Abstract Introduction Sarcoidosis is a multisystem granulomatous disease of unknown etiology, characterized by noncaseating granulomas in affected organs. Necrotizing sarcoid granulomatosis (NSG) is a rare variant of sarcoidosis characterized by sarcoid-like noncaseating granulomas with areas of necrosis and granulomatous vasculitis, most often involving the lungs. Its histologic necrosis and radiologic findings can mimic infection, vasculitis, or malignancy, leading to diagnostic delay and extensive exclusion of alternative causes before confirmation. Our case exemplifies a rare form of extra-pulmonary necrotizing sarcoidosis. Case Description A 46-year-old female with no significant past medical history presented with acute dyspnea. She was found to have new acute diastolic heart failure with pericardial and lymphocytic predominant exudative pleural effusions on thoracentesis. Computed tomography (CT) noted multiorgan calcification of hilar and mediastinal lymph nodes, spleen, and liver. Interferon-gamma release assay, ANCA, rheumatoid factor, antibodies to double stranded DNA were negative while ANA was positive, ESR 121 and CRP 143. Pleuroscopy with pleural biopsy confirmed non-necrotizing granulomas. Positron emission tomography further denoted tracer avid hilar and mediastinal lymph nodes most suspicious for multi-system sarcoid. Upon discharge, the patient was initiated on goal directed therapy for heart failure as well as prednisone for sarcoidosis. On her 4-month follow up, the patient reported a painless supraclavicular lump with no associated fevers, chills, weight loss or night sweats. Magnetic resonance imaging of the neck highlighted a hypodense and irregular 3cm by 3cm mass concerning for necrosis. Biopsy of the mass noted necrotizing granulomas with CD45+ staining indicating necrotic lymphoid population, with sterile cultures, including for acid-fast bacteria. Flow cytometry was negative for lymphoma. The patient was transitioned from prednisone to methotrexate, with interval improvement in her dyspnea. Diffusion capacity on pulmonary function test and ejection fraction on echocardiogram both demonstrated recovery. Discussion Necrotizing lymphadenopathy in young females with sarcoidosis has been previously described in literature. The coexistence of both necrotizing and non-necrotizing granulomatous sarcoidosis across two anatomical locations in the same patient is exceptionally rare. This case highlights the diversity in radiologic findings that can be seen in the same patient with multisystem sarcoid. Serial or multisite biopsies may reveal variable histologic patterns within the same patient, and infections such as tuberculosis, and malignancy such as lymphoma should be ruled out. This abstract is funded by: None
Dolan et al. (Fri,) studied this question.