Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten

Abstract

Sarcoidosis is a systemic granulomatous disease of unknown aetiology characterised by the appearance of noncaseifying epithelioid granulomas in the affected organs, most commonly the lungs, skin, and eyes (Iannuzzi et al. 2007). Necrotizing Sarcoid Granulomatosis (NGS) is a rare and little-known form of disease, which also presents nodular lung lesions, and it shares pathologic and clinical findings with sarcoidosis, where the presence of necrosis may lead to misdiagnosis of tuberculosis (TB), leading to a consequent delay in treatment of the underlying entity (Chong et al. 2015). This is exactly what happened with the two cases that we present here.

Successful corticosteroid treatment of necrotizing sarcoid granulomatosis associated with tracheal lesion recurred after a surgical lung biopsy

“…Relative to sarcoidosis, NSG is a relatively rare disease with approximately 200 cases reported in the literature [ 5 ]. The clinical presentation of NSG includes pulmonary and/or systemic symptoms (i.e., fever, weight loss, night sweats, malaise).…”

“…The clinical presentation of NSG includes pulmonary and/or systemic symptoms (i.e., fever, weight loss, night sweats, malaise). Several organs have been reported to be involved, including eye, skin, lymph node, liver, spleen, and lacrimal gland [ 5 ]. Although a few sarcoidosis cases have been reported with tracheal polypoid lesions [ 12 , 13 ], there have been no reports of NSG accompanied by tracheal lesions.…”

“…NSG is a rare disease characterized by sarcoidosis-like granulomas and vasculitis with necrosis [ 2 ]. To date, approximately 200 cases have been reported in the world [ [3] , [4] , [5] ]. The clinical presentations of NSG include pulmonary symptoms, such as cough, dyspnea, chest pain, and extrapulmonary symptoms, such as general fatigue, fever, and weight loss [ 2 , 3 ].…”

Pulmonary sarcoidosis with arterial involvement

“…Above all, NSG shows a histopathological pattern of more severe necrosis and vasculitis. 2 However, other authors suggest that necrosis represents a final stage of the typical sarcoidosis when the vascular obstruction leads to necrosis. 3 NSG is a systemic disease, involving predominantly the lungs.…”

“…The diagnosis is commonly based on the histopathology, and despite the multisystemic involvement, NSG has a benign clinical course with a good response to corticoid therapy. 2 Herein, we describe a patient with NSG whose diagnosis was based on the exploration of a pulmonary nodule identified by chest imaging. This is the second case reported in Brazil.…”