Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

Kubaisi, Buraa; Samra, Khawla Abu; Foster, C. Stephen

doi:10.5582/irdr.2016.01014

Cited by 108 publications

(82 citation statements)

References 85 publications

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“…The treatment of serious ocular manifestations of GPA, such as scleritis and uveitis, has focused on systemic therapy in addition to local therapy [11]. We previously reported success with intravenous (i.v.)…”

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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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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“…Orbital involvement occurs in 45% of cases of ocular and adnexal involvement and is characterized by orbital pain, proptosis, limited movements of extraocular muscles, erythematous edema of the eyelids [119], diplopia, and deterioration of vision [120]. The most common manifestation was proptosis [3,121] and was refractory to treatment in a Fauci et al study [37].…”

Section: Ophthalmological Involvementmentioning

confidence: 99%

Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature

Miłkowska-Dymanowska

Laskowska

Rzuczkowski

et al. 2019

SN Compr. Clin. Med.

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Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is a type of ANCA-associated vasculitis that affects small-to medium-sized vessels. GPA occurs with a prevalence of 24-152:1000000. The disease affects all races at every age. Various factors may have an impact on the etiology of GPA which is treated as an autoimmune disease. Genetic factors, infectious agents (like Staphylococcus aureus), environmental factors (like silica, hydrocarbons, fumes, pesticides, and farming) are considered elements for the development of the disease. Mostly, GPA affects the upper and lower respiratory tracts and kidneys and associated with otorhinolaryngological and renal manifestations. However, numerous untypical manifestations may also occur. Our review is aimed at discussing the most significant of them, including the neurological, cardiac, gastrointestinal tract, joints and muscles, skin, and ophthalmological manifestations. The whole literature was searched in PubMed. It has been used phrases granulomatosis with polyangiitis', Wegener's granulomatosis' and GPA'. The initial research for every sentence yielded subsequently, 4472, 5043, and 7110 results. Only studies with available full text were retrieved. After a threestage evaluation including a language evaluation, a heading evaluation, and an abstract evaluation, we obtained 139 relevant papers on which our review is based. GPA is a huge challenge for contemporary diagnostics and medicine. Our review is aimed at demonstrating the multiplicity of untypical manifestations and proving that every doctor may come into contact with a patient with GPA.

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“…They reach the cornea through the limbal vessels (89,90). PUK pathogenesis in WG is explained through pathological B and T lymphocytes, and possibly antineutrophil cytoplasmic antibodies (ANCA) (91). The antineutrophil cytoplasmic antibody test in cases of PUK coexisting with WG is a specific and sensitive indicator (92).…”

Section: A) Clinic and Pathogenesismentioning

confidence: 99%

“…Systemic immunosuppressive therapy should definitely be applied in WG patients. Other therapies provide a benefit only if they are given in addition to immunosuppressive treatment (91). Conjunctival excision combined with cryotherapy in addition to immunosuppressive treatment can be effective in these cases.…”

Section: B) Treatmentmentioning

confidence: 99%

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Clinical findings, pathogenesis and treatment in non-infectious peripheral ulcerative keratitis

Açıkalın¹

2018

Beyoglu Eye J

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Peripheral ulcerative keratitis (PUK) is a group of diseases that manifest with ulceration and/or thinning in the peripheral cornea. Although this group of diseases can occur as a result of infection, most cases are of immunological origin and are associated with autoimmune diseases (AID). The most common form of AID associated with PUK is rheumatoid arthritis (RA). However, PUK may also be seen with other AIDs, such as systemic lupus erythematosus and Wegener's granulomatosis. There are immunological differences between the peripheral and central cornea that may explain the localization of PUK in the peripheral cornea. For example, the proximity to limbal blood vessels and conjunctival lymphatics is different. Both humoral and cellular immunity play a role in the development of PUK. Although the number of CD8+ T cells did not vary significantly in RA patients, the number of CD4+ T cells was significantly greater. As PUK is very serious, early diagnosis and treatment are very important. Local and systemic corticosteroids, immunosuppressants, biological agents, bandage contact lenses, tissue adhesives, and in some cases, surgical treatment can be applied. Adjacent conjunctival excision, patch lamellar grafts, and keratoplasty are options in surgical treatment. It is very important not to forget the possibility of disease progression despite surgery, and the treatment of underlying disease is crucial. Keratoplasty in PUK cases may not be successful as a result of several factors, such as dry eye and the underlying immunological disease..

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Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

Cited by 108 publications

References 85 publications

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

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

Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature

Clinical findings, pathogenesis and treatment in non-infectious peripheral ulcerative keratitis

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