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Year : 2020  |  Volume : 8  |  Issue : 2  |  Page : 62-64

A case of peripheral ulcerative keratitis associated with limited granulomatosis polyangiitis

1 Department of Ophthalmology, Dr. Radhakrishnan Government Medical College and Hospital, Hamirpur, Himachal Pradesh, India
2 Department of Ophthalmology, Himachal Pradesh University, Shimla, Himachal Pradesh, India

Date of Submission16-Jul-2019
Date of Decision22-Oct-2019
Date of Acceptance30-Oct-2019
Date of Web Publication2-Jul-2020

Correspondence Address:
Anushree Gupta
Room Number 229, Eye OPD, Dr. Radhakrishnan Government Medical College and Hospital, Agriculture Colony, Hamirpur -177 001, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_54_19

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A 28-year-old Indian female presented with sudden onset reduced vision and pain in the left eye following blunt trauma. There was chronic history of redness, tearing, and irritation in both the eyes. She was diagnosed with peripheral ulcerative keratitis and corneal perforation in the left eye. Her corneal perforation was repaired with direct closure. Diagnostic workup revealed limited granulomatosis polyangiitis. The patient was treated with topical and systemic steroids and immunosuppressant drugs (rituximab).

Keywords: Granulomatosis with polyangiitis, immunosuppressive, peripheral ulcerative keratitis

How to cite this article:
Gupta A, Anand S. A case of peripheral ulcerative keratitis associated with limited granulomatosis polyangiitis. J Clin Ophthalmol Res 2020;8:62-4

How to cite this URL:
Gupta A, Anand S. A case of peripheral ulcerative keratitis associated with limited granulomatosis polyangiitis. J Clin Ophthalmol Res [serial online] 2020 [cited 2022 Jun 29];8:62-4. Available from: https://www.jcor.in/text.asp?2020/8/2/62/288848

Peripheral ulcerative keratitis (PUK) is characterized by progressive thinning of the peripheral cornea. PUK has a strong association with various autoimmune diseases and collagen vascular diseases are associated with over half of all PUK cases.[1] Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis with small-sized vessel involvement and granulomatous inflammation of respiratory tract and/or kidney that is associated with antineutrophil cytoplasmic autoantibody (ANCA) positivity. Ocular manifestations including orbital mass, scleritis, and PUK may occur in up to 30%–40% of GPA patients.[2] The therapeutic regimen of GPA includes systemic steroids, cyclophosphamide, and rituximab. This case report highlights the importance of ruling out any associated systemic disease, which can be potentially fatal without treatment.

  Case Report Top

A 28-year-old Indian female presented in the outpatient department at our ophthalmology outpatient department with sudden onset reduced vision and pain in the left eye for the past 1 day. She gave a history of mild blunt trauma to the left eye. She gave a history of redness, tearing, and irritation in both the eyes for the past 3 years. She had taken topical corticosteroid medications without much relief. Past medical history revealed epistaxis and recurrent nasal obstruction. Best-corrected visual acuity was 6/6 in the right eye and 6/60 in the left eye.

Slit-lamp examination of the left eye showed peripheral corneal thinning with infiltration within 2 mm of the limbus extending the entire circumference of cornea. A linear corneal perforation was extending from 10 to 12 o' clock position in the left eye. Minimal loss of tissue was seen.

Slit-lamp examination revealed corneal pannus with stromal infiltration superiorly in the right eye [Figure 1]. The adjacent conjunctiva was also inflamed and injected. Intraocular pressure was normal. The fundus examination was normal in both the eyes.
Figure 1: Peripheral corneal thinning with superficial vascularization and infiltration in the right eye

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Tissue scrapings for fungus and bacteria were negative. The patient was positive for anti-neutrophilic cytoplasmic antibody (p-ANCA) (titer of 1:20). Laboratory tests were negative for C-reactive protein (3.19 mg/l), rheumatoid factor, antinuclear antibodies, cytoplasmic- anti-neutrophilic cytoplasmic antibody, venereal disease research laboratory (VDRL) test, hepatitis B, hepatitis C, and human immunodeficiency virus. Mantoux skin test was negative (8 mm). Computed tomography para nasal sinuses revealed left deviated nasal septum with spur. Chest X-ray, complete blood count, renal function tests, and routine urinalysis were normal.

Her vitals were normal. Saddle nose deformity was seen [Figure 2]. The rest of systemic examination were normal.
Figure 2: Saddle.shaped nasal deformity

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Since there was minimal loss of tissue, corneal perforation was closed with interrupted 10–0 nylon sutures [Figure 3]. A bandage contact lens was applied. Sutures were opened after 3 weeks.
Figure 3: Peripheral corneal thinning with superficial vascularization and infiltration with sutured perforation in the left eye

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Since the patient had saddle nose, epistaxis, nasal crusting, positive p-ANCA, and PUK, a diagnosis of a limited GPA with PUK was made. Rheumatology consultation was sought, and she was started on oral steroids and intravenous rituximab. The patient was started on topical fluorometholone eye drops and preservative-free artificial teardrops. The conjunctival injection decreased and the PUK epithelialized, but corneal guttering persisted. She was counseled to use safety glasses in future.

  Discussion Top

PUK is a group of destructive inflammatory diseases involving the peripheral cornea, which is characterized by the sloughing of corneal epithelium and keratolysis.[3] It can progress to perforation resulting in significant vision loss and may be life-threatening when associated with a systemic autoimmune condition.

