Pulmonary sarcoidosis

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Review of Radiographics article 2010

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Dr. Varun The Great Mimic

Introduction :

Introduction Multisystem chronic inflammatory condition of unknown etiology Non caseous epitheloid cell granulomas Lung and mediastinal lymph nodes involved >90% cases Dr. Varun

Epidemiology :

Epidemiology <40 yrs of age, peaking in 3 rd decade F>M 40 per 100,000 Black americans , swedes , danes Dr. Varun

Clinical features:

Clinical features Respiratory symptoms Cough, dyspnea , bronchial hyperactivity Fatigue, night sweats, weight loss and erythema nodosum 50% asymptomatic PFT – restrictive with decreased volumes and DLCO Endobronchial sarcoid – obstructive pattern, presents early, florid symptoms, stage 4 disease, poor prognosis Clinical course 2/3 – stable or experience remission within decade after diagnosis 20% develop chronic disease Dr. Varun

Clinical features:

Clinical features Poor prognosticators Stage 2 or 3 disease at time of initial diagnosis, onset after 40yrs, black race, hypercalcemia , splenomegaly , osseous involvement, chronic uveitis and lupus pernio Good prognosticators Fever, polyarthritis , erythema nodosum , bilateral hilar node enlargement. (Lofgren syndrome) Dr. Varun

Pathogenesis :

Pathogenesis One or more specific environmental agents trigger an inflammatory response in the immune system Epitheloid cell granulomas are due to cell mediated immunity Activated macrophages and T cells release IL-1, fibronectin and macrophage derived growth factor Activated T cells release IL-2, monocyte chemotactic factor, immune interferon Dr. Varun

Histologic findings:

Histologic findings Histologic hallmark – noncaseous granulomas Central core of histiocytes , epitheloid cells, multinucleated giant cells CD4 Surrounded by lymphocytes, scattered plasma cells, varying quantities of fibroblasts and collagen in the periphery CD8 Giant cells may contains intracytoplasmic inclusions – asteroid bodies and Schaumann bodies Necrotizing sarcoid granulomatosis – variant Dr. Varun

Distribution :

Lung distribution of granulomas follow lymphatics Peribronchovascular interstitial space Subpleural interstitial space Interlobular septa Upper lobes severely affected Vascular involvement in >50% patients Dr. Varun Distribution

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Dr. Varun

Staging and prognosis:

Staging and prognosis Dr. Varun

Staging and prognosis:

This staging came in based on radiograph findings At presentation Stage 0 – 5-10% Stage 1 – 50% Stage 2 – 25-30% Stage 3 – 10-12% Radiographic stage does not correlate with PFT Dr. Varun Staging and prognosis

Diagnosis :

Diagnosis 4 steps Histologic verification Extent and severity of organ involvement Assessment if disease is stable or likely to progress Determine if patient will benefit from treatment D/D TB, berylliosis , leprosy, hypersensitivity pneumonitis , Crohns , primary biliary cirrhosis, fungal disease, chemo, RT Transbronchial , CT guided or surgical biopsy Cervical mediastinoscopy , Chamberlain procedure ( parasternal mini thoracotomy ), video assisted thoracoscopic surgical biopsy Endobronchial USG Dr. Varun

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Dr. Varun

Role of HRCT:

Role of HRCT Dr. Varun

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Dr. Varun

PowerPoint Presentation:

Dr. Varun

Typical patterns of lymphadenopathy:

Typical patterns of lymphadenopathy Dr. Varun Bilateral hilar lymph node enlargement D/D – fungal/ mycobacterial infection, lymphoma

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Dr. Varun

Atypical patterns of lymphadenopathy:

Atypical patterns of lymphadenopathy More common in patients diagnosed > 50yrs of age Internal mammary, preverterbal , retrocrural (Tb, lymphoma) Isolated unilateral hilar (right) Mediastinal without hilar Calcified lymph nodes. 3% calcify after 5 yrs, 20% after 10yrs Amorphous, popcorn or egg shell (silicosis, tb , histoplasmosis ) Dr. Varun

Typical parenchymal manifestations:

Typical parenchymal manifestations Micronodules with perilymphatic distribution Sharply defined, small (2-4mm) rounded nodules, with bilateral and symmetric distribution, predominantly in upper and middle zones Subpleural peribronchovascular interstitium , less often in interlobular septa Nodules may coalesce to form macronodules Nodular or irregular thickening of peribronchovascular interstitium . Dr. Varun

Typical parenchymal manifestations:

