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[답] sarcoidosis 입니다.
환자는 lower leg 의 skin biopsy 를 통해 pathology 상 진단하였습니다.
Noncaseating granulomas, consistent with sarcoidosis.
[Additional report]
D-PAS stain for fungi and Ziehl-Neelsen stain for acid-fast bacilli are negative.
** Uptodate 해설
PULMONARY IMAGING — Lung involvement occurs in over 90 percent of patients with sarcoidosis [15,20]. The "classic" chest roentgenogram reveals bilateral hilar adenopathy. This finding, however, may be absent or, if present, may occur in combination with parenchymal opacities. Parenchymal opacities may be interstitial, alveolar, or both. Pleural involvement is unusual (<5 percent of patients), but can result in lymphocytic exudative effusion, chylothorax, hemothorax, and pneumothorax [21-23]. (See "Diagnostic evaluation of a pleural effusion in adults: Initial testing".)
Chest radiograph — The stage of pulmonary involvement is based upon the chest radiograph. Although the chest radiograph provides an anatomical guide to lung involvement, it cannot measure disease activity or assess functional defects. The radiographic stages are as follows:
Stage I — Stage I is defined by the presence of bilateral hilar adenopathy, which is often accompanied by right paratracheal node enlargement (image 1). Fifty percent of affected patients exhibit bilateral hilar adenopathy as the first expression of sarcoidosis. Regression of hilar nodes within one to three years occurs in 75 percent of such patients, while 10 percent develop chronic enlargement that can persist for 10 years or more.
Stage II — Stage II consists of bilateral hilar adenopathy and reticular opacities (the latter occurring in the upper more than the lower lung zones). These findings are present at initial diagnosis in 25 percent of patients (image 2). Two-thirds of such patients undergo spontaneous resolution, while the remainder either have progressive disease or display little change over time. Patients with stage II disease usually have mild to moderate symptoms, most commonly cough, dyspnea, fever, and/or easy fatigue.
Stage III — Stage III consists of reticular opacities with shrinking hilar nodes (image 3). Reticular opacities are predominantly distributed in the upper lung zones.
Stage IV — Stage IV disease is characterized by reticular opacities with evidence of volume loss, predominantly distributed in the upper lung zones (image 4). Conglomerated masses with marked traction bronchiectasis may also be seen. Extensive calcification and cavitation or cyst formation may also be seen [24].
Nodular sarcoid — The chest radiograph may show multiple, bilateral lung nodules and minimal hilar adenopathy, findings that may simulate metastatic disease. Nodular consolidation with ill-defined borders is seen by computed tomography [25].
CT scan — Chest computed tomography (CT) can demonstrate a variety of abnormalities in patients with sarcoidosis (image 5A-D) [26,27]:
§ Hilar and mediastinal lymphadenopathy
§ Beaded or irregular thickening of the bronchovascular bundles
§ Nodules along bronchi, vessels, and subpleural regions
§ Bronchial wall thickening
§ Ground glass opacification
§ Parenchymal masses or consolidation
§ Parenchymal bands
§ Cysts
§ Traction bronchiectasis
§ Fibrosis with distortion of the lung architecture
High resolution CT (HRCT) scanning reveals a mid-to-upper zone predominance of these changes. It can also detect parenchymal abnormalities that are not seen on the plain chest radiograph.
The HRCT findings may correlate with the histologic abnormalities. Ground-glass opacities, for example, are associated with sarcoid granulomas rather than alveolitis [28]. This finding has raised the question of whether alveolitis is a prominent feature of sarcoidosis [29]. Although it can occur, alveolitis is rarely identified in those with clinically significant disease.
PET scan — A fluorine-18-fluorodeoxyglucose (18F-FDG) PET scan may be helpful to identify occult lesions and possibly reversible granulomatous disease [30,31]. This test does not differentiate sarcoidosis from malignancy, as 18F-FDG PET may be positive in both processes. However, in a small study (24 sarcoid, 17 lung cancer), the combination of 18F-FDG and 18F-FMT (L-[3-18F]-methyltyrosine) PET scanning was able to differentiate sarcoidosis from malignancy; sarcoid lesions were positive on 18F-FDG PET, but negative on 18F-FMT PET (both are positive in patients with cancer) [32]. Additional studies are needed for confirmation. This tracer may not be available at all institutions.
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