Abstract
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.
Introduction
IgG4-related disease (IgG4-RD) is a chronic inflammatory disease of unknown etiology that causes infiltration of IgG4-positive plasma cells and fibrosis, leading to swelling, nodules and hypertrophic lesions in various organs [ ]. It is known that IgG4-RD involves paranasal sinuses [ , ]. In addition, IgG4-positive cells were even found in sinonasal tissues of chronic rhinosinusitis patients without IgG4-RD [ ]. However, little is known whether IgG4-RD or IgG4-positive cells is associated with rhinitis alone. Here we reported a case with hypertrophic rhinitis without sinusitis in which histological finding was compatible with IgG4-RD.
Case presentation
A 80-year-old male complained bilateral nasal obstruction especially at night for several years. He also had dry mouth and insomnia due to oral breathing. He visited a hospital where hypertrophic rhinitis was pointed out. A physician did not recommend surgery due to risk for bleeding because the patient took anti-coagulants for angina pectoris. Because conservative treatment such as antileukotrienes was not effective, he took over-the-counter nasal decongestant. After continuous use, his quality of life (QOL) was strongly impaired due to exacerbation of the nasal obstruction and insomnia, then he visited a previous physician and was referred to our hospital whether he could receive laser surgery for hypertrophic rhinitis.
On his first visit to our hospital, he complained bilateral nasal obstruction with occasional oral breathing and watery rhinorrhea. He did not complain other nasal symptoms such as sneezing, postnasal drip, epistaxis, pain or loss of smell.
Nasal endoscopic examination showed swelling of anterior part of bilateral inferior turbinate leading to almost complete blockade of common meatus ( Fig. 1 A and B). In contrast, the swelling was mild in the posterior part. Serous discharge was also seen. Serum total IgE and IgG4 was 9 IU/ml and 42.6 mg/dL, respectively, both of which were within normal limit. Serum antigen-specific IgE (ImmunoCAP specific IgE, Thermo Fisher Diagnostics K.K., Tokyo, Japan) for major inhaled antigens, including mites, pollens, animal epithelium, fungi, and insects was negative. Computed tomography (CT) also showed bilateral swelling of anterior part of inferior turbinate with no abnormal shadow in paranasal sinuses ( Fig. 1 C and D). The nasal cytology for eosinophil was negative.
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