Abstract Introduction Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Necrotizing Sarcoid Granulomatosis is a rare disease, and to date, remains unclear if it’s a variant of Sarcoidosis or a distinct entity. Case report A sixty-six-year-old female with remote history of breast cancer in remission, presented to our office with chronic cough of two years duration. She denied dyspnea, chest pain, upper airway symptoms, fever, constitutional, and musculoskeletal symptoms. She completed two courses of antibiotics for presumed bronchitis. Vitals signs and physical exam were unremarkable. A Computed Tomography(CT) Chest (Fig-A/B) revealed multiple lung nodules, largest measuring 9mm in left lower lobe with mediastinal lymphadenopathy. A PET-CT (Fig C/D) revealed hypermetabolic thoracic and abdominopelvic lymphadenopathy suspicious for metastatic processes, given history of breast cancer. Additionally, new hypermetabolic focal consolidations were noted in right upper and lower lobes. Endobronchial ultrasound biopsy of mediastinal and hilar nodes were unrevealing for infection, inflammation, and malignancy. An excision biopsy of right paratracheal lymph node revealed focally necrotizing granulomatous lymphadenitis with extensive scarring, no infection or malignancy. CD4 to CD8 ratio was 2.3:1. Flow cytometry was unrevealing. An initial working diagnosis of Kikuchi’s disease was made. However, the resolving nature over 6 months, waxing and waning pulmonary nodules along with systemic lymphadenopathy prompted revisiting the histopathology. Upon multidisciplinary discussions, she was diagnosed with Necrotizing Sarcoid Granulomatosis, an atypical variant of Sarcoidosis. In the interim, extensive serological workup for infectious, inflammatory, rheumatological disorders was remarkable. She was treated with Prednisone 0.5mg/kg/day with a slow taper over six months, with clinical and radiographic resolution. Discussion Necrotizing Sarcoid Granulomatosis (NSG) is a rare disease of unknown etiology with sparse data. Seen predominantly in younger females with a median age of 42 years, and in nonsmokers. Presentation is nonspecific including fever, cough, dyspnea, pleuritic pain, weight loss. Radiographically, solitary or multiple nodular opacities with or without mediastinal and hilar lymphadenopathy is seen. It is a diagnosis of exclusion. Bronchoscopy is usually unrevealing, in contrast to Sarcoidosis, CD4 to CD8 ratio is normal. Histopathology is crucial in diagnosing, as other granulomatous processes like Tuberculosis, vasculitides etc. needs to be excluded. The presence of Sarcoid-like granulomas, necrosis, granulomatous vasculitis with no evidence for infectious etiology, like our patient, supports the diagnosis of NSG. Clinical course is mostly benign, often with spontaneous remission. If unresolving, corticosteroids remain treatment of choice, like in our patient. This abstract is funded by: None
Thimmareddygari et al. (Fri,) studied this question.