A 38-year-old Asian man with a history of sinusitis status presented at the Ophthalmology outpatient department following a surgical intervention at another hospital 3 months previously, with complaints of gradual painless blurred vision and proptosis for 6 months. There was no periocular sensory change or recent trauma. His family history was unremarkable.\nOn ophthalmic examination, best-corrected visual acuity (BCVA) was 20/30 on the right eye (OD) and 20/40 on the left eye (OS). Slit-lamp and funduscopic examination results were normal. The pupil reacted sluggishly without a relative afferent pupillary defect. Color vision was normal. Ocular motility was intact without diplopia, but exophthalmometry measurements were 21.5 mm with base 112 mm using one accurately calibrated Inami exophthalmometer (Hertel K-0161; Inami & Co., Ltd., Bunkyo-ku, Tokyo, Japan) on both eyes (OU). There was neither periocular sensory change nor palpated mass in the lower eyelid.\nThyroid function tests revealed normal free thyroxine and thyroid-stimulating hormone levels, but serological studies showed extremely high serum concentrations of IgG4 (2650 mg/dL; normal range < 135 mg/dL); elevated serum concentrations of IgG (2630 md/dL; normal range 751.0–1560.0 mg/dL); abnormal erythrocyte sedimentation rate (31 mm/h; normal range < 15 mm/1 h); and high levels of total IgE (1903.00 IU/dL; normal range < 87.00 IU/mL). The calculated serum IgG4/IgG ratio was 100% (normal range < 6%). No eosinophilia was found in the blood.\nPostcontrast T1-weighted fat-saturated magnetic resonance imaging (MRI) images showed a “doughnut” sign encircling the right optic nerve, and inferior sheath enhancement surrounding the left optic nerve was visible on the coronal MRI images (Fig. a). Bilateral “tram-track” signs were evident on the axial images (Fig. b). Bilateral enhancing lesions at the pterygoid plate, pterygomaxillary fissure, and sella region, with involvement of both maxillary nerves, were visualized using gadolinium-enhanced T1-weighted MRI (Fig. c, d). Bilateral infraorbital nerve involvement was also evident on the MR images (Fig. d, e). Enlargement of extraocular muscles, especially severe at the bilateral inferior rectus, right medial rectus, bilateral superior rectus, and bilateral lateral rectus muscles, was noted on coronal MRI images (Fig. e). Mucosal thickening in the ethmoid, maxillary, sphenoid, and frontal sinuses was also visible.\nWe referred this patient to the Department of Otolaryngology in our hospital for surgical biopsy. Biopsy with functional endoscopic sinus surgery was performed, and a tissue specimen measuring 0.5 × 0.3 × 0.2 cm was sent to the Department of Pathology for further testing. The result revealed chronic sinusitis with dense infiltrate of lymphocytes and plasma cells. Chronic inflamed granulation tissue, aggregates of foamy histiocytes, and fibrosis of sinonasal tissue were also noted (Fig. a–c). Organisms were not identified in the Periodic Acid-Schiff stain and the Acid-fact stain tests. Immunhistochemistry revealed CD138 accentuated plasma cells (Fig. d) and highlighted numerous IgG4-positive plasma cells (> 50 IgG4-positive plasma cells per high-power field), of which over 40% were IgG-positive plasma cells (Fig. e, f).\nThe patient underwent a treatment regimen comprising oral systemic corticosteroids (prednisolone 1 mg per kg body weight daily) for 2 weeks followed by slow tapering. Visual acuity improved to 20/25 (OU) 1 month after initiating treatment. Four months after the initial treatment, the vision got worse again, with the BCVA dropping to 20/30 (OD) and 20/50 (OS), respectively. Visual field test showed parafoveal scotoma (OD) and an enlarged blind spot (OS). Visual evoked potentials indicated bilateral delayed P100 latency (Fig. f). No active lesions were found after systemic screening. Based on the combined clinical, radiographic, and histopathologic findings, the final diagnosis was IgG4-RD with bilateral optic perineuritis, branches of trigeminal nerves involvement, and pansinusitis.\nThe patient underwent another course of oral prednisolone daily for 2 weeks with gradually tapering of dosage. This was followed by treatment with the immunosuppressive agent azathioprine (50 mg, twice daily). The final BCVA was 20/20 (OD) and 20/25 (OS) during the follow-up period of 2 years.
A 23-year-old Indian female presented to a tertiary institute of Northeast India with the chief complaints of dimness of vision, pain and redness in the right eye (OD) for 3 months. The patient was previously treated elsewhere with two posterior sub-Tenon injections of triamcinolone acetonide given 6 weeks apart. She was also on topical steroids and cycloplegic. She did not report any benefit from the previous treatment. There was no history of trauma to the eye or elsewhere. Other medical, surgical, and family histories were unremarkable.\nOn examination, ocular alignment was normal with a visual acuity of counting finger at 3 m in the OD. There was minimal sclerouveitis noted in the inferior-temporal quadrant of OD. On slit-lamp examination, OD showed 360° posterior synechiae with anterior chamber flare and cells (+++) with 1 mm hypopyon []. There was a suspected mass in the inferior-temporal quadrant behind the iris, pushing it anteriorly. The left eye examination was unremarkable.\nB-scan ultrasound (B-scan USG) of OD was suggestive of ciliary body mass with adjoining exudative retinal detachment (ERD). USG bio-microscopy findings were consistent with the findings of B-scan USG. The patient was advised with complete blood count with peripheral blood smear which was within normal limit. Syphilis screening (venereal disease research laboratory) was nonreactive with normal thyroid function test and negative Mantoux test (5TU) reading. Based on the clinical examination and baseline investigations, we came to the differential diagnosis of ciliary body melanoma or similar tumors. Magnetic resonance imaging suggested the possibility of ciliary body melanoma in OD [Fig. and ]. After obtaining informed consent, enucleation was carried out in OD, and eyeball specimen was sent to ocular pathology laboratory for histopathological analysis. In grossing, there was a grayish-white-colored tumor seen in the inferior-temporal part of the ciliary body measuring (10.99 × 7.98) mm. Adjoining the grayish-white ciliary body mass, there was an ERD noted [].\nMicroscopic description of enucleated eyeball showed peripheral corneal vascularization. Few inflammatory cells were seen in the anterior chamber []. Part of the iris tissue showed inflammation. An eosinophilic mass was seen in the ciliary body with diffuse plasmacytoid cells, with reactive lymphoid follicle and other inflammatory cells []. Episcleral and scleral tissue were inflamed and infiltrated by various inflammatory cells [Fig. and ]. Occasional vascularization of the sclera was noted []. Cut end of the optic nerve did not show any tumor involvement. Immunohistochemistry (IHC) was advised to explore the possibility of various inflammatory lesions. The steps for IHC were carried by kit methods available commercially.\nIHC for both Kappa and Lambda was positive. Cluster differentiation (CD20, CD3, and CD45) was focal-positive. CD138 was positive in the specimen and HMB45 was negative. IHC for IgG4 was positive in occasional cells which also expressed CD138 (plasma cells). All positive controls showed appropriate positive immunostaining. Negative control slide did not show immunostaining. IgG4-positive plasma cells were seen in high-power field in the documented slides. Nearly, 30% of all the CD138 positive plasma cells were found to be IgG4-positive [].\nProtein chemistry revealed hypergammaglobulinemia with higher levels of total protein and gamma fraction. No myeloma band or Bence Jones protein was detected []. Serum IgG4 levels were also elevated. A hematologist was consulted, and bone marrow examination revealed mild anemia, for which she was given supportive treatment. In the postoperative period, the patient was given a course of oral steroids. On follow-up at 6 weeks, she was given prosthesis in OD, and the patient was doing well till her last review.