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Year : 2022  |  Volume : 4  |  Issue : 1  |  Page : 36

Granulomatosis with polyangiitis patient with retinitis as an initial manifestation

Department of Ophthalmology of Penido Burnier Institute . Campinas, 1Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil

Date of Submission28-Jun-2022
Date of Acceptance12-Jul-2022
Date of Web Publication30-Jul-2022

Correspondence Address:
Valdez Melo Dos Anjos Filho
José Paulino Street, 159, Apartment 1007, Bosque, Campinas, São Paulo
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_33_22

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The German pathologist Friedrich Wegener described granulomatosis with polyangiitis (GPA), formerly known as Wegener's Granulomatosis, in 1936. Lesions are inflammatory, and typically include vasculitis, necrosis, and granulomatous changes. Ocular symptoms may be the first manifestation of this disease in 16%–58% of cases. The ocular manifestations of GPA occur in the most diverse ways. Delayed diagnosis can have serious consequences for patients' vision, as can the preservation of the globe, in addition to other systemic involvement, which can be rapidly progressive in some cases, leading to long-term disability or mortality.

Keywords: Granulomatosis with polyangiitis, retinitis, uveitis

How to cite this article:
Anjos Filho VM, Arruda MP, Nogueira Costa MA, Caso Rodriguez EE. Granulomatosis with polyangiitis patient with retinitis as an initial manifestation. Pan Am J Ophthalmol 2022;4:36

How to cite this URL:
Anjos Filho VM, Arruda MP, Nogueira Costa MA, Caso Rodriguez EE. Granulomatosis with polyangiitis patient with retinitis as an initial manifestation. Pan Am J Ophthalmol [serial online] 2022 [cited 2023 Jun 3];4:36. Available from: https://www.thepajo.org/text.asp?2022/4/1/36/353007

  Background Top

The German pathologist Friedrich Wegener described granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, in 1936. He reported three cases of patients with septic conditions, necrotizing granulomatous inflammation inside the nose, involvement of the larynx and pharynx, associated with glomerulonephritis, and periarteritis nodosa.[1],[2]

It is a necrotizing vasculitis and, along with microscopic polyangiitis and eosinophilic GPA (known as Churg–Strauss Syndrome) makes up the vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA).[2]

GPA is also associated with a cytoplasmic staining pattern directed against proteinase 3 and distinguished from other vasculitides by its predilection for the upper and lower respiratory tracts and, in most cases, the kidneys.[2],[3]

The course of the disease varies from indolent to progressive.[3],[4] Ocular symptoms may be the first manifestation of the disease in 16%–58% of cases, with the development of focal vasculitis in the anterior or posterior segments of the eye, with conjunctivitis, episcleritis, scleritis, peripheral ulcerative keratitis, and uveitis. In rare cases, optic nerve vasculitis or central retinal artery occlusion can occur.[5],[6]

In this article, we will present a case of retinitis as the initial manifestation of GPA.

  Case Report Top

A 51-year-old male patient presented to the clinic complaining of hyperemia and visual loss in the right eye for 1 week. On ophthalmologic examination, visual acuity in the right eye was 20/30p and in the left eye was 20/20. Biomicroscopy demonstrated mild conjunctival hyperemia and anterior chamber reaction. Fundoscopy demonstrated vascular thinning 1+/4+, serous retinal detachment with yellowish subretinal infiltrates in the superior temporal arcade and temporal periphery, not affecting the fovea [Figure 1] and [Figure 2].
Figure 1: Right eye fundus photograph (superior temporal)

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Figure 2: Right eye optical coherence tomography

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Considering posterior uveitis as a possible diagnosis, routine examinations were requested. Examinations for syphilis, human immunodeficiency virus, cytomegalovirus, herpes, tuberculosis, toxoplasmosis, rheumatoid factor, and antineutrophil cytoplasmic antibodies were all negative. The patient was seen by a rheumatologist and it was confirmed that PR3 antiproteinase antibody was reactive. The systemic investigation showed renal and respiratory changes, with pulmonary lesions on chest tomography. The diagnostic hypothesis was of GPA with “lung–kidney syndrome.”

Treatment with oral corticosteroids (40 mg/day) began and after 10 days, the patient developed necrotizing anterior scleritis in the same eye. Therefore, treatment was modified to cyclophosphamide 200 mg per day, after discussion with the rheumatologist. On the 20th day, the patient showed significant improvement in subretinal infiltrates and scleritis [Figure 3] and [Figure 4].
Figure 3: Right eye biomicroscopy

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Figure 4: Right eye fundus photograph (superior temporal) - after treatment

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  Discussion Top

The current incidence of GPA is approximately 2.4–11.3 cases per 1 million, predominantly in Caucasians and with no sex predilection.[7] The age at which symptoms appear varies but predominates between 41 and 68 years.[7]

Studies in North America, with 701 patients, reported an association of GPA with ocular involvement of 30%, whereas other reports reached results of up to 50%–58%.[6]

A retrospective study listed the complications of ocular involvement in GPA in prevalence: orbital (45%), scleral (38%), corneal (28%), lachrymal pathways (25%), and optic nerve (22%). Involvement of the optic nerve can occur, for example, through nerve ischemia due to occlusive vasculitis or granulomatous compression or inflammation due to contiguity of the nasal sinuses.[5]

Other forms of involvement were described, such as retinitis, chorioretinitis, macular edema, retinal necrosis, exudative retinal detachment, uveitis, central retinal artery or vein occlusion, and proptosis associated with orbital pseudotumor or orbital cellulitis.[8]

In our case, retinitis presented as the initial manifestation of ocular GPA and later developed scleritis. This differs from the literature since ocular scleritis is more common as an initial manifestation.

