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5, 6, 7, 8, 9, 10, 11, 12 Pulmonary sarcoidosis can be categorized into 4 stages as described in Table 1 and Figure 1, Figure 2, Figure 3 through through4 4.
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The most commonly affected organs in sarcoidosis are the lungs and intrathoracic lymph nodes (over 90% of patients). The purpose of this review is to summarize contemporary knowledge about the epidemiology, clinical manifestations, diagnosis, and treatment of sarcoidosis. 3, 4 Because of its heterogeneous manifestations and relative lack of data on treatment efficacy, managing patients with sarcoidosis is often a challenge for clinicians. It is generally thought that both genetic predisposition and environmental factors play essential roles in its pathogenesis. 1, 2 The etiology of sarcoidosis is not known with certainty despite decades-long effort. The hallmark of sarcoidosis is the presence of noncaseating granuloma, a cluster of macrophages, epithelioid cells, mononuclear cells, and CD4 + T cells with a few CD8 + T cells in the peripheral zone. Sarcoidosis is a multisystem disorder that can affect practically any organ of the body. Glucocorticoids are the first-line therapy, whereas glucocorticoid-sparing agents and biologic agents are used in refractory/recurrent cases.Treatment is generally reserved for patients with disabling symptoms or with progressive organ damage/dysfunction because spontaneous remission is frequent.The diagnosis of sarcoidosis relies on the presence of noncaseating granuloma on histopathologic examination, compatible clinical presentation, and exclusion of other causes of granulomatous inflammation.Sarcoidosis is a systemic disease that can affect any organ, with the lungs and intrathoracic lymph nodes being the most frequently affected sites.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. Glucocorticoids are the cornerstone of treatment of sarcoidosis even though evidence from randomized controlled studies is lacking. Spontaneous remission is frequent, so treatment is not always indicated unless the disease is symptomatic or causes progressive organ damage/dysfunction. Diagnosis requires the presence of noncaseating granuloma and compatible presentations after exclusion of other identifiable causes. Clinical presentations of sarcoidosis are diverse, ranging from asymptomatic, incidental findings to organ failure. Although intrathoracic involvement is the hallmark of the disease, present in over 90% of patients, sarcoidosis can affect virtually any organ. The focus of this review is current knowledge about the epidemiology, clinical manifestations, diagnosis, and treatment of both pulmonary sarcoidosis and extrapulmonary sarcoidosis.