2020 Volume 42 Issue 1 Pages 21-27
Background. IgG4-related disease develops in various organs, and the clinical features are very diverse. Case. A 62-year-old man had a chief complaint of a fever and productive cough. Computed tomography showed not only wall thickening in the trachea and central bronchi but also mucosal thickening in the paranasal sinus, mainly the maxillary sinus. Although bronchoscopy revealed marked bronchial mucosal edema and multiple white nodules, the bronchial mucosal biopsy specimens did not provide a definite diagnosis. Therefore, we performed a nasal septum biopsy and found the marked infiltration of IgG4-positive plasma cells with an IgG4/IgG ratio exceeding 50%. Due to the high value of serum IgG4 in addition to these findings, we diagnosed the patient with IgG4-related disease. After the administration of corticosteroids (prednisolone 35 mg/day), the patient's symptoms and the above imaging findings had improved. Conclusion. Wall thickening in the central airway is a rare condition in IgG4-related disease. A nasal septum biopsy might be useful for the diagnosis of IgG4-related disease.
