The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study

Abstract

ObjectiveThis study aimed to examine the natural course and relapse rate of IgG4-related ophthalmic disease (IgG4-ROD) after debulking surgery in Japanese patients.Methods and analysisThis retrospective review included patients with IgG4-ROD who did not undergo further treatment following debulking surgery. The patients were diagnosed between January 2009 and December 2018 at the Department of Ophthalmology and Pathology, Niigata University Medical and Dental Hospital. The main outcome measures included postoperative IgG4-ROD recurrence rate and differences between patients with and without recurrent disease.ResultsFifteen patients (six male, 9 female; 61.8±16.2 years) were included. Twelve patients (80.0%) had dacryoadenitis disease and three patients (20.0%) had orbital fat tissue disease. About 70%–100% of the lesion was resected in the debulking surgery and the pathological diagnosis was rendered. A definitive diagnosis was made in 13 cases (86.7%) and a probable diagnosis in 2 cases (13.3%). Patients were followed up for 39.0±25.5 months following operation. All patients had lesion volume reduction and patients with dacryoadenitis had eyelid swelling improvement after surgery. Two patients (13.3%) had disease recurrence and six patients (40.0%) had extraophthalmic lesions. There was no statistically significant difference in clinical features between relapsed and non-recurring cases.ConclusionWe observed a 13.3% relapse rate following debulking surgery in patients with IgG4-ROD who did not undergo further treatment. This rate is lower than the documented relapse rate of 30%–70% following oral prednisolone therapy. Therefore, debulking surgery may be a treatment option for IgG4-ROD.

The Role of Symptom Duration and Serologic Factors in the Relapse of IgG4-Related Ophthalmic Disease following Surgery: A Retrospective Cohort Study

“…Tacelli et al revealed that maintenance steroid therapy lasting longer than 1 year could reduce the risk of relapse [ 24 ]. Surgical treatment as an alternative treatment for IgG-ROD has been shown to have a lower relapse rate than glucocorticoid therapy [ 25 ]. All the IgG4-ROD patients in our cohort had a relapse rate of 38.9%, and in particular, those in the short-duration group had a relapse rate of 26.9%; these findings confirmed the results of previous studies, suggesting that surgical treatment for IgG4-ROD likely has a lower relapse rate than glucocorticoid treatment.…”

Surgical management for IgG4-related ophthalmic disease by a transcranial biopsy combined with extraorbital decompression: illustrative case

“…Ominato et al 13 reported a case series of patients with IgG-ROD treated solely with surgical debulking. The results showed that debulking surgery could be a treatment option for IgG4-ROD.…”

Debulking Surgery Combined with Low-Dose Oral Prednisolone and Azathioprine for Intractable IgG4-Related Orbital Disease: A Case Report

“…Ominato et al [ 13 ], in 2019, also reported that debulking surgery might be an effective treatment for IgG4-dacryoadenitis with a relapse rate of 13.3%, which is much lower than the reported relapse rate (26–50%) of the prednisolone therapy. However, compared with the cases in the Ominato et al [ 13 ] study, our case was more complicated with not only the lacrimal gland involvement but also involvement of the right eyelid and bilateral supraorbital nerves. Moreover, baseline elevations of the value of serum IgG4 (1970 mg/dL) and eosinophilia are strongly associated with the greater risk of subsequent relapse [ 7 ].…”