The manifestations of uveitis in patients with GPA are nonspecific, and can be of any form: anterior, intermediate, or posterior, associated or not with vitritis.[9]

Scleritis associated with Granulomatosis with polyangiitis

Its association with systemic diseases is approximately 40%–50% of patients, and the most frequently associated morbidity is rheumatoid arthritis. This association was described in 17%–33% of patients. The second most frequently associated disease is GPA.[6]

Scleritis associated with GPA is usually more severe than other diseases and may be nodular, diffuse, or necrotizing, and anterior or posterior.[7] In most cases, the pain radiates to the temporal region and jaw, worsening at night, and waking the patient. Redness and ocular sensitivity can also occur.[7]

Its necrotizing form can lead to significant functional ocular damage if not adequately treated with topical and systemic agents. Scleral thinning can cause perforation of the globe, and endophthalmitis, requiring enucleation.[7]

The definition of GPA according to the American College of Rheumatology is having two of the following four criteria: Sinus involvement; chest radiograph with evidence of nodules, fixed pulmonary infiltrate, or cavitations; urinary sediment with hematuria or erythrocyte casts; granulomas within an artery or in the area near an artery or arteriole. The sensitivity of these criteria is 88.2%, whereas their specificity is 92.0%.[2]

A definitive diagnosis requires histological confirmation of a granulomatous inflammatory process, vasculitis, and giant cells.[10]

In some patients, the combination of suggestive clinical signs associated with the presence of ANCA and/or anti-PR3 may be sufficient for diagnosis and treatment.[3]

In milder cases, such as episcleritis, conjunctivitis, and anterior uveitis, topical corticosteroids can be used.[7]

In cases of advanced disease, recent protocols suggest the induction of disease remission, associating high doses of systemic corticosteroids with cyclophosphamide or rituximab (immunobiological). The cyclophosphamide should be discontinued after remission, due to its toxic effects, and replaced by another drug, such as azathioprine, methotrexate, or immunobiological. If there is no remission, cyclophosphamide should be replaced by immunobiological and vice versa.[3],[8]

Corneal and scleral thinning can be severe enough to cause ocular perforation, requiring surgical interventions such as tarsorrhaphy, corneal transplants, conjunctival resections, or scleral grafts, despite systemic treatment.[8]

GPA is a rare disease and misdiagnosis can happen in patients with scleritis. Delay in diagnosis can have consequences for patients' vision, as well as ocular globe preservation, in addition to other systemic disorders, which can be rapidly progressive, leading to long-term disability or mortality.

Cases treated early, with immunosuppressive therapy and well-indicated corticosteroids, evolve with good visual acuity and improvement in the patients' quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Wegener FT. Uber generalisierte, septische gefasserkrankungen. Verh Dtsch Pathol Ges 1936;29:202-9.  Back to cited text no. 1
Comarmond C, Cacoub P. Granulomatosis with polyangiitis (wegener): Clinical aspects and treatment. Autoimmun Rev 2014;13:1121-5.  Back to cited text no. 2
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992;116:488-98.  Back to cited text no. 3
Rottem M, Fauci AS, Hallahan CW, Kerr GS, Lebovics R, Leavitt RY, et al. Wegener granulomatosis in children and adolescents: Clinical presentation and outcome. J Pediatr 1993;122:26-31.  Back to cited text no. 4
Suga K, Nomoto Y, Sudo A, Isogai J, Suzuki Y, Kagami SI. Granulomatosis with polyangiitis complicated with refractory optic neuritis and maxillary osteomyelitis. Mod Rheumatol Case Rep 2020;4:79-83.  Back to cited text no. 5
Cocho L, Gonzalez-Gonzalez LA, Molina-Prat N, Doctor P, Sainz-de-la-Maza M, Foster CS. Scleritis in patients with granulomatosis with polyangiitis (wegener). Br J Ophthalmol 2016;100:1062-5.  Back to cited text no. 6
Sfiniadaki E, Tsiara I, Theodossiadis P, Chatziralli I. Ocular manifestations of granulomatosis with polyangiitis: A review of the literature. Ophthalmol Ther 2019;8:227-34.  Back to cited text no. 7
Gheita TA, Abd El Latif EM. Relationship of ocular presentation in granulomatosis with polyangiitis to autoantibodies and disease activity. Z Rheumatol 2019;78:281-6.  Back to cited text no. 8
Sorin SM, Răzvan-Marian M, Daniela MM, Dan-Alexandru T. Therapy of ocular complications in ANCA+associated vasculitis. Rom J Ophthalmol 2021;65:10-4.  Back to cited text no. 9
Masuda T, Izumi Y, Takeshita H, Kawahara C, Tsuji Y, Kurohama H, et al. Granulomatosis with polyangiitis presenting as a choroidal tumor. Case Rep Rheumatol 2015;2015:271823.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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