IgG4-related disease (IgG4-RD) is an inflammatory condition that exhibits diffuse or local organ swelling and hypertrophic changes with elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in multiple organs. The patient was a 72-year-old Japanese man, in whom magnetic resonance imaging (MRI) revealed retroperitoneal fibrosis with elevated serum IgG4 levels. He was referred to our department for a possible salivary gland biopsy to avoid invasive examination in the retroperitoneal region. Examinations with computed tomography and MRI failed to detect significant abnormalities in the salivary glands. Labial salivary gland biopsy was then performed under local anesthesia and resulted in diagnosis of IgG4-RD with typical histopathological findings (marked lymphoplasmacytic infiltration, fibrosis, and infiltration of IgG4-positive cells: IgG4⁺/IgG⁺ >40% and >10 IgG4⁺ plasma cells/HPF). This case suggests that labial salivary gland biopsy is useful as a low-invasive method for diagnosis of IgG4-RD, although a biopsy targeting the site of origin is ideal.

Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is defined as “other iatrogenic immunodeficiency-associated lymphoproliferative disorder” and has recently been reported in many patients with rheumatoid arthritis (RA) under treatment with MTX. Here, we report a case of an 81-year-old woman with RA under MTX treatment who complained of taste disorder. A hard palate fistula and sinus tumor were found to have destroyed the nasal septum. Oral and nasal biopsies were performed, and histopathological results showed B-cell non-Hodgkin lymphoma, which is most common in MTX-LPD. After diagnosis of MTX-LPD, the patient stopped taking MTX and remission was achieved. This case indicates the importance of awareness of MTX-LPD and of taking a medical and drug history in diagnosis of a case with suspected MTX-LPD.

Vertigo is a common occurrence and peripheral vertigo is most frequent and may occur as a symptom of Pendred syndrome. In addition to vertigo, this syndrome is associated with severe sensorineural hearing loss, an enlarged vestibular canal, and goiter, but these symptoms do not always occur at the same time and are often diagnosed individually. Here, we report the case of a patient with Pendred syndrome who had suffered from severe deafness since early childhood and developed severe vertigo in middle age. At the time of admission to the referral hospital, mixed horizontal-torsional nystagmus was prominent in a positional nystagmus examination. A pure tone audiometry test showed bilateral severe sensorineural hearing loss, and imaging showed marked bilateral vestibular aqueduct enlargement and goiter swelling. We subsequently made a diagnosis of Pendred syndrome based on these observations. Vertigo was improved by treatment with anti-vertigo drugs. Other symptoms and findings of Pendred syndrome are currently being followed up in cooperation with the referral hospital.