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  • • Also known as Boeck sarcoid, benign lymphogranulomatosis

    • Noncaseating granulomatous disease involving lungs, liver, spleen, lymph, skin, and bones

    • Cause is unknown

    • 20% have myocardial involvement

    • 30% have cutaneous involvement

    • 70% have hepatic and splenic involvement

    • Mediastinal and scalene lymph nodes microscopically involved in 90% and 80%, respectively

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Epidemiology

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  • • Highest incidence: Scandinavia, England, United States

    • Incidence in blacks 15 fold that of whites

    • 50% between ages 20 and 40 years

    • Women affected more often than men

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Symptoms and Signs

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  • • May present with nonspecific pulmonary symptoms (20-30%), including fever (15%) and cough

    • Erythema nodosum may herald onset

    • Weight loss, fatigue, weakness, malaise, enlarged lymph nodes (75%)

    • Migratory or persistent polyarthritis

    • CNS symptoms (rarely)

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Laboratory Findings

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  • • Elevated serum and bronchoalveolar ACE levels

    • Elevated lysozyme levels

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Imaging Findings

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  • • Chest film: 5 stages

    • -Stage 0: No abnormality

      -Stage 1: Hilar/mediastinal adenopathy alone

      -Stage 2: Hilar adenopathy with pulmonary abnormalities

      -Stage 3: Diffuse pulmonary disease without adenopathy

      -Stage 4: Pulmonary fibrosis

    • Mediastinal lymph node involvement characteristically bilateral and symmetric

    • Asymmetric hilar adenopathy: Consider lymphoma or other disease

    • Pleural effusions/cavitation rare: Consider TB, pneumonia

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  • • Diagnosis of exclusion

    • Suggested by chest film, gallium 67 scanning, elevated serum and bronchoalveolar ACE and lysozyme levels

    • Pathologic documentation of noncaseating granulomas via transbronchial or mediastinoscopy

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Rule Out

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  • • Hodgkin disease

    • Non-Hodgkin lymphoma

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  • • Chest x-ray

    • Chest CT scan

    • Bronchoscopy and biopsy if pulmonary compromise

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  • • No therapy needed for asymptomatic patients

    • Corticosteroids for patients with pulmonary impairment or are symptomatic

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Complications

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  • • Corticosteroid side effects

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Prognosis

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  • • Long-term mortality as high as 10%

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References

Paramothayan S et al. Immunosuppressive and cytotoxic therapy for pulmonary sarcoidosis. Cochrane Database Syst Rev. 2003;(3):CD003536.

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