Necrotizing Sarcoid Granulomatosis in a Family of Patients with Sarcoidosis Reinforces the Association between Both Entities

Abstract

Necrotizing sarcoid granulomatosis (NSG) is a rare entity mainly characterized by a prominent granulomatous vasculitis affecting middle-aged or old individuals and with a favorable prognosis. Although many believe it is a variant of sarcoidosis, the proper classification is still a matter of debate as some of its features are found in sarcoidosis but also in Churg-Strauss syndrome, Wegener’s disease and hypersensitivity pneumonitis. In this paper, we described for the first time a case of NSG in a family with several cases of sarcoidosis, reinforcing the relationship between NSG and sarcoidosis. Additional interesting findings were the young age of the patient (15 years old), the symptoms limited to the respiratory tract (uncommon when NSG affects youngsters) and the increase in serologic markers of autoimmune disease. Though complete criteria for autoimmune disease were not present, systemic lupus erythematosus and Sjögren’s syndrome are possible candidates. As sarcoidosis is described to be associated with several autoimmune diseases, this finding is an additional suggestion of the relationship between both entities.

Necrotizing Sarcoid Granulomatosis With an Uncommon Manifestation: Clinicopathological Features and Review of Literature

“…Moreover, the vasculitis of Wegener's granulomatosis is more necrotizing and often suppurative. 18,19,27 A differential diagnosis with infection is more difficult: conventional microbiological analysis and use of overall tissue ancillary techniques such as special stains, immunohistochemistry, and molecular analysis (the latter for mycobacterial detection) are important. Above all, the use of a highly specific and sensitive molecular technique such as nested polymerase chain reaction for mycobacterial detection is mandatory.…”

Connecting the Dots: A Rare Cause of Pulmonary Nodules in a 13-Year-Old Boy

“…1 Since then, nearly 100 adult cases and 10 pediatric cases have been reported in the literature. [2][3][4][5][6][7][8][9][10][11] Extrapulmonary involvement is estimated to occur in nearly 86% of adult patients, and has been reported in half of the pediatric cases reported. 2,3 The majority of patients experience resolution of their disease, and some require steroids or immunosuppression, particularly those with more severe systemic or neurologic manifestations.…”

“…15,16 Lazzarini et al reported a 15-year-old girl with NSG and a strong family history of sarcoidosis, and suggested a possible autoimmune link between the two diseases. 8 However, the peripheral location of the nodules, lack of hilar adenopathy, and normal angiotensin converting enzyme would suggest that NSG and sarcoidosis are distinct entities. NSG shares some pathologic features with granulomatous angiitides, but is not associated with an elevated ANCA, renal disease, or asthma.…”

Four Decades of Necrotizing Sarcoid Granulomatosis: What Do We Know Now?

“…Nine of the publications reporting 9 cases of NSG did not meet the histologic criteria for NSG and were considered to be NS. [50][51][52][53][54][55][56][57][58] Six NSG publications encompassing 8 cases were rejected for this study for the following reasons: did not meet the histologic criteria for NSG 36,46 ; tuberculosis was the likely diagnosis 30,47 ; no pathologic examination of lung tissue and no convincing evidence of cutaneous vasculitis 40 ; the diagnosis presented was ''polyarteritis overlap syndrome.'' 26 The publication by Chittock et al 6 presented 7 patients stated to have NSG, but vasculitis was said to be present in only 4.…”