Peripheral ulcerative keratitis in a patient with systemic lupus erythematosus: A rare but devastating association

Sujatha Mohan; Madhumita Prasad; Madhuvanthi Mohan; Sashwanthi Mohan

doi:10.4103/IJO.IJO_3241_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 285-287

Peripheral ulcerative keratitis in a patient with systemic lupus erythematosus: A rare but devastating association

Sujatha Mohan, Madhumita Prasad, Madhuvanthi Mohan, Sashwanthi Mohan
Department of Cornea, Rajan Eye Care Hospital, Chennai, Tamil Nadu, India

Date of Submission	12-Dec-2022
Date of Acceptance	08-Feb-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Sashwanthi Mohan
Rajan Eye Care Hospital, Chennai, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_3241_22

Abstract

Systemic lupus erythematosus (SLE) can have various ophthalmic manifestations, of which a rare but serious association is peripheral ulcerative keratitis (PUK), which can progress to corneal melt and perforation. Adequate immuno-suppression and close follow-up are required in these patients to prevent ocular and systemic progression of disease. We report a case of PUK in SLE which progressed rapidly despite immuno-suppression and required surgical intervention.

Keywords: Penetrating keratoplasty, peripheral ulcerative keratitis, PUK, SLE, systemic immuno-suppression, systemic lupus erythematosus

How to cite this article:
Mohan S, Prasad M, Mohan M, Mohan S. Peripheral ulcerative keratitis in a patient with systemic lupus erythematosus: A rare but devastating association. Indian J Ophthalmol Case Rep 2023;3:285-7

How to cite this URL:
Mohan S, Prasad M, Mohan M, Mohan S. Peripheral ulcerative keratitis in a patient with systemic lupus erythematosus: A rare but devastating association. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 10];3:285-7. Available from: https://www.ijoreports.in/text.asp?2023/3/2/285/375030

Systemic lupus erythematosus (SLE) is a multi-system, auto-immune disorder that affects various organs including the eye. Ocular manifestations occur in 1/3^rd patients and indicate that the disease is active.^[1] These include keratoconjunctivitis sicca, conjunctivitis, episcleritis, scleritis, uveitis, retinal vascular changes, choroidopathy, optic neuritis, and so on.^[1],[2] Corneal changes also occur.^[3] Peripheral ulcerative keratitis (PUK) is a rare but important ocular manifestation of SLE indicating advanced systemic disease and requires immediate systemic immuno-suppression and inflammation control.^[4] We report a case of SLE-associated PUK which progressed rapidly needing penetrating keratoplasty (PK) despite immuno-suppression.

Case Report

A 65-year-old male presented with complaints of watering in the left eye (OS) for 2 days. He was diagnosed with SLE with Sjogren's syndrome 10 years ago and was treated for dry eye disease earlier but was not on any medications at present. He had a history of uneventful cataract surgery with intra-ocular lens implantation in OU 11 years ago. On examination, the best corrected visual acuity (BCVA) in right eye (OD) was 6/6, N6, and in OS, it was 6/12, N8. Anterior segment OU showed meibomian gland disease and filamentary keratitis in OS. Schirmer's test gave values of 6 mm in OD and 4 mm in OS. Fundus OU was normal. He was started on artificial tear supplements 1% hourly, cyclo-immune 0.05% eyedrops twice/day, low-dose topical steroids, flurometholone 0.1% 4 times/day in weekly tapering dose, and warm compresses. He underwent a complete rheumatological evaluation, and the rheumatologist advised tablet mycophenolate mofetil 500 mg twice daily. A week later, he presented with redness and pain in OS for 1 day. A 4 × 3 mm epithelial defect along with peripheral corneal stromal melt from 2 to 3 o'clock hours was noted [Figure 1]a. Anterior chamber was quiet. Anterior segment optical coherence tomography (ASOCT) was performed to see the extent of thinning [Figure 1]b. The topical antibiotic moxifloxacin 0.5% 2 hourly was started, and topical steroids were continued. Two days later, the peripheral melt had progressed. Hence, cyanoacrylate glue application and bandage contact lens (BCL) in OS were provided [Figure 1]c. Post-operatively, topical steroids, dexamethasone 0.1% 4 times/day for 1 week with weekly tapering and oral steroids, tablet prednisolone 40 mg once daily in 10 days tapering dose, capsule omeprazole 20 mg twice daily, and calcium supplements were started. Two weeks later, he presented with severe pain and redness. Examination revealed severe conjunctival congestion and a second area of corneal melt between 7 and 8 o'clock hours, suggestive of recurrent inflammation. Glue was applied to this area, and BCL was replaced. The patient was given three doses of intravenous methylprednisolone (IVMP) 500 mg once daily for 3 days, followed by tablet prednisolone 50 mg once daily with weekly tapering. A month later, OS presented with a central corneal melt, impending perforation with iris incarceration, and multiple areas of thinning [Figure 2]a. ASOCT showed extreme thinning of cornea with iris incarceration temporally [Figure 2]b. Gentle ultrasound B-scan was normal. Because re-application of glue BCL was not effective, he was planned for a therapeutic PK to address the problem [Figure 2]c. Post-operatively, topical fortified cefotaxime 500 mg 2 hourly, tobramycin 0.3% 1 hourly, and dexamethasone 0.1% 6 times daily in tapering dose along with lubricants were prescribed. Systemic steroids and immuno-suppressants were continued. The patient is under the care of the rheumatologist with stable systemic and ocular conditions.

Figure 1: (a): Diffuse slit lamp image of the left eye showing diffuse, severe conjunctival congestion with peripheral corneal stromal melt from 2 to 3 o'clock hours. (b): ASOCT of the left eye showing the area of peripheral stromal melt and corneal thinning. (c): Diffuse slit lamp image of the left eye showing presence of cyanoacrylate glue at 2–3 o'clock hour

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Figure 2: (a): Diffuse slit lamp image of the left eye showing presence of previously applied glue at 7–8 o'clock hour and corneal melt centrally with impending perforation and iris incarceration. (b): ASOCT of the left eye showing the area of central corneal stromal melt and severe thinning with iris incarceration noted temporally and BCL in situ. (c): Diffuse slit lamp image of the left eye after penetrating keratoplasty with graft in situ and inferior layered hyphema

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Discussion

We report a case of PUK and corneal melt in an SLE patient. Despite initiation of systemic immuno-suppression, the ocular condition progressed leading to impending corneal perforation, which required surgical intervention. This throws light on the fact that PUK in SLE signifies advanced disease which requires long-term and aggressive immuno-suppression. The patient was also previously diagnosed to have Sjogren's syndrome, and the low Schirmer's also suggested the same. Secondary Sjogren's syndrome can also be responsible for sterile corneal melts, thus aggravating the corneal condition.^[5] PUK is a crescent-shaped juxtalimbal lesion of the corneal stroma associated with epithelial defect and corneal stromal thinning. Rapid progression causes necrosis of the corneal stroma and perforation.^[6] PUK is seen in collagen vascular disorders such as rheumatoid arthritis (RA), Wegener's granulomatosis, granulomatosis with polyangiitis, and SLE.^[7] It is either the first sign of these disorders or presents during advanced stages.^[5] Thus, a thorough rheumatologic examination should be performed in all PUK patients. PUK is more common in RA than SLE. Navas et al. reported an RA patient who had stopped anti-rheumatic medication and developed corneal melt. Immuno-suppressive therapy was immediately initiated, and a marked improvement in symptoms and signs and reduction in ulcer area was seen. This stresses the importance of systemic immuno-suppression to prevent systemic and ocular progression of such diseases.^[8] Sule et al. reported on four RA patients with PUK with corneal melt who were treated successfully with oral steroids and pulse cyclophosphamide. They stressed that oral steroids alone are not sufficient and additional immuno-suppressants are necessary to reduce inflammation.^[9] PUK is extremely rare in SLE. Chen et al. reported a case in which a 72-year-old woman with PUK and corneal perforation underwent rheumatological evaluation and was diagnosed with SLE. She was started on oral hydroxychloroquine and underwent PK to seal the corneal perforation.^[4] Mehta et al. reported a case of PUK and episcleritis in a 40-year-old lady 3 weeks post rituximab infusion. Ocular inflammation resolved with systemic immuno-suppression with immediate IVMP, followed by tapering oral steroids.^[10] However, in our patient, progression to corneal perforation occurred despite the systemic immuno-suppression, signifying the advanced systemic stage of the condition. Associated Sjogren's syndrome could have accelerated progression. The most serious complication of PUK is corneal perforation, resulting in vision loss. PUK can be managed with glue and BCL or may require surgical intervention in the form of amniotic membrane transplantation, conjunctival resection, patch grafts, or PKs depending on the disease stage.^[6] Importantly, adequate immuno-suppression is required to prevent progression of PUK. In our patient, surgery was required to preserve the globe integrity. Long-term immuno-suppression is required for stabilization of systemic condition.

Conclusion

Although rare, SLE can present with PUK and corneal melt. PUK in SLE usually signifies an advanced stage of systemic auto-immune condition and requires immediate and adequate immuno-suppression. Close follow-up is required because these cases can progress to corneal perforation despite systemic immuno-suppression.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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