114 Sarcoidosis

CASE 114


image Clinical Presentation


52-year-old woman with dyspnea and uveitis


image Radiologic Findings


PA (Fig. 114.1) and lateral (Fig. 114.2) chest radiographs demonstrate profuse bilateral small pulmonary nodules most numerous in the upper and middle lung zones in association with symmetrical bilateral hilar lymphadenopathy. HRCT (Figs. 114.3, 114.4) reveals diffuse bilateral multi-focal micronodules distributed along perilymphatic areas, subpleural regions, interlobular septa, and bronchovascular bundles


image Diagnosis


Sarcoidosis


image Differential Diagnosis


• Pulmonary Infection


• Silicosis


• Lymphoproliferative Disorder


• Pulmonary Metastases



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Fig. 114.1 (Reproduced with permission from Miller BH, Rosado de Christenson ML, McAdams HP, Fishback NF. Thoracic Sarcoidosis: Radiologic-Pathologic Correlation. Radiographics 1995;15:421–437.)



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Fig. 114.2




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Fig. 114.3



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Fig. 114.4


image Discussion


Background


Sarcoidosis is a systemic granulomatous disease that frequently affects the lung and the lymphatic system. The diagnosis is established based on typical clinical and radiologic findings supported by histologic evidence of non-caseating epithelioid granulomas in more than one organ.


Etiology


The etiology of sarcoidosis remains unknown. An abnormal immune-mediated response to a yet unidentified antigen has been postulated.


Clinical Findings


Most affected patients are under the age of 40 years, with a peak incidence between 20 and 29 years. Annual incidence in the United States is 35.5 per 100,000 persons for African Americans and 10.9 per 100,000 for Caucasians. High disease prevalences are also reported in Swedes and Danes. Löfgren syndrome is an acute form of sarcoidosis characterized by fever, polyarthralgias, erythema nodosum and bilateral hilar lymphadenopathy. Heerfordt syndrome is the association of fever, parotid enlargement, facial palsy, and anterior uveitis. Lupus pernio is a chronic form of sarcoidosis typified by indurated plaques and raised discoloration of the central face and ears.


Approximately 50% of patients with sarcoidosis are asymptomatic. Symptomatic patients may present with dry cough, dyspnea, chest pain, and hemoptysis. Non-specific symptoms are also reported, including constitutional symptoms, fatigue, weight loss, malaise, and fever. Pulmonary function studies may reveal restrictive abnormalities and decreased DLCO, but obstructive abnormalities can be seen with endobronchial sarcoidosis. Laboratory abnormalities include elevation of serum angiotensin converting enzyme (ACE), decreased blood serum CD4 to CD8 ratios, and hypercalcemia due to increased intestinal calcium absorption and activation of vitamin D by the sarcoid granulomas. Approximately 20% of patients develop pulmonary fibrosis, which may lead to pulmonary insufficiency and pulmonary hypertension.


Imaging Findings


Chest Radiography

Radiographic Staging System

image Stage 0: normal chest radiographs


image Stage 1: hilar and/or mediastinal lymphadenopathy


image Stage 2: lymphadenopathy and parenchymal abnormalities (Figs. 114.1, 114.2

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Jan 14, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on 114 Sarcoidosis

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