[Clinical analysis of 9 cases of IgG4-related disease with head and neck organ involvement as the initial symptom].

Objective

To summarize the clinical features and diagnostic/management experience of diagnosis and treatment of IgG4-related disease (IgG4-RD) presenting with initial head-and-neck organ involvement.

Methods

A retrospective analysis was conducted of 9 IgG4-RD patients (7 males, 2 females; aged 54-65 years, with a median of 59 years) treated in department of Otorhinolaryngology Head and Neck Surgery of the Second Affiliated Hospital of Shanxi Medical University from May 2018 to January 2025. Descriptive statistics were used to analyze involved organs, clinical manifestations, imaging findings, histopathology, therapy and outcome.

Results

The initial head-and-neck involvement comprised bilateral submandibular glands in 4 cases, nasal cavity/paranasal sinuses in 2, unilateral parotid gland in 1, larynx in 1, and both nasal septum and subglottis in 1. Serum IgG4 levels were elevated in all cases, the range was 2 380-17 000 mg/L. All pathological findings fulfilled the diagnostic criteria for IgG4-RD. All patients were treated with systemic corticosteroids. During 6-80 months of follow-up, one case of relapse occurred and was controlled by increasing steroid dose and prolonging treatment course.

Conclusions

IgG4-RD is rare in clinic and their presentations are highly variable due to different organs involved. The head-and-neck region is one of the common initial sites. Obtaining a pathological diagnosis is particularly critical. Glucocorticoids remain the first-line therapy for IgG4-RD.