mofetil with which she gradually improved with normal renal functions.

Childhood Sarcoidosis mimicking tuberculosis: a case report

Abstract Sarcoidosis is a rare multisystem chronic inflammatory disease in children. We present a case of a five-year-old child with clinical features mimicking several diseases, including tuberculosis. After failure of treatment based on the suspected diagnosis, an axillary lymph node biopsy showed noncaseating granulomas compatible with sarcoidosis and appropriate treatment was then started.

Unusual Clinical Presentations in Early-Onset Childhood Sarcoidosis: A Correlation or Coincidence?

Sarcoidosis is a multisystem granulomatous disease which frequently affects young adults. Because of its rarity, the exact incidence and prevalence of childhood sarcoidosis is not known. It mostly affects children of older age group i.e., 13-15 years. Early onset sarcoidosis (<5 years) is characterized by a triad of arthritis, uveitis and rash. Late onset sarcoidosis present with a multisystem disease similar to adults, with frequent pulmonary infiltrations and lymphadenopathy. Herein, we report a case of early-onset childhood sarcoidosis in a four-year-old female along with uncommon clinical features like cutaneous ulceration, onycholysis and geographical tongue and its rarity in the literature.

Sarcoidosis en la infancia. Una rara enfermedad sistémica / Sarcoidosis in childhood. A rare systemic disease

Resumen: Introducción: La sarcoidosis es una enfermedad sistémica de etiología desconocida que raramente se presenta en la infancia. Generalmente afecta los pulmones; sin embargo, puede involucrar diversos órganos. Ocasionalmente afecta el estado general, y origina fiebre, hepatomegalia y esplenomegalia. Caso clínico: Se presenta el caso de un adolescente de doce años de edad con sarcoidosis infantil de inicio tardío, cuyo diagnóstico fue confirmado con un estudio histopatológico de ganglio linfático. El paciente cursó con afección general, hipercalcemia, eritema nodoso, alteraciones pulmonares graves, adenopatías, hepatomegalia y masa testicular. Recibió tratamiento con esteroides, con excelente respuesta clínica. Conclusiones: Se resalta la importancia de considerar el diagnóstico de sarcoidosis en los pacientes con hepatomegalia, adenopatías, daño pulmonar difuso, eritema nodoso, masa testicular e hipercalcemia, así como la necesidad del abordaje multidisciplinario para valorar el compromiso orgánico múltiple y el inicio oportuno de la terapia con esteroides, con el fin de evitar la progresión de la enfermedad.

Abstract: Background: Sarcoidosis is a systemic disease of unknown etiology that rarely occurs in children. It usually affects the lungs, however, it may involve various organs. It occasionally affects the general condition, and causes fever, hepatomegaly and splenomegaly. Case report: We report the case of a twelve-year-old adolescent with late-onset childhood sarcoidosis which diagnosis was confirmed by lymph node histopathological study. The patient presented general condition, hypercalcemia, erythema nodosum, severe lung disorders, lymphadenopathy, hepatomegaly and testicular mass. He received treatment with steroids, with excellent clinical response. Conclusions: We highlight the importance of considering the diagnosis of sarcoidosis in patients with hepatomegaly, lymphadenopathy, diffuse lung damage, erythema nodosum, testicular mass and hypercalcemia, as well as the need for a multidisciplinary approach to assess multiple organ involvement and the early beginning of steroid treatment in order to prevent the progression of the disease.

[Sarcoidosis in childhood. A rare systemic disease]. / Sarcoidosis en la infancia. Una rara enfermedad sistémica.

BACKGROUND: Sarcoidosis is a systemic disease of unknown etiology that rarely occurs in children. It usually affects the lungs, however, it may involve various organs. It occasionally affects the general condition, and causes fever, hepatomegaly and splenomegaly. CASE REPORT: We report the case of a twelve-year-old adolescent with late-onset childhood sarcoidosis which diagnosis was confirmed by lymph node histopathological study. The patient presented general condition, hypercalcemia, erythema nodosum, severe lung disorders, lymphadenopathy, hepatomegaly and testicular mass. He received treatment with steroids, with excellent clinical response. CONCLUSIONS: We highlight the importance of considering the diagnosis of sarcoidosis in patients with hepatomegaly, lymphadenopathy, diffuse lung damage, erythema nodosum, testicular mass and hypercalcemia, as well as the need for a multidisciplinary approach to assess multiple organ involvement and the early beginning of steroid treatment in order to prevent the progression of the disease.

Childhood sarcoidosis: Louisiana experience.

A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with manifestations similar to those of adult patients. Low-dose MTX seems to be effective, steroid sparing, and safe adjunct to treat sarcoidosis with multiorgan involvement. Early-onset disease is less common and associated with increased morbidity, flares, and poor prognosis.

Sarcoidosis en pediatría: reporte de 7 casos / Pediatric sarcoidosis: a report of seven cases

La sarcoidosis es una enfermedad granulomatosa crónica de origen no infeccioso que puede comprometer diversos órganos. Su prevalencia es baja, y en Colombia se han reportado casos de manera aislada. Su etiología y fisiopatología aún no se conocen completamente. La presentación clínica y las diferentes manifestaciones de la enfermedad son variables. Cuando su debut se presenta en niños menores de 5 años se denomina sarcoidosis de inicio temprano, mientras que cuando lo hace en niños mayores de 5 años recibe el nombre de sarcoidosis de inicio tardío. En este reporte de caso se presentan 7 pacientes pediátricos, de los cuales 5 correspondieron a sarcoidosis de inicio temprano y 2 a sarcoidosis de inicio tardío. Todos los pacientes tuvieron un diagnóstico tardío de la enfermedad, manifestaciones de varios órganos y sistemas, y recibieron tratamiento inmunosupresor. Cuatro tuvieron curso crónico, 2 remisiones de la enfermedad y 1 recaídas frecuentes. Fue llamativa una asociación poco usual de 2 pacientes con sarcoidosis de inicio temprano quienes adicionalmente presentaron la enfermedad de Ollier.

Sarcoidosis is a chronic granulomatous disease of non-infectious origin which can involve various target organs. Its prevalence is low and there have been only isolated cases reported in Colombia. Its etiology and pathophysiology are not well known. The clinical presentation and signs of the disease vary. When its onset is before five years of age it is recognized as early onset sarcoidosis, while if its onset is after five years of age it is called late onset sarcoidosis. In this case report all patients had a delayed diagnosis and they presented with a multiple organ involvement which required an immunosuppressive treatment. Of the 7 patients, 4 had a chronic course, 2 had remission, and 1 with frequent relapses of the disease. There was an unusual relationship of two patients with early onset disease who additionally presented with Ollier's disease.