Sarcoidosis extrapulmonary

Division of Pulmonary and Critical Care Medicine, Medical University of South Carolina, Charleston, South Carolina, U.S.A. 

Sarcoidosis is a multisystem granulomatous disease that most commonly affects the lung but may involve any organ. The manifestations of extrapulmonary sarcoidosis may vary from asymptomatic involvement to the presence of severe symptoms that may adversely impact the quality of life. 

The diagnosis of extrapulmonary sarcoidosis is often problematic. It is important to recognize when extrapulmonary symptoms should be considered to be manifestations of sarcoidosis. When extrapulmonary sarcoidosis is suspected, a diagnostic approach may be preferred that avoids the invasive biopsy of a visceral organ or biopsy confirmation altogether. 

The treatment of extrapulmonary sarcoidosis varies depending on the organs that are involved. This manuscript will outline the clinical aspects of extrapulmonary sarcoidosis in the most common and clinically important sites. Organ involvement will be discussed individually in terms of epidemiology, clinical manifestations, diagnosis, and treatment. 

The frequency of ocular involvement in sarcoidosis ranges between 10% and 50% in American and European studies (1). In these reports, extraocular disorders such as lacrimal gland involvement and sicca syndrome were included (1). Ocular sarcoidosis seems to be more common in the Japanese where eye involvement has been reported in 64% to 89% of sarcoidosis patients (1). In the United States, ocular sarcoidosis is more common in African Americans than Caucasians (2,3). The low rates of eye involvement with sarcoidosis in some series may be because of the lack of thoroughness in examination of the eye (1). 

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The spectrum of sarcoidosis is protean, and virtually any organ can be involved (1 ). Multisystemic involvement is characteristic, but pulmonary involvement usually dominates (l-3,5-7). A recent study of 736 patients with sarcoidosis in the United States found that 95% had intrathoracic disease (8). In the next chapter, Dr. Judson discusses extrapulmonary sarcoidosis in depth. In this chapter, we limit our discussion to pulmonary manifestations of sarcoidosis (6). 

Lynch J III, Baughman R, Shatma O. Extrapulmonary sarcoidosis. Semin Respir Infect 1998 13 229-254. 

Table 2 Clincial Criteria for Extrapulmonary Sarcoidosis Organ Involvement in Patients with Biopsy-Confirmed Sarcoidosis in Another Organ ... 

Judson MA. Extrapulmonary sarcoidosis. Semin Respir Crit Care Med 2007 28(1) 83-101. 

Ali Y, Popescu A, Woodlock TJ. Extrapulmonary sarcoidosis rapid spontaneous remission of marked splenomegaly. J Natl Med Assoc 1996 88 714—716. 

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