Progressive subretinal fibrosis with uveitis (PSFU) syndrome is a rare clinical entity presenting with multifocal granulomatous chorioretinitis and subsequently developing extensive subretinal fibrosis over weeks or months. Subretinal fibrosis affecting the macula may cause profound and permanent vision loss. We report the presentation and management of this condition in a child, to our knowledge, the youngest reported. The condition caused substantial visual loss and required high-dose systemic corticosteroid and immunosuppression with methotrexate and ciclosporin to control aggressive intraocular inflammation.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4612310PMC
http://dx.doi.org/10.1136/bcr-2015-212526DOI Listing

Publication Analysis

Top Keywords

subretinal fibrosis
16
progressive subretinal
8
multifocal chorioretinitis
4
chorioretinitis progressive
4
subretinal
4
fibrosis
4
fibrosis young
4
young child
4
child progressive
4
fibrosis uveitis
4

Similar Publications

Retinal pigment epithelial (RPE) cells undergoing epithelial‑mesenchymal transition (EMT) are a key factor in promoting the progression of subretinal fibrosis. The klotho protein and gene exert anti‑fibrotic effects in multiple fibrotic diseases. However, the mechanisms involved in the role of klotho are unclear in subretinal fibrosis.

View Article and Find Full Text PDF

Neovascular age-related macular degeneration (nAMD), characterized by choroidal neovascularization (CNV), is one of the leading causes of severe visual impairment and irreversible vision loss around the world. Subretinal fibrosis (SRF) contributes to the incomplete response to anti-vascular endothelial growth factor (VEGF) treatment and is one of the main reasons for long-term poor visual outcomes in nAMD. Reducing SRF is urgently needed in the anti-VEGF era.

View Article and Find Full Text PDF

Ribonucleoprotein (RNP)-based CRISPR/Cas9 genome editing holds great potential for the treatment of choroidal neovascularization (CNV), which however, is challenged by the lack of efficient cytosolic protein delivery tools. Herein, reversibly-phosphorylated pro-proteins (P-proteins) with conjugated adenosine triphosphate (ATP) tags are engineered and coupled with a membrane-penetrating, guanidine-enriched, α-helical polypeptide (GP) to mediate robust and universal cytosolic delivery. GP forms salt-stable nanocomplexes (NCs) with P-proteins via electrostatic interaction and salt bridging, and the helix-assisted, strong membrane activities of GP enabled efficient cellular internalization and endolysosomal escape of NCs.

View Article and Find Full Text PDF

Emerging clinical evidence of a dual role for Ang-2 and VEGF-A blockade with faricimab in retinal diseases.

Graefes Arch Clin Exp Ophthalmol

December 2024

Doheny Eye Institute, University of California, Los Angeles, 150 N. Orange Grove Blvd, Suite 232, Pasadena, CA, USA.

Anti-vascular endothelial growth factor (VEGF) therapies have transformed the treatment of retinal diseases. However, VEGF signaling is only one component of the complex, multifactorial pathophysiology of retinal diseases, and many patients have residual disease activity despite ongoing anti-VEGF treatment. The angiopoietin/tyrosine kinase with immunoglobulin and epidermal growth factor receptor-2 (Ang/Tie2) signaling pathway is critical to endothelial cell homeostasis, survival, integrity, and vascular stability.

View Article and Find Full Text PDF

Assessment of distributions and gender difference in adverse events related to faricimab: a real-world study based on FDA adverse event reporting system.

Expert Opin Drug Saf

December 2024

Geriatric Diseases Institute of Chengdu/Cancer Prevention and Treatment Institute of Chengdu, Department of Ophthalmology, Chengdu Fifth People's Hospital (The Second Clinical Medical College, Affiliated Fifth People's Hospital of Chengdu University of Traditional Chinese Medicine), Chengdu, China.

Background: Faricimab is a novel bispecific antibody drug for treating retinal disease. We aim to study the adverse events of faricimab based on the FDA Adverse Event Reporting System (FAERS) database.

Research Design And Methods: The FAERS data from 2020 to 2024 was extracted to conduct disproportionality analysis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!