SESSION TYPE: ILD Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: The common histopathologic features of pulmonary sarcoidosis consist of non-caseating, non-necrotizing granulomas with lymph node involvement. The presence of necrotizing granulomas raises concern for infectious or vasculitic processes. Although rare, necrotizing sarcoid granulomatosis (NSG) should be considered in the differential diagnosis. We present a case of NSG.
CASE PRESENTATION: A 44 year-old lady with history of breast cancer and bilateral mastectomy in 10/2009 presented in 10/2010 with exertional dyspnea. Her medical history included a negative PPD test and asthma since childhood. A never smoker, she worked as a real estate broker with possible mold exposure. Medications included albuterol and Advair inhalers. She had no allergies and family history non contributory. Examination was unremarkable. Chest CT scan showed bilateral scattered nodules concerning for metastasis (Figure 1) with PET scan showing FDG avidity. CT guided biopsy of a prominent nodule was non diagnostic. Wedge biopsy of a left upper lobe nodule revealed granulomatous inflammation involving lung parenchyma and blood vessels with organizing pneumonia and infarct like necrosis and thrombi within blood vessels (Figure 2). No significant neutrophilic or eosinophilic infiltration was seen. Bacterial, mycobacterial, fungal and viral stains and cultures were negative. Extensive blood work including ANA, ANCA, ACE levels, ESR, CRP, immunoglobulin and complement levels were normal. A diagnosis of NSG was made and she was started on oral prednisone with near complete resolution of CT scan abnormalities. She remains asymptomatic and off prednisone.
DISCUSSION: Most information available on NSG is from case reports/series. Common in non-smokers, the clinical course is usually benign with mostly pulmonary involvement only(1-3). Unlike Wegener’s or Churg-Strauss diseases, upper airway and renal involvement are rare. Uveitis or cutaneous involvement is rare(2). Bilateral infiltrates, adenopathy and nodules are the commonest radiological findings. Histopathology reveals sarcoid like granulomata with vasculitis and varying degrees of necrosis(1-3). Prognosis with NSG is excellent and most patients respond well to systemic steroids. The observations that NSG has a more benign clinical course, normal ACE levels, little extrapulmonary involvement, specific histopathological features and excellent prognosis have led to calls to classify NSG as a separate entity(1-3).
CONCLUSIONS: A diagnosis of NSG has important clinical implications as management and prognosis significantly differs from infectious or other autoimmune granunlomatous pulmonary disorders.
1) Liebow AA. The J Burns Amberson Lecture-Pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973;108:1-18.
2) Churg A et al. Necrotizing sarcoid granulomatosis. Chest 1979;76:406-413.
3) Quaden C et al. Necrotising sarcoid granulomatosis: clinical, functional, endoscopical and radiographical evaluations. Eur Respir J 2005;26:778-785
DISCLOSURE: The following authors have nothing to disclose: Satish Kalanjeri, Hangjun Wang, Andre Moreira, Diane Stover, Robert Lee
No Product/Research Disclosure InformationPulmonary Service, Department of Medicine, Memorial-Sloan Kettering Cancer Center, New York, NY