Childhood sarcoidosis: long-term follow-up

Milman, Nils; Hoffmann, Anne Lisbeth

doi:10.1183/09031936.00011507

Cited by 83 publications

(37 citation statements)

References 16 publications

(28 reference statements)

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“…Thirty-six (78 %) children recovered completely, 30 (65 %) showed complete clinical regression at a median of 0.7 (range 0.6-5.9) years after onset of disease, 2 (4 %) recovered with organ damage (unilateral loss of vision, abnormal chest radiograph), 5 (11 %) still had chronic active disease with multiorgan involvement and impaired lung function, and 3 (7 %) were deceased, due to central nervous system sarcoidosis and acute myeloid leukemia probably caused by immunosuppressive agents [19].…”

Section: Discussionmentioning

confidence: 98%

Childhood sarcoidosis: Louisiana experience

et al. 2015

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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.

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Section: Discussionmentioning

confidence: 98%

Childhood sarcoidosis: Louisiana experience

et al. 2015

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“…Common symptoms of respiratory involvement are exertional dyspnoea, chest pain, cough and wheeze, although many may be asymptomatic. Radiographic changes are divided into the categories seen in table 2, with the majority of children presenting with stage I disease (61–71%) 51 54. PFTs may be normal in stage 0 or stage I disease, but a restrictive pattern with decreased DLCO is the most common abnormality 54…”

Section: Sarcoidosismentioning

confidence: 99%

Respiratory complications of the rheumatological diseases in childhood

2016

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Pleuropulmonary manifestations of rheumatological diseases are rare in children but pose a significant risk to overall morbidity and mortality. We have reviewed the literature to provide an overview of the respiratory complications of the commonest rheumatological diseases to occur in children (juvenile systemic lupus erythematosus, scleroderma, juvenile dermatomyositis, mixed connective tissue disease, granulomatosis with polyangitis and juvenile idiopathic arthritis). Pulmonary function testing in these patients can be used to refine the differential diagnosis and establish disease severity, but also has a role in ongoing monitoring for respiratory complications. Early detection of pulmonary involvement allows for prompt and targeted therapies to achieve the best outcome for the child. This is best achieved with joint specialist paediatric rheumatology and respiratory reviews in a multidisciplinary setting.

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“…In Danish children who had a longterm follow-up, sarcoidosis had a favorable prognosis as the majority recovered less than 6 years after the onset of the disease. [79] An automatic resolution rate of chest-X-ray (CXR) abnormalities over time was estimated to be as high as 75%. Hilar lymphadenopathy in combination with acute or subacute onset (fever, arthralgia, erythema nodosum) is associated with a remission rate of 80%-90%.…”

Section: Prognosismentioning

confidence: 99%