Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease consisting of necrotizing granulomatosis of the respiratory tract, necrotizing vasculitis, and necrotizing glomerulonephritis. It is under the category of ANCA-associated vasculitis, which involves small vessels. The nose, sinus, and ear were the most affected sites besides lung and kidney in localized form. They might precede other disease manifestations before progressing to the systemic form. Our patient presented with an intractable headache, followed by acute vision loss. His symptoms deteriorated regardless of antibiotic treatment for paranasal sinusitis. The sequential CT/MRI images showed the inflammatory raid of the orbital apex and cavernous sinus within days. The sinus biopsy and elevated PR3-anti-neutrophil cytoplasmic antibody led us to the diagnosis of GPA. Fortunately, the patient’s vision improved gradually after steroid and immunosuppressant treatment.
Apocrine hidrocystomas are benign cystic tumors resulting from apocrine sweat glands’ proliferation. They typically present as solitary, slow-growing nodules at the head and neck, especially in the periorbital cutaneous region. We present a case of periorbital apocrine hidrocystoma in a 22-year-old woman that was treated as chalazion previously. Besides the hallmark histopathological findings of apocrine hidrocystoma, IgG4 plasma cell infiltration of the cystic wall was also found. The ratio of IgG4-to-IgG-positive plasma cells was high, whereas serum IgG4 was within the standard limit. This is, to date, the only probable IgG4-related ophthalmic disease associated with apocrine hidrocystoma.
Direct chemical reprogramming provides a potentially scalable approach for generating retinal lineage-associated cells without genetic manipulation. In this study, human Tenon’s capsule fibroblasts were converted into retinal progenitor-like cells using a defined small-molecule cocktail. Retinal lineage-associated features were evaluated by immunofluorescence staining, quantitative reverse-transcription PCR, Western blot analysis, and bulk RNA sequencing, showing upregulation of neural and retinal markers, including VSX2, and transcriptomic remodeling consistent with transcriptional features associated with neuronal differentiation programs. Functional responsiveness was assessed by glutamate-evoked intracellular calcium imaging, revealing glutamate-responsive intracellular calcium dynamics in induced cells but not in parental fibroblasts. For in vivo assessment, induced cells were delivered via intravitreal transplantation in Wistar rats and subretinal transplantation in Long–Evans rats. One month after transplantation, structural and functional evaluations using optical coherence tomography, electroretinography, and histological analyses showed localized alterations in retinal structure at the subretinal injection site, while no significant differences were observed in scotopic ERG responses under the present experimental conditions. In contrast, fibroblast transplantation showed more prominent structural alterations under similar conditions. Human nuclei-positive signals were detectable in a subset of eyes, exhibiting focal and heterogeneous distribution within retinal regions at the one-month endpoint. Collectively, these suggest the induction of retinal lineage-associated molecular and functional features, with short-term functional tolerability observed in vivo under the present experimental conditions.