Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature
Abstract Background Necrotizing scleritis is one of the most destructive ocular manifestations of underlying systemic diseases that can lead to a variety of severe complications, including globe perforation or vision loss. Necrotizing scleritis can occur in various conditions, such as systemic vascu...
Saved in:
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2025-04-01
|
| Series: | BMC Ophthalmology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12886-025-04027-6 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850184297697247232 |
|---|---|
| author | Mohammadreza Tahavvori Sahba Fekri Kiana Hassanpour Mohammad-Mehdi Sadoughi Mohammadali Javadi |
| author_facet | Mohammadreza Tahavvori Sahba Fekri Kiana Hassanpour Mohammad-Mehdi Sadoughi Mohammadali Javadi |
| author_sort | Mohammadreza Tahavvori |
| collection | DOAJ |
| description | Abstract Background Necrotizing scleritis is one of the most destructive ocular manifestations of underlying systemic diseases that can lead to a variety of severe complications, including globe perforation or vision loss. Necrotizing scleritis can occur in various conditions, such as systemic vasculitis like Anti-Neutrophil Cytoplasmic Antibody (ANCA) and ANCA-Associated vasculitis (AAV), systemic autoimmune disorders, infections, or as a result of surgical procedures. Case presentation In this case report, we present a patient with acute bilateral isolated necrotizing sclerokeratitis associated with positive c-ANCA without any manifestation of other organ involvement. A 52-year-old man with acute anterior bilateral necrotizing scleritis with diffuse areas of necrosis, thinning of the sclera, and the choroidal show was observed which was impending perforation on both sides. Systemic medications, including intravenous methylprednisolone and cyclophosphamide, and topical medications were administered. However, due to an incomplete response to these drugs, intravenous rituximab was initiated. Significant improvements in clinical manifestations were initiated after treatment with Rituximab. Conclusion Our case highlights the importance of systemic rituximab therapy in treating isolated ANCA-associated necrotizing scleritis when initial immunosuppressive treatments are not fully effective. |
| format | Article |
| id | doaj-art-0f706e03ea1946f0aadc300e3f3ff80c |
| institution | OA Journals |
| issn | 1471-2415 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Ophthalmology |
| spelling | doaj-art-0f706e03ea1946f0aadc300e3f3ff80c2025-08-20T02:17:05ZengBMCBMC Ophthalmology1471-24152025-04-012511510.1186/s12886-025-04027-6Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literatureMohammadreza Tahavvori0Sahba Fekri1Kiana Hassanpour2Mohammad-Mehdi Sadoughi3Mohammadali Javadi4Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical SciencesOphthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical SciencesOphthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical SciencesOphthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical SciencesOphthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical SciencesAbstract Background Necrotizing scleritis is one of the most destructive ocular manifestations of underlying systemic diseases that can lead to a variety of severe complications, including globe perforation or vision loss. Necrotizing scleritis can occur in various conditions, such as systemic vasculitis like Anti-Neutrophil Cytoplasmic Antibody (ANCA) and ANCA-Associated vasculitis (AAV), systemic autoimmune disorders, infections, or as a result of surgical procedures. Case presentation In this case report, we present a patient with acute bilateral isolated necrotizing sclerokeratitis associated with positive c-ANCA without any manifestation of other organ involvement. A 52-year-old man with acute anterior bilateral necrotizing scleritis with diffuse areas of necrosis, thinning of the sclera, and the choroidal show was observed which was impending perforation on both sides. Systemic medications, including intravenous methylprednisolone and cyclophosphamide, and topical medications were administered. However, due to an incomplete response to these drugs, intravenous rituximab was initiated. Significant improvements in clinical manifestations were initiated after treatment with Rituximab. Conclusion Our case highlights the importance of systemic rituximab therapy in treating isolated ANCA-associated necrotizing scleritis when initial immunosuppressive treatments are not fully effective.https://doi.org/10.1186/s12886-025-04027-6ScleritisAnti-Neutrophil cytoplasmic antibodyRituximab |
| spellingShingle | Mohammadreza Tahavvori Sahba Fekri Kiana Hassanpour Mohammad-Mehdi Sadoughi Mohammadali Javadi Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature BMC Ophthalmology Scleritis Anti-Neutrophil cytoplasmic antibody Rituximab |
| title | Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature |
| title_full | Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature |
| title_fullStr | Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature |
| title_full_unstemmed | Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature |
| title_short | Isolated ANCA-associated scleritis successfully treated with systemic rituximab; a case report and review of literature |
| title_sort | isolated anca associated scleritis successfully treated with systemic rituximab a case report and review of literature |
| topic | Scleritis Anti-Neutrophil cytoplasmic antibody Rituximab |
| url | https://doi.org/10.1186/s12886-025-04027-6 |
| work_keys_str_mv | AT mohammadrezatahavvori isolatedancaassociatedscleritissuccessfullytreatedwithsystemicrituximabacasereportandreviewofliterature AT sahbafekri isolatedancaassociatedscleritissuccessfullytreatedwithsystemicrituximabacasereportandreviewofliterature AT kianahassanpour isolatedancaassociatedscleritissuccessfullytreatedwithsystemicrituximabacasereportandreviewofliterature AT mohammadmehdisadoughi isolatedancaassociatedscleritissuccessfullytreatedwithsystemicrituximabacasereportandreviewofliterature AT mohammadalijavadi isolatedancaassociatedscleritissuccessfullytreatedwithsystemicrituximabacasereportandreviewofliterature |