Abstract:

Objective To investigate the clinical and imaging features of ocular lesions of IgG4-related disease(IgG4-RD) . Design Retrospective case series. Participants Twenty-five patients presented to ophthalmology department with IgG4-RD confirmed by pathology and／or laboratory examinations. Methods The clinical manifestations and imaging characteristics were analyzed. Main Outcome Measures Clinical manifestation, orbit magnetic resonance imaging (MRI), Somatostatin receptor scintigraphy (SRS) single-photon emission computed tomography (SPECT/CT). Results The main clinical manifestations were eyelids swelling and eyeballs protrusion in 25 cases with bilateral involvement happened in 23 cases, and 22 patients had 2 or more organs involved. Salivary gland (13 cases) and nose (19 cases) were most commonly involved organs besides eyes. Radiographic findings showed diffuse enlargement of lacrimal gland (22 cases), extraocular muscle hypertrophy (14 cases), infiltration of orbital soft tissue (10 cases). SRS indicated that the uptakes of tracer in orbits were high (UR=1.87±0.43) before treatment. After treatment by glucocorticoids and immunosuppressants, the uptakes in orbits reduced or disappear(UR=1.51±0.24). Conclusions Diffuse enlargement of bilateral lacrimal glands was the most common imaging finding of ocular lesions of IgG4-RD. Immune activity in orbits involvement can be objectively assessed with SRS, which is valuable for assessing clinical therapeutic effect.  (Ophthalmol CHN, 2015, 24: 309-312)

Key words: IgG4-related disease, orbit, magnetic resonance imaging, somatostatin receptor scintigraphy