It is hypothesized that T-cells lead to antibody production and the formation of immune complexes that deposit in the peripheral cornea. The complement pathway is activated with the recruitment of inflammatory cells to the cornea. Collagenases and other proteases are secreted by neutrophils and macrophages, which leads to the destruction of the peripheral corneal stroma.[4]

PUK may be associated with various ocular and systemic infectious and noninfectious diseases (e.g., Mooren ulcer, marginal keratitis).[5]

Approximately 50% of all noninfectious PUK cases have an associated collagen vascular disease such as rheumatoid arthritis, GPA, polyarteritis nodosa, relapsing polychondritis, and systemic lupus erythematosus.[6]

Patients with PUK present with ocular irritation, pain, redness, photophobia, and corneal opacity.

Granulomatosis with polyangiitis (GPA) is a rare autoimmune multisystem disorder characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis. The classic organs involved in GPA are the upper respiratory tract, lungs, and kidneys.[2] The exact cause of GPA is not known.

Limited GPA is a form of the disease that only involves one or two organs, such as the respiratory tract. This disease usually occurs in females, in the younger age group and is less severe than the classic form.[7] The diagnosis in cases of limited GPA may be missed.

Ophthalmologic manifestations have been reported in 40%–50% of GPA patients in either the classic or limited form of the disease.[8]

Ophthalmologic involvement includes: an orbital inflammatory mass, often with proptosis and/or optic nerve compression, small-vessel vasculitis causing conjunctivitis, scleritis, episcleritis, PUK, uveitis, optic neuritis, optic nerve vasculitis, or retinitis.[9]

The ophthalmologic disease can be the initial clinical manifestation in the limited form of GPA.

The diagnosis of GPA is facilitated by the detection of ANCAs in blood, CT chest/X-Ray of the lungs and biopsy. The diagnosis of limited GPA may be missed as they lack classic systemic symptoms.

We used the Iran criteria for the diagnosis of GPA and a diagnosis of a limited GPA was made.[10] The patient had a good clinical response in her eye condition with systemic corticosteroids and rituximab.

Treatment of autoimmune disease patients with PUK includes nonsteroidal anti-inflammatory drugs, corticosteroids, systemic immunosuppressives, biologicals, and surgical therapy.

Patients with PUK associated with GPA do not respond to local corticosteroid therapy and require systemic immunosuppressive treatment, including systemic prednisolone, cyclophosphamide, and biologicals such as rituximab. Rituximab, is a chimeric anti-CD20 monoclonal antibody that has a depletion effect on the B-cell lineage.[11]

Surgical treatment is required to maintain the integrity of the globe. This includes use of cyanoacrylate glue for impending perforations, tectonic lamellar and penetrating keratoplasty, conjunctival flaps, and amniotic membrane grafts.[12]

  Conclusion Top

Our case highlights the importance of detailed systemic examination and investigations to rule out any systemic disease that can be potentially lethal. The diagnosis of a limited GPA is difficult and maybe missed. Patients with corneal or scleral thinning should be counseled to wear protective safety glasses as they are more prone to perforation even after minor trauma.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Yagci A. Update on peripheral ulcerative keratitis. Clin Ophthalmol 2012;6:747-54.  Back to cited text no. 1
Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of wegener's granulomatosis. Ophthalmology 1983;90:279-90.  Back to cited text no. 2
Cao Y, Zhang W, Wu J, Zhang H, Zhou H. Peripheral ulcerative keratitis associated with autoimmune disease: Pathogenesis and treatment. J Ophthalmol 2017;2017:7298026.  Back to cited text no. 3
Yanoff M, Duker J. Peripheral ulcerative keratitis. Ophthalmolgy. 5th ed. Philadelphia, United States: Elsevier; 2019.  Back to cited text no. 4
Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: Clinical features and treatment results. Am J Ophthalmol 2000;130:469-76.  Back to cited text no. 5
Recillas-Gispert C, Serna-Ojeda JC, Flores-Suárez LF. Rituximab in the treatment of refractory scleritis in patients with granulomatosis with polyangiitis (Wegener's). Graefes Arch Clin Exp Ophthalmol 2015;253:2279-84.  Back to cited text no. 6
Straatsma BR. Ocular manifestations of wegener's granulomatosis. Am J Ophthalmol 1957;44:789-99.  Back to cited text no. 7
Biswas J, Babu K, Gopal L, Krishnakumar S, Suresh S, Ramakrishnan S, et al. Ocular manifestations of wegener's granulomatosis. Analysis of nine cases. Indian J Ophthalmol 2003;51:217-23.  Back to cited text no. 8
[PUBMED]  [Full text]  
Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations. Intractable Rare Dis Res 2016;5:61-9.  Back to cited text no. 9
Salehi-Abari I, Khazaeli S, Khak M, Motesaddi Zarandy M, Hasibi M. Early diagnosis of granulomatosis with polyangiitis: An introduction to the newly designed Iran criteria. Indian Journal of Rheumatology 2013;8:107-11.  Back to cited text no. 10
Rodriguez-Garcia A. Rituximab for necrotizing scleritis and peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis. EC Ophthalmol 2016;01:12.  Back to cited text no. 11
Lu CW, Zhou DD, Wang J, Hao JL. Surgical treatment of peripheral ulcerative keratitis and necrotizing scleritis in granulomatosis with polyangiitis. Saudi Med J 2016;37:205-7.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]


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