IgG4-Related Disease in the Frontal Convexity Concomitant with Smoldering Multiple Myeloma: A Case Report and Review of the Literature Regarding Therapeutic Implications

Tanaka, Toshihide; Fuga, Michiyasu; Teshigawara, Akihiko; Hasegawa, Yuzuru; Nishiwaki, Kaichi; Murayama, Yuichi; Yokoo, Hideaki

doi:10.1016/j.wneu.2020.07.212

Cited by 3 publications

(1 citation statement)

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“…It is to be noted that postoperative nonrecurrence of IgG4-RD has been documented as an additional advantage of surgical intervention, in patients who are good candidates for surgical management. [9,10,17,27,28,36] Our case also highlights the pitfalls of steroid therapy. Our patient had poor response to initial steroid therapy, which led to the need for surgical intervention.…”

Section: Discussionmentioning

confidence: 76%

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Mayeku

Deisch

López-González

2021

Surgical Neurology International

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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