Successful Treatment Strategies in Granulomatosis With Polyangiitis-Associated Peripheral Ulcerative Keratitis

Ebrahimiadib, Nazanin; Modjtahedi, Bobeck S.; Roohipoor, Ramak; Anesi, Stephen D; Foster, C. Stephen

doi:10.1097/ico.0000000000000919

Cited by 35 publications

(24 citation statements)

References 36 publications

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“…In this report, the patients had most classic manifestations of GPA and gradually developed to hypertension, and even cataract due to long-term use of steroid. Besides, PUK was also presented concurrently in one patient [17].…”

Section: Discussionmentioning

confidence: 92%

Accelerated corneal endothelial cell loss in two patients with granulomatosis with polyangiitis following phacoemulsification

Hsiao

Chen

et al. 2020

BMC Ophthalmol

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Background Generally, the loss rate of human endothelial cells (HCEC) in routine cataract surgery is 8.5%. When the corneal endothelial cells density (ECD) drops, the HCEC may decompensate to keep cornea dehydration which leads to corneal edema. Granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disease involving multiple organs including eyes such as conjunctivitis, scleritis, uveitis, and corneal ulcer. In this study, we report two cases of GPA whose corneal ECD decreased significantly after phacoemulsification cataract surgery. Case presentation In the first case of 69-year-old male with GPA, the ECD dropped 39.6% (OD) four months after phacoemulsification and 38.1% (OS) six months postoperatively respectively. At the final follow-up, the residual ECD was only 55% in the right eye in the 49th month, and 56% remained in the left eye in the 39th month. In the second case of 54-year old female, left ECD dropped 63.9% at the 4th month after surgery and 69.6% ECD remained at the 15th month postoperatively while similar ECD of right eye before and after left eye surgery. Conclusion Extensive preoperative ophthalmic evaluation and meticulous postoperative inflammation control should be applied to prevent severe loss of HCEC in GPA patients.

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Section: Discussionmentioning

confidence: 92%

Accelerated corneal endothelial cell loss in two patients with granulomatosis with polyangiitis following phacoemulsification

Hsiao

Chen

et al. 2020

BMC Ophthalmol

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“…In nonresponding or severe cases, steroid-sparing agents such as antimetabolites, T cell inhibitors, alkylating agents, and biologic agents are indicated. In our case, cyclophosphamide was chosen based on the successful results reported in the literature, which had similar outcomes [ 3 , 4 ].…”

Section: Discussionmentioning

confidence: 99%

Peripheral Ulcerative Keratitis in a Patient with Bilateral Scleritis: Medical and Surgical Management

Artieda

Estébanez-Corrales

Sánchez‐Pernaute

et al. 2020

Case Rep Ophthalmol

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Peripheral ulcerative keratitis (PUK) is a group of corneal disorders that cause peripheral corneal thinning, threatening globe integrity in advance stages. It is usually associated with systemic autoimmune diseases and management is based on local and systemic approaches. We present the case of a 47-year-old man with a previous history of bacterial keratitis in his left eye presenting with 1 month of bilateral ocular pain and redness. At examination, diffuse bilateral globe inflammation with paracentral corneal thinning in his left eye was observed. He was diagnosed with bilateral scleritis and PUK in his left eye. Workup for associated systemic autoimmune disease yielded negative results. The patient was started on pulses of intravenous methylprednisolone followed by oral prednisone failing to achieve sufficient control of the inflammatory syndrome. Subsequently, periodic intravenous cyclophosphamide was administered with a favorable response. A multilayer amniotic membrane graft was applied, but there was rapid melting with reabsorption of the tissue, resulting in extreme corneal thinning at the inferior paracentral cornea. A decentered 8.5-mm superficial anterior lamellar keratoplasty (SALK) was then performed obtaining the donor graft with a femtosecond laser but performing manual trepanation in the recipient. At 12 months, visual and biomicroscopic measures do not show deterioration and inflammation remains under control with oral azathioprine as maintenance regime. The management of PUK includes both systemic immunosuppression and tectonic procedures to preserve the globe integrity. Diverse surgical techniques have been attempted, but no definitive guidelines are available. Decentered large SALK is a simple technique that can yield acceptable visual results.

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“…We previously reported success with intravenous (i.v.) cyclophosphamide and rituximab in the treatment of GPA-associated PUK in a retrospective analysis [12]. However, the treatment outcomes of scleritis and uveitis have still to be fully explored.…”

Section: Introductionmentioning

confidence: 99%

Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis

Ahmed

Foster

2018

Ophthalmic Res

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Purpose: Vision-threatening ocular inflammation can be a devastating complication of granulomatosis with polyangiitis (GPA). Here we performed a retrospective observational study to describe the safety and efficacy of treating scleritis and uveitis with either cyclophosphamide or rituximab in GPA. Methods: A chart review of patients diagnosed with GPA-associated scleritis or uveitis, treated with either cyclophosphamide or rituximab as the final therapy at our clinic, was conducted. A total of 1 year of follow-up visits was required for inclusion in the study. Results: Thirteen patients (19 eyes) suffering from GPA-associated scleritis and/or uveitis were identified. As the final therapy, rituximab was administered to 9 patients and cyclophosphamide to 4. Mean duration of follow-up was 55 months (range 16–23 months). Remission was observed in all patients. Three patients had a flare of scleritis after the completion of therapy, and they were restarted on their respective agents. One patient had a flare of retinal vasculitis during rituximab therapy. One patient on cyclophosphamide experienced transient leukopenia. No adverse side effects of rituximab were noted throughout the course of treatment. Conclusions: Cyclophosphamide and rituximab are safe and effective agents for controlling scleritis and uveitis associated with GPA, with eventual progression towards steroid-sparing remission.

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Successful Treatment Strategies in Granulomatosis With Polyangiitis-Associated Peripheral Ulcerative Keratitis

Abstract: Rituximab achieved a high rate of disease control in PUK patients with GPA and is the preferred agent in halting disease progression.

Cited by 35 publications

References 36 publications

Accelerated corneal endothelial cell loss in two patients with granulomatosis with polyangiitis following phacoemulsification

Accelerated corneal endothelial cell loss in two patients with granulomatosis with polyangiitis following phacoemulsification

Peripheral Ulcerative Keratitis in a Patient with Bilateral Scleritis: Medical and Surgical Management

Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis

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