Immunoglobulin G4-Related Disease Presented as Recurrent Otitis Media and Mixed Hearing Loss Treated With Cyclophosphamide and Rituximab: A Case Report

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune disease, primarily affecting various organ systems such as the pancreas, biliary tree, and liver. The orbit, salivary glands, the thyroid, facial skin, the trigeminal nerve, and the cervical lymph nodes are often affected by IgG4-RD in the head and neck region. 1 Although the ear is rarely affected, few case reports (we found 11 cases, listed in Table 1) of otologic manifestation can be found in the literature. 2-6 We excluded one case in Israel which was presented only in the congress. Of the 11 cases, seven were reported in Asia, and all the 11 cases were treated with steroids, one of them with a combination of methotrexate. Herein, we report the 12 th case of IgG4-RD involving the middle and inner ear. We treated this patient with cyclophosphamide and rituximab, which have never been used to treat otologic symptoms, but were used to treat other organs affected by IgG4-RD. 7-10 Pure tone audiometry was recorded serially to define the clinical improvement. In addition, we found that early treatment may lead to better outcomes.

IgG4-related disease presenting as otogenic skull base osteomyelitis

“…There have been several reports of IgG4-RD with otologic manifestations [8 , 9] . For example, San et al reported one case with recurrent otitis media and mixed hearing loss, with 11 previously reported cases of IgG4-RD with otologic manifestations [9] . These cases were all related to hearing loss or otitis media, but no cases of IgG4-RD presenting as otogenic SBO have been reported.…”

Isolated Otologic Involvement of IgG4 Related Disease: A Case Report and Review of Literature

“…Furthermore, the patient experienced continued hearing loss at a five-week follow-up which is not unexpected. Improvement or remission of the disease process generally occurs once the systemic treatment is initiated; however, several months of persistent therapy is often required in order to see objective improvement on the audiogram [ 3 , 7 ]. Outcomes of immunosuppressive treatment are generally efficacious, with a narrowing of an air-bone gap of 40dB seen within several months following initiation of treatment [ 7 ].…”

“…While involvement within the head and neck region is relatively rare, conditions such as Mikulicz disease, Riedel's thyroiditis, and chronic sclerosing sialadenitis are all well-characterized IgG4 related processes [ 2 ]. Additionally, isolated otologic involvement of IgG4-RD is incredibly rare as only a few case reports are available in the literature [ 3 , 4 ]. In this article, we report a case of a 26-year-old female with a history of recurrent left-sided middle ear infections who presented to a tertiary care hospital with acutely worsening hearing loss, otalgia, and headache.…”

Update on classification, diagnosis, and management of immunoglobulin G4-related disease

“…The thyroid lesions presented with higher levels of circulating thyroid autoantibodies, subclinical hypothyroidism, larger thyroid size, and diffuse low sonographic echogenicity [53] . The gastrointestinal tract involvement often appears as inflammatory pseudotumor; wall thickening happens when the gallbladder is involved, whereas a pseudotumor can be noted in the liver, which can also develop into chronic hepatitis; involvement in ear-nose-throat regions presented with soft tissues occupied in the middle-ear/mastoid cavity or sinus or laryngopharynx; the skin involvement presents as subcutaneous nodules and/or skin plaques; the bone involvement can present with an inflammatory pseudotumor and bone erosion; the testes can develop IgG4-related epididymo-orchitis; the prostate involvement is easily misdiagnosed as chronic prostatic hyperplasia [53–63] . Although IgG4-RD in pediatrics is uncommon, it has been described in certain case reports.…”

“…[53] The gastrointestinal tract involvement often appears as inflammatory pseudotumor; wall thickening happens when the gallbladder is involved, whereas a pseudotumor can be noted in the liver, which can also develop into chronic hepatitis; involvement in ear-nose-throat regions presented with soft tissues occupied in the middle-ear/mastoid cavity or sinus or laryngopharynx; the skin involvement presents as subcutaneous nodules and/or skin plaques; the bone involvement can present with an inflammatory pseudotumor and bone erosion; the testes can develop IgG4-related epididymo-orchitis; the prostate involvement is easily misdiagnosed as chronic prostatic hyperplasia. [53][54][55][56][57][58][59][60][61][62][63] Although IgG4-RD in pediatrics is uncommon, it has been described in certain case reports. The commonly affected sites and diagnostic criteria in children were approximately the same as those in adults, and therefore, awareness is required for the pediatricians, when symptoms or signs similar to IgG4-RD presenting in a child.…”