Abstract
Hyper-IgG4 syndrome, or IgG4-related systemic disease (IgG4-RSD), is a recently described entity of fibro-inflammatory systemic damage. Multiorgan lesions can occur synchronously or metachronously in a same patient. The common histological features include a lymphoplasmacytic infiltration (especially to IgG4), and fibrosis. Elevated serum level of IgG4 is also often present. The main concerned organs are: pancreas, biliary ducts, mediastinal lymph nodes, peritoneum, lacrimal and salivary glands. We report a case of an unusual location of this disease at the nasophyrnx in a patient of 84years for which the diagnosis was made through immunolabelling with IgG4 in lesions biopsied, and we illustrate the potential role of PET-FDG in this systemic disease.
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