20 something year old male
Chest x-ray - bilateral hilar fullness
1. Mediastinal and hilar lymphadenopathy
2. Perilymphatic pulmonary nodules (peribronchiovascular and pleural)
Thoracic Sarcoidosis 'The Great Mimic'
Multi-systemic disease with variable presentation, prognosis and progression. Characterized by noncaseous epithelioid cell granulomas in absence of causitive agent such as TB, berylliosis, leprosy, hypersensitivity pneumonitis, Crohn disease, primary bilary cirrhosis and fungal disease. Sarcoid-like reactions can also be seen in neoplasm draining to lymph nodes, chronic inflammation, post-chemo or radiation therapy.
Thoracic involvement common and cause of the majority of morbidity and mortality.
90% patients have some sort of thoracic involvement. 20% develop chronic lung disease and subsequently pulmonary fibrosis
immune-mediated multisystem disease.
Thought to be a specific environmental agent triggering an inflammatory response in genetically susceptable host.
Bilateral hilar lymphadenopathy most common radiographic finding, followed by interstitial lung disease, occasional calcification of affected lymph nodes, pleural effusions and thickening.
Interstitial lung disease to include: perilymphatic distribution of micronodules, fibrotic changes and bilater perihilar opacities
Atypical findings: masslike/alveolar opacities, honeycomb-like cysts, miliary opacities, mosaic atenuation, tracheobronchial involvement (may have obstructive ventilatory defect on PFT's), pleural disease (effusion and/or thickening).
If biopsy of lymph nodes or parenchyma required, may do so through transbronchial, CT guided or surgical biopsy.
Historical based off of chest radiographic findings. Siltzback classification following five stages. Staging and prognisis have not been established with CT and CT high resolution findings.
Stage 0 Normal chest xray, 5-10%
Stage 1 Lymphadenopathy only, 50%
Stage 2 Lymphadenopathy and parenchymal lung disease, 25-30%
Stage 3 Parenchymal lung disease, 10-12%
Stage 4 Pulmonary fibrosis, 25%
50% are asymptomatic
25% cough or dyspnea, bronchial hyperreactivity
25% skin lesions (erythema nodosum)
Also can see fatigue, night sweats and weight loss
Pulmonary function tests often show restrictive ventilatory defect with decreased volumes and decreased carbon monoxide diffusing capacity.
2/3 remain stable or remission within decade of diagnosis with minimal to no recurrances/sequella
20% go on to develop chronic disease with pulmonary fibrosis
5% of sarcoidosis die from pulmonary fibrosis, cardiac or neurologic involvement
Recurrence after 1 year in remission is rare however may occur in any organ system
Poor prognosis if:
Good prognosis ( >85% spontaneous remission rate):
women > men
35.5 vs 10.9 per 100,000 black to whites
Whites often present without symptoms. Blacks often have severe multisystem disease
March 1995 RadioGraphics, 15, 421-437.
Pulmonary Sarcoidosis:Typical and Atypical Manifestations at High- Resolution CT with Pathologic Correlation1. October 2010 RadioGraphics, 30, 1567-1586. http://radiographics.rsna.org/content/30/6/1567.full.pdf+html
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Case Number: 48156371Last Updated: 11-14-2010 The reader is fully responsible for confirming the accuracy of this content.
The reader is fully responsible for confirming the accuracy of this content.