Systemic Involvement in Immunoglobulin G4-Related Ophthalmic Disease

ABSTRACT

Background

Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) poses clinical challenges due to its heterogeneous ocular and systemic manifestations. We aim to report the systemic involvement and the clinical, serological and radiological associations of a cohort of Chinese patients.

Methods

A territory-wide, biopsy-proven, Chinese cohort. A retrospective, masked chart review of medical records, orbital images, and histopathology reports.

Results

A total of 122 (65 male) patients with a follow-up of 81 ± 49 (24 to 84) months were reviewed. Ninety (74%) patients presented bilaterally. Subacute upper eyelid swelling was the commonest presentation (82/122, 67%). During follow-up, 91/122 patients (75%) underwent extra-orbital imaging including computer tomography (692 films), ultrasonography (182 films), magnetic resonance imaging (76 films) and whole body FDG-PET scan (33 films). Eighty-six (95%) of these 91 patients had extra-orbital involvement radiologically (2.7 ± 1.6 regions, range: 0 to 9). Lymph node was the most prevalent (N = 60,66%), followed by salivary gland (N = 51,56%), lung (N = 49,54%), kidney (N = 22, 24%), hepatobiliary tree (N = 18, 20%) and pancreas (N = 17, 19%). Other organs include thyroid, aorta, meninges/brain and skin. Twenty-eight (23%) patients had allergic diseases (19 asthma, 16 allergic rhinitis, and 6 eczemas). Fifty-seven (48%) patients had paranasal sinusitis. Serum eosinophilia was associated with a higher number (3.24 versus 2.52, P = 0.0304) of organ involvement. Patients with deep organ involvement was associated with a higher age of IgG4-ROD onset (70 ± 12 versus 56 ± 13, P < 0.0001).

Conclusions

95% of the patients who underwent systemic imaging in our cohort had systemic organ involvement. An early physicians’ assessment and radiological imaging are recommended after the diagnosis of IgG4-ROD.

KEYWORDS:

• Immunoglobulin G4
• immunoglobulin G4-related ophthalmic disease
• systemic involvement

Disclosure statement

No potential conflict of interest was reported by the author(s).

Authors contribution

KL, MA, JS, KC designed the study. KL, FA, JS, CY collected recruited patients and recorded clinical data. KL wrote the first draft of the manuscript. KL, YW and RJ performed data analysis. All co-authors interpreted the data and reviewed the manuscript. All authors have read and approved the final manuscript.

Additional information

Funding

This study is supported in part by the General Research Fund, Research Grants Council, Hong Kong (Grant 14103221 to CPP).