Definition
- Systemic granulomatous disease of unknown etiology
- affects young & middle-aged adults
- Frequent hilar/mediastinal lymphadenopathy,
- pulmonary involvement, eye & skin lesions
Radiography
- Abnormal in up to 90% of cases
- Lymphadenopathy (75-90%)
- Upper/mid lung small nodular/reticular opacities
- Large nodules/consolidations ± air bronchograms
- Upper lung zone fibrosis (stage IV)
CT/HRCT
- Bilateral upper/mid lung zone perilymphatic micronodules
- Nodular sarcoidosis: Large lung nodules/masses
- Airway involvement: Stenosis, small airway disease
- Pulmonary fibrosis (20%), mycetoma formation
Top Differential Diagnoses
- Silicosis
- Lymphangitic carcinomatosis
- Pathology
- Noncaseating epithelioid granulomas
- Diagnosis of exclusion
Clinical Issues
- Asymptomatic (30-50% of patients)
- Dyspnea, cough, chest pain, wheezing
- Fatigue, fever, weight loss, sweats, arthralgias
- Affected patients typically < 40 years
Prognosis
- Stability or remission in 2/3 of patients
- Chronic diseang fibrosis in 20%
Treatment:
Corticosteroids, immunosuppressants
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