Ophthalmic involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 573 patients.

PURPOSE: To investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD). METHODS: A total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD. RESULTS: Male predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland. CONCLUSIONS: Lacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.

MR Imaging of Orbital Inflammatory Pseudotumors with Extraorbital Extension

OBJECTIVE: To demonstrate a variety of MR imaging findings of orbital inflammatory pseudotumors with extraorbital extension. MATERIALS AND METHODS: We retrospectively reviewed the MR features of five patients, who were diagnosed clinically and radiologically as having an orbital inflammatory pseudotumor with extraorbital extension. RESULTS: The types of orbital pseudotumors were a mass in the orbital apex (n = 3), diffuse form (n = 2), and myositis (n = 1). The extraorbital extension of the orbital pseudotumor passed through the superior orbital fissure in all cases, through the inferior orbital fissure in two cases, and through the optic canal in one case. The orbital lesions extended into the following areas: the cavernous sinus (n = 4), the middle cranial fossa (n = 4), Meckel's cave (n = 2), the petrous apex (n = 2), the clivus (n = 2), the pterygopalatine fossa and infratemporal fossa (n = 2), the foramen rotundum (n = 1), the paranasal sinus (n = 1), and the infraorbital foramen (n = 1). On MR imaging, the lesions appeared as an isosignal intensity with gray matter on the T1-weighted images, as a low signal intensity on the T2-weighted images and showed a marked enhancement on the post-gadolinium-diethylene triamine pentaacetic acid (post-Gd-DTPA) T1-sequences. The symptoms of all of the patients improved when they were given high doses of steroids. Three of the five patients experienced a recurrence. CONCLUSION: MR imaging is useful for demonstrating the presence of a variety of extraorbital extensions of orbital inflammatory pseudotumors.

Comparison of Orbital Pseudotumor and Lymphoma: Clinical and Radiological Findings

PURPOSE: To compare the clinical and radiological findings of orbital pseudotumor with those of orbital lymphoma. MATERIALS AND METHODS: The clinical and radiological features of 12 orbital pseudotumors were compared with those of 17 orbital lymphomas, the nature of all lesions being confirmed by tissue biopsy. Twenty-four CT scans and nine MR images were retrospectively reviewed and compared, with special focus on the location of a tumor in the orbit, the invasion of periorbital structures, tumor margin, bilaterality, and signal intensity. The initial symptoms at admission were also classified and compared. RESULTS: In 50% of cases, orbital pseudotumors were located in both extraconal and intraconal space; 75% involved two or more extraocular muscles, and 33% involved the optic nerve. Margins were either infiltrative (75%) or ill-defined (92%). As for orbital lymphomas, 64% occupied extraconal space, invading one or less extraocular muscle (76%) and conjunctiva (29%). Seventy-one percent had a sharp margin, and 65% were lobulated or round. In pseudotumors, orbital pain and visual loss were major symptoms, while in lymphomas a painless orbital mass was the initial symptom. CONCLUSION: CT or MR image analysis of lesion location, margin, and the involvement of adjacent extra-ocular muscle or optic nerves may help differentiate between orbital lymphoma and orbital pseudo tumor.

MR Imaging Findings of Orbitofacial Infarction Secondary to Rhinoorbital Mucormycosis: A Case Report

Rhino-orbital mucormycosis is the most frequently fatal fungal infection commonly occurring in patients with poorly controlled diabetes mellitus and and those who are immunocompromised, and requires prompt treatment. We describe a case of rhino-or-bital mucormycosis with orbital cellulitis and paranasal sinusitis, as seen on initial MR images, which on follow-up images had evol ved to orbitofacial infarction. MR imaging was useful for the demonstration of orbitofacial infarction, seen as areas of lack of enhancement and thus suggesting vascular invo l vement by mucor hyphae.