Clinical Manifestations, Diagnosis, and Treatment of Sarcoidosis

Abstract

The focus of this review is current knowledge about the epidemiology, clinical manifestations, diagnosis, and treatment of both pulmonary sarcoidosis and extrapulmonary sarcoidosis. Although intrathoracic involvement is the hallmark of the disease, present in over 90% of patients, sarcoidosis can affect virtually any organ. Clinical presentations of sarcoidosis are diverse, ranging from asymptomatic, incidental findings to organ failure. Diagnosis requires the presence of noncaseating granuloma and compatible presentations after exclusion of other identifiable causes. Spontaneous remission is frequent, so treatment is not always indicated unless the disease is symptomatic or causes progressive organ damage/dysfunction. Glucocorticoids are the cornerstone of treatment of sarcoidosis even though evidence from randomized controlled studies is lacking. Glucocorticoid-sparing agents and biologic agents are often used as second- and third-line therapy for patients who do not respond to glucocorticoids or experience serious adverse effects.

Keywords: ATS, American Thoracic Society; AV, atrioventricular; CMRI, cardiovascular magnetic resonance imaging; DLCO, diffusing capacity of the lung for carbon monoxide; DMARD, disease-modifying antirheumatic drugs; ECG, electrocardiographic; ERS, European Respiratory Society; FDG-PET, 18F-fluorodeoxyglucose–positron emission tomography; FVC, forced vital capacity; GI, gastrointestinal tract; LVEF, left ventricular ejection fraction; NSAID, nonsteroidal anti-inflammatory drug; PFT, pulmonary function test; TBB, transbronchial lung biopsy; TNF-α, tumor necrosis factor α; WASOG, World Association of Sarcoidosis and other Granulomatous Disorders.

Figure 1

Chest radiograph of a 31-year-old man with stage I pulmonary sarcoidosis, demonstrating bilateral hilar lymphadenopathy without evidence of parenchymal lung involvement.

Figure 2

Chest radiograph of a 62-year-old woman with stage II pulmonary sarcoidosis, demonstrating bilateral nodular parenchymal opacities in a perihilar and mid-lung distribution associated with bilateral hilar and aortopulmonary window lymphadenopathy.

Figure 3

Computed tomographic image of the chest of a 53-year-old woman with stage III pulmonary sarcoidosis, demonstrating numerous small pulmonary nodules that are upper-lobe predominant and perilymphatic in distribution.

Figure 4

Chest radiograph of a 54-year-old woman with stage IV pulmonary sarcoidosis, demonstrating extensive parenchymal scarring throughout both lungs, most marked in the upper lungs and in the perihilar regions.

Figure 5

Papular sarcoidosis as a cutaneous manifestation seen on the upper back region. Multiple erythematous raised lesions are evident.

Figure 6

Plaque sarcoidosis as a cutaneous manifestation seen on the upper arms. Multiple erythematous plaques are evident.