Hypertrophic rhinitis without sinusitis histologically compatible with IgG4-related disease

IgG4-related disease (IgG4-RD) is a chronic inflammatory disease of unknown etiology that causes mass-forming lesion consisting of lymphoplasmacytic infiltrates, fibrosis and sclerosis especially in exocrine tissues. Although chronic paranasal sinusitis is known to be a nasal manifestation of IgG4-RD, little is known whether chronic rhinitis accompanies IgG4-RD. Here we report a case with hypertrophic rhinitis without sinusitis histologically compatible with IgG4-RD. An 80-year-old male complained bilateral nasal obstruction with occasional oral breathing and watery rhinorrhea for several years. Nasal endoscopic examination showed a pale and swollen anterior part of bilateral inferior turbinate leading to almost complete blockade of common meatus. Levels of serum total IgE and IgG4 were normal, and serum specific IgE to major inhaled antigens was negative. Due to resistance to conservative treatments, we performed an endoscopic mucosal resection of bilateral anterior part of inferior turbinate. The pathological findings met the diagnostic criteria for IgG4-RD. Additional examinations did not find lesion suspicious of IgG4-RD in other organs. After surgery, symptoms and objective nasal passage were improved with the use of intranasal corticosteroid. In case of hypertrophic rhinitis with severe swelling especially in the anterior part of the inferior turbinate, IgG4-related rhinitis should be suspected.