Fibrotic changes 20% progress to fibrosis Linear opacities, traction bronchiectasis , and architectural distortion (displacement of fissures and bronchovascular bundles) Upper and mid zones with a patchy distribution Secondary PAH and right heart failure RV enlargement, dilated main right and left pulmonary arteries, attenuation of peripheral vessels Dr. Varun Typical parenchymal manifestations

Typical parenchymal manifestations:

Bilateral perihilar opacities Confluent nodular opacities appear as bilateral areas of lung consolidation with irregular edges and blurred margins, radiating from hilum towards periphery Dr. Varun Typical parenchymal manifestations

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

Atypical parenchymal manifestations :

Atypical parenchymal manifestations Pulmonary nodules and masses 15-25% cases Ill defined irregular opacities 1-4cm in diameter – coalescent interstitial granulomas Multiple, bilateral, perihilar or peripheral lesions May be a/w air bronchograms May cavitate <3% Small satellite nodules - galaxy sign Dr. Varun

Atypical parenchymal manifestations :

Sarcoid cluster Multiple micronodules distributed along the lymph vessels on HRCT Deep subpleural locations, peripheral regions of upper and middle zones Parenchymal conglomerate masses with peribronchovascular fibrous tissue – upper and mid zones Berylliosis , tb , talcosis,silicosis Dr. Varun Atypical parenchymal manifestations

Atypical parenchymal manifestations :

Patchy air space consolidation 10-20% cases Bilateral, symmetric, mid and upper zones, peribronchovascular regions Consolidation fades to a nodular pattern in the periphery With or without bronchograms Confluence of numerous micronodules Acinar or alveolar sarcoidosis (pneumonia, TB, boop ) Dr. Varun Atypical parenchymal manifestations

Atypical parenchymal manifestations :

Patchy ground glass opacities 40% cases with parenchymal disease Airway compression due to fibrotic intersitital lesions Background of interstitial nodules Bronchoalveolar cell carcinoma, pneumoconiosis, pneumonia, bronchiolitis obliterans , organizing pneumonia Dr. Varun Atypical parenchymal manifestations

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

Atypical parenchymal manifestations :

Linear reticular opacities Isolated in 50% cases Interlobular and intralobular septal thickening Subpleural space upper and mid lung zones Mimic lymphangitic carcinomatosis Fibrocystic changes Fibrotic cysts, bullae , paracicatricial emphysema are in favor of advanced disease Upper and mid zones with perihilar distribution Posterior displacement of main or upper lobe bronchus with upper lobe volume loss Dr. Varun Atypical parenchymal manifestations

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

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Honeycomb like cysts Subpleural regions upper and mid zones Basal lung spared Cavitation of parenchymal lesions Primary cavitatory sarcoidosis – very rare Young individuals Pseudocavities lined by dense fibrous tissue Dr. Varun

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Mycetoma formation Complication of stage 4 cystic sarcoidosis Aspergillus species fungal balls develop in pre existing bullae and cysts Air fluid levels within sarcoid pseudcavities Mobile opacity occupying part or most of cavity bordered by peripheral sliver of air – air crescent or Monod sign Dr. Varun

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Dr. Varun

PowerPoint Presentation:

Dr. Varun

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Associated with massive hemoptysis – life threatening Second MC cause of death in sarcoidosis Walls of aspergillomas are supplied by bronchial arteries Hypothesis for hemoptysis Aspergillus invading blood vessel Effect of endotoxins produced Mechanical friction from fungal ball Inflammatory reaction – increased local VEGF, neoangiogenesis , fragile vessels Dr. Varun

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Miliary opacities Rare, <1% TB, pneumoconiosis, metastasis Histoplasmosis , chicken pox, LCH Airway involvement Mosaic attenuation pattern Small airway involvement by granuloma or fibrosis Air trapping Common non specific finding. Tracheobronchial abnormalities, atelectasis Right middle lobe preferentially affected – length, small caliber, acute angle of origin, proximity to lymph nodes Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

PowerPoint Presentation:

Dr. Varun

Pleural disease:

Pleural disease 1-4% cases Exudative / transudative pleural effusion hemorrhagic or chylous Pneumothorax Ruptured emphysematous bleb or necrosis sub pleural sarcoid granuloma Rarely pleural thickening/calcification Pleural plaque like opacities – pulmonary opacities simulating plaques Bilaterally, continguous with pleura, mostly upper and middle lung zones D/d: silicosis, pneumoconiosis Dr. Varun

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