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2.1
Early-Onset Sarcoidosis

Presentation

Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-γ receptor 1 deficiency that presented with BCG-osis. [ncbi.nlm.nih.gov]

Sarcoidosis can present with an acute onset (usually Löfgren's syndrome (LS)) or a gradual onset (non-LS)... [readbyqxmd.com]

Childhood sarcoidosis in Denmark 1979-1994: Incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr 2004;93:30-6. 2. Schaller JG. Juvenile rheumatoid arthritis. [ijdvl.com]

Workup

Workup for recurrent disease revealed thrombocytopenia, hepatosplenomegaly, and a left pleural effusion. Cultures obtained from pleural fluid were positive for Mycobacterium tuberculosis. The infection was treated and the patient recovered. [omicsonline.org]

"Sarcoidosis Workup". Medscape Reference. WebMD. Archived from the original on 1 March 2014. Retrieved 19 February 2014. ^ Allmendinger A, Perone R (2009). "Case of the Month". Diagnostic Imaging. 31 (9): 10. ^ Joanne Mambretti (2004). [en.wikipedia.org]

Standard Medical Workup for Alzheimer's Disease. Alzheimer Research Forum [On-line information]. Available online at http://www.alzforum.org/members/research/treatment_guide/workup.html. Family Caregiver Alliance. [labtestsonline.it]

Treatment

Involvement of the orbit and posterior segment of the eye in early-onset sarcoidosis may necessitate prompt and aggressive immunosuppressive treatment to prevent a poor visual outcome. [ncbi.nlm.nih.gov]

[…] for further treatment. 5 There are no specific recommendations about when to initiate corticosteroid treatment. [aafp.org]

The drug treatment aims to control symptoms and halt the progression of the disease. 2.4 What are the treatments? At present, there is no evidence concerning the optimal treatment for Blau syndrome/EOS. [printo.it]

Prognosis

Early and correct diagnosis is important in light of the severe disease prognosis, and the possible therapeutic options. [ncbi.nlm.nih.gov]

Secondary glaucoma, together with sarcoid uveitis, may be severe and portends a poor prognosis with associated severe visual loss. Posterior segment lesions occur in up to 20% of patients with ocular sarcoidosis. [aao.org]

Early sarcoid arthritis is often self-limited, lasting for days to months, and the prognosis is excellent. [rheumaknowledgy.com]

Genetic factors may also be important in defining the pattern of disease presentation, its severity, and the prognosis. [emedicine.medscape.com]

However, the activity of the disease may fluctuate over time. 2.10 What is the long-term prognosis (predicted outcome and course) of the disease? The available data concerning the prognosis in the long-term is limited. [printo.it]

Etiology

The aim of this study was to assess the efficacy of thalidomide in patients with severe EOS, based on etiology supporting an initial role of NF-kappaB in activation of this disease. [ncbi.nlm.nih.gov]

These findings indicate that the majority of EOS cases reported in Japan shows the genetic etiology of NOD2 mutations that cause constitutive NF-kappaB activation. [jstage.jst.go.jp]

The possibility of genetic etiology has also been explored by Baughman et al. in an expert review [ 4 ]. The focus of the review was an update on sarcoidosis etiology, diagnosis and treatment. [omicsonline.org]

DISEASE: Sarcoidosis, early-onset Entry H01309 Disease Name Sarcoidosis, early-onset Description Early-onset sarcoidosis (EOS) is a special subtype of sarcoidosis, a systematic inflammatory disease with unknown etiology. [genome.jp]

Epidemiology

References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology The cause of sarcoidosis is still unknown ;. [amboss.com]

There are very few epidemiological data on children. [elsevier.es]

The purpose of the prospective study was to review epidemiologic data on incidence, prevalence, and clinical characteristics of sarcoidosis. Data was obtained through surveys obtained twice a year. [omicsonline.org]

"The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival" (PDF). American Journal of Epidemiology. 123 (5): 840–5. doi : 10.1093/oxfordjournals.aje.a114313. [en.wikipedia.org]

Epidemiology Sarcoidosis occurs worldwide, with a prevalence of about 4.7-64 in 100,000, and an incidence of 1.0-35.5 in 100,000 per year [ 1 ]. The highest rates are reported in northern European/Scandinavian and African-American individuals. [patient.info]

Pathophysiology

Signaling protein and cytokine expression were examined, and the results of these experiments challenge current theories of constitutive NOD2 activation in the pathophysiology of Blau syndrome... [readbyqxmd.com]

Current hypotheses suggest that the etiology is multifactorial : References: [3] Pathophysiology Sarcoidosis is a systemic disorder characterized by both T-cell dysfunction and increased B-cell activity with subsequent local immune hyperactivity and inflammation [amboss.com]

The exact mechanism of this relation is not known, but some evidence supports the hypothesis that this is a consequence of Th1 lymphokine prevalence. [55] [82] Tests of delayed cutaneous hypersensitivity have been used to measure progression. [83] Pathophysiology [en.wikipedia.org]

Prevention

Involvement of the orbit and posterior segment of the eye in early-onset sarcoidosis may necessitate prompt and aggressive immunosuppressive treatment to prevent a poor visual outcome. [ncbi.nlm.nih.gov]

Conclusion: TNF inhibitors appear to be more effective than IL-6 inhibitor in the treatment of EOS, and may have a potential to prevent the onset of uveitis. Disclosure: T. Nagakura, None; T. Yamatou, None; T. Kubota, None; H. Wakiguchi, None; Y. [acrabstracts.org]

A 1, 5 Calcitonin (Miacalcin) and bisphosphonates may be useful in preventing osteoporosis in patients with sarcoidosis taking long-term steroid therapy. [aafp.org]

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