Pulmonary Necrotizing Granulomas in a patient with familial mediterranean fever

Abstract

We herein report a case of familial Mediterranean fever (FMF) presenting with granulomatous lung lesions with neuronal apoptosis inhibitory protein (NAIP), MHC class II transcription activator (CIITA), incompatibility locus protein from Podospora anserina (HET-E), and telomerase-associated protein (TP1) (NACHT) leucine-rich-repeat 1-positive inflammatory cell infiltrates. FMF is an autoinflammatory disorder characterized by recurrent and self-limited attacks of pyrexia, arthritis and erysipelas-like skin lesions. Lung disorders associated with FMF are extremely rare. This is the first report of an immunologically-confirmed case of pulmonary manifestations of this disease.

Keywords:

• familial Mediterranean fever
• Immunohistochemistry
• Lung

Acknowledgements

We thank Dr. Yoshinori Kawabata (Division of Pathology, Saitama Cardiovascular Respiratory Center, Saitama), Dr. Kenji Kashima and Ms. Kanako Ito (Oita University Faculty of Medicine, Oita) for their valuable assistance in the pathological diagnosis.

Conflicts of interest

None.