A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease.

Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies.

Diagnosis and Treatment of Central Serous Chorioretinopathy in Patients with Scleritis.

Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from steroid use or an inflammation-related uveal effusion. We report the case of a 40-year-old man who presented to our department with intermittent redness and dull pain in both eyes that had persisted for three months. He was diagnosed with scleritis with SRF in both eyes and steroid therapy was started. Inflammation improved with steroid use, but SRF increased. This indicated that the fluid was not caused by the posterior scleritis-related uveal effusion but by steroid use. SRF and clinical symptoms subsided after steroids were discontinued completely and immunomodulatory therapy was initiated. Our study highlights that steroid-associated CSCR must be considered in the differential diagnosis of patients with scleritis, and prompt diagnosis with an immediate shift from steroids to immunomodulatory therapy can resolve SRF and clinical symptoms.

Prevalence of bacillary layer detachment in diabetic macular edema and response to 3 anti-vascular endothelial growth factor treatment.

Spectral-domain optical coherence tomography is widely used in maculopathy, including diabetic macular edema (DME). Bacillary layer detachment (BALAD) is a novel optical coherence tomography finding, defined as the separation of the intraretinal layer between the inner segment myoids and ellipsoids. A total of 161 treatment-naïve eyes with centrally involved DME that underwent 3 monthly loading doses of anti-vascular endothelial growth factor (anti-VEGF) intravitreal injections were enrolled and analyzed retrospectively. BALAD was found in 6.2% of eyes with concurrent subretinal fluid (SRF). All eyes were divided into 3 groups: no either group had neither SRF or BALAD; the SRF only group had SRF but no BALAD; and the BALAD group had both SRF and BALAD. A significant increase in baseline central foveal thickness (CFT) in the BALAD group was observed (no either vs SRF only vs BALAD, baseline CFT: 387.6 ±â€…74.29 vs 440.6 ±â€…106.79 vs 642.0 ±â€…188.86; P < .01). Total resolution of BALAD was noted after anti-VEGF therapy, along with a significant decrease in CFT in all groups (CFT decrease: 82.4 ±â€…87.07 vs 187.6 ±â€…138.88 vs 252.1 ±â€…127.63; P < .01). Eyes with BALAD tended to have the worst baseline visual acuity (baseline logarithm of the minimum angle of resolution VA: 0.76 ±â€…0.353 vs 0.63 ±â€…0.303 vs 1.15 ±â€…0.300; P = .046) but showed the most improvement after treatment (logarithm of the minimum angle of resolution VA change: -0.14 ±â€…0.235 vs -0.22 ±â€…0.275 vs -0.27 ±â€…0.250; P = .079). After resolution of BALAD, all eyes in the BALAD group exhibited ellipsoid zone and/or interdigitation zone disruption corresponding to the BALAD area. BALAD is a novel optical coherence tomography finding associated with a spectrum of diseases including DME. With anti-VEGF therapy, total resolution of BALAD and a significant decrease in CFT can be obtained. However, ellipsoid zone/interdigitation zone disruption tended to develop.