Sarcoidosis is a chronic disorder that can virtually affect any organ system in the body. Histologically, it is characterized by the presence of T lymphocytes, mononuclear phagocytes, and noncaseating granulomas. Most commonly affected are the intrathoracic structures, with 90% of the reported cases involving the lungs. Esophageal involvement in sarcoidosis is extremely rare. Dysphagia is the most common presentation in these patients and can be attributed to various mechanisms such as direct esophageal wall infiltration, extrinsic compression, cranial neuropathy, and brainstem involvement. A thorough online literature review revealed only 23 reported cases of esophageal involvement in sarcoidosis. This paper reviews these reported cases in detail along with newer diagnostic and treatment options, including direction of future therapy. 1. Introduction Sarcoidosis is an inflammatory, granulomatous, multisystem disorder of unclear etiology [1]. The lungs are predominantly involved, but it can entail involvement of any other organ or organ systems such as the skin, lymphatics, heart, musculoskeletal, neurological, and gastrointestinal system [2, 3]. The first description of sarcoidosis was published by Jonathan Hutchinson in 1877, where he described a patient with multiple raised purple lesions on his skin [4]. However, it was Caesar Boeck in 1899 who coined the term “sarkoid,” due to the histological appearance of the skin lesion that he thought was similar to sarcoma [5]. Sarcoidosis is a global disease with variable incidences, presentation, and prognosis. It has the highest incidence in the United States and Sweden [4]. In the United States, it is more common in African Americans with an age adjusted annual incidence rate of 35.5 in 100,000, whereas in Caucasians it is 10.9 in 100,000. The lifetime risk of developing sarcoidosis is 2.4% in African Americans compared to 0.85% in whites [6]. Sarcoidosis also has a preponderance to occur more frequently in women as compared to men. Though it can affect any age group, sarcoidosis tends to affect individuals aged 40 years or younger. However, in Japanese and Scandinavian women, the disease process appears to be biphasic with the prevalence most common in the third and seventh decades of life [7]. Clinical presentation also varies among individuals in different parts of the world. Lofgren’s syndrome, which is an acute form of sarcoidosis, with patients manifesting with a triad of bilateral hilar lymphadenopathy, arthritis and erythema nodosum, is more common among those hailing from Southern Europe. African
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