[A rare cause of eosinophilic asthma: Hyper-IgG4 syndrome (IgG4-related sclerosing disease)]

The hyper-IgG4 syndrome is an emerging disease with various thoracic manifestations: eosinophilic asthma, adenomegalies or mediastinal fibrosis, asthma, infiltrative pneumonia, nodules or pseudo-tumors with bronchial and pleural localizations.

We report the case of a 38-year-old woman who was admitted for acute exacerbation of eosinophilic asthma with high total IgE levels. A medical history of idiopathic acute pancreatitis, associated with sclerosing cholangitis and renal failure, suggested a diagnosis of hyper-IgG4 syndrome in this woman with late-onset severe and non-atopic asthma. A previous hepatic histology and a reinterpretation of renal tomodensitometry have confirmed this hypothesis.

The hyper-IgG4 syndrome is responsible of different clinico-radiological patterns that should be evoked because of the pejorative evolution of this steroid sensitive disease.