A Rare Case Study About Necrotizing Granulomatous Sarcoidosis

Abstract

Sarcoidosis is a granulomatous disorder with an elusive etiology and pathogenesis. Classically, sarcoidosis is associated with non-caseating granulomas composed of mononuclear phagocytes, lymphocytes, and multinucleated giant cells. Necrotizing granulomas can also be associated with sarcoidosis but is scarcely reported in the medical literature. Necrotizing sarcoid granulomatosis is challenging to diagnose due to its rarity and similarity with other necrotizing disorders. Therefore, it is mainly considered a diagnosis of exclusion. We report one such case study, which could prompt further research to lay the course of treatment strategies for this disease. Moreover, our patient had a family history of sarcoidosis, which raises questions regarding possible genetic predisposition, and future work might help solve this medical mystery.

Sarcoidosis

“…6 The pathology triggers macrophages and T cells to propagate an immune response, leading to the accumulation of neutrophils and CD4 cells; the release of interleukin (IL) at disease locations, mainly the lungs, skin, and eyes; and increased cytokine (such as interferon) production. [6][7][8] The heterogeneous multisystem inflammatory response is the result of tumor necrosis factor (TNF), glycoprotein KL-6, and surfactant protein D (SP-D) release. This inflammatory response is classically characterized by the development and accumulation of noncaseating granulomas in many organ systems, which then change the normal structure of the organ and affect the organ function.…”

“…(See Granulomatous tissue formation in sarcoidosis. 7 ) Half of patients with sarcoidosis have elevated angiotensin-converting enzyme (ACE) levels. [6][7][8][9][10] In the giant cells of both infectious and noninfectious granulomas, spiderlike cytoplasmic inclusions may be present.…”

“…This inflammatory response is classically characterized by the development and accumulation of noncaseating granulomas in many organ systems, which then change the normal structure of the organ and affect the organ function. (See Granulomatous tissue formation in sarcoidosis 76-10.…”

“…7 ) Half of patients with sarcoidosis have elevated angiotensin-converting enzyme (ACE) levels. [6][7][8][9][10] In the giant cells of both infectious and noninfectious granulomas, spiderlike cytoplasmic inclusions may be present. 11 Although nonspecific, these inclusions should raise a concern for the diagnosis of sarcoidosis.…”