A case of IgG4-related disease with bronchial asthma and chronic rhinosinusitis in Korea.

IgG4-related disease (IgG4-RD) is characterized by a systemic involvement of tumor-like lesions with IgG4-positive plasmacytes. We experienced a case of IgG4-RD developed in a patient with bronchial asthma (BA) and chronic rhinosinusitis (CRS). A 55-yr-old female patient with BA and CRS complained of both eyes and neck swelling as well as a recurrent upper respiratory infection in recent 1 yr. The serum levels of IgG4, creatinine, and pancreatic enzymes were elevated. A biopsy of the submandibular gland showed an abundant infiltration of IgG4-positive plasmacytes. Her symptoms remarkably improved after the treatment of a systemic steroid that has been maintained without recurrence. We report a rare case of IgG4-RD developed in a patient with BA and CRS. Graphical Abstract Keywords: IgG4-Related Disease, Asthma, Bronchitis, Chronic INTRODUCTION IgG4-related disease (IgG4-RD) is an immune-mediated disease which is newly recognized as a systemic disease. It was initially described there was a relationship between autoimmune pancreatitis and IgG4 molecules in 2001 (1). However, several recent studies have revealed that IgG4 molecules have a relationship not only with the pancreas but also with the salivary gland, lung, liver, stomach, colon, and lymph node (2, 3). IgG4-RD has been reported in almost every organ system and is characterized by the formation of tumor-like lesions, storiform fibrosis and infiltration of IgG4-positive plasmacytes in involved organs. A notable study reported that approximately 44% of patients with autoimmune pancreatitis (AIP) also had allergic diseases (4). However, there have been few reported cases of this disease with comorbid allergic diseases due to the low prevalence of IgG4-RD. We report a first case of IgG4-RD accompanied with bronchial asthma (BA) and chronic rhinosinusitis (CRS) in Korea.