Necrotizing sarcoid granulomatosis: A distinctive form of pulmonary granulomatous disease

Abstract

Objectives: To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognised by pulmonologists and pathologists.

Data source: PubMed was searched for the term necrotising or necrotizing sarcoid granulomatosis.

Study selection: All cases reported in the English literature were included.

Results: NGS is presented at all ages (range 8-68 years) with a median age of 42 years old. It shows female (62%) and Caucasian (80%) predominance. The most frequent symptoms are cough, fever, dyspnoea and chest pain. Extra-pulmonary involvement is found in one third of the cases, with ocular being the most common (12.5%). At imaging, multiple nodules (64.75%) or a solitary mass (20.49%) are found accompanied by mediastinal lymphadenopathy at one third of the cases. It can be clinically mistaken for malignancy as it is tumour-like, increases rapidly in size and it is hyperfixating in PET-SCAN. Histologically, NGS is defined by large areas of necrosis, well-formed granulomas and vascularitis.

Conclusion: NGS is a disease often confounded clinically with malignancy or with sarcoidosis even histologically when all criteria are not strictly applied. This review provides NGS' characteristics and discusses its differential diagnosis form sarcoidosis, Wegener granulomatosis and tuberculosis.

Keywords: Wegener granulomatosis; granulomatosis; lung; necrotizing sarcoid granulomatosis; sarcoidosis.