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Case Report
 
Bilateral necrotizing scleritis isolated in patients with granulomatosis with polyangiitis and p-ANCA positivity
Anita Syla Lokaj1, Blerta Rama1, Ylfete Retkoceri1
1Eye Clinic, University Clinical Center of Kosovo, Pristina.

Article ID: Z01201608CR10688AL
doi:10.5348/ijcri-2016100-CR-10688

Address correspondence to:
Anita Syla Lokaj
Prishtina, Postal Code: 10000
Republic of Kosovo

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How to cite this article
Lokaj AS, Rama B, Retkoceri Y. Bilateral necrotizing scleritis isolated in patients with granulomatosis with polyangiitis and p-ANCA positivity. Int J Case Rep Images 2016;7(8):559–562.


Abstract
Introduction: Granulomatosis with polyangiitis (GPA) is a rare systemic disease of unknown etiology characterized with tissue granulomatous inflammation and vasculitis. The affected sites are the upper and lower respiratory system, skin, eyes, nervous system, kidney and rarely other organs. The disease exists in two forms; milder, regional located form (limited) and severe, progressive with renal involvement form (generalized).
Case Report: In this study, we represent a case of bilateral necrotizing isolated scleritis, where we had difficulties to diagnose GPA with only p-ANCA positive titrate and negative c-ANCA titrate. His c-ANCA was negative whereas p-ANCA (seen in 5% of cases) was positive. The scleral biopsy revealed histology suggestive of granulomatosis with polyangiitis. He responded well with steroid and cyclophosphamide therapy with great caution because he had diabetes mellitus.
Conclusion: In cases with few organ involvements or no active disease as showed in our case, where only scleritis is present and high suspicion of GPA clinically, a biopsy of organ with active disease is mandatory, as diagnosis cannot be made only on the presence of ANCA and signs of scleritis.

Keywords: Bilateral necrotizing scleritis, Granulomatosis, p-ANCA, Polyangiitis


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Author Contributions
Anita Syla Lokaj – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Blerta Rama – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Ylfete Retkoceri – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Anita Syla Lokaj et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



About The Authors

Anita Syla Lokaj is Ophthalmologist at University Center Clinic of Kosova. She is a represantitve of young ophthalmologist from Kosova at SOE group. Her field of interests are anterior segment disease.



Blerta Rama is resident in Ophthalmology in University Clinic Center of Kosovo in Pristina. She is an assistant in Occupational Medicine in Medical University of Pristina. She earned the degree Doctor of Medicine in Faculty of Medicine in University of Pristina. She intends to pursue fellowship in Ophthalmology after completing her residency.



Ylfete Retkoceri Eye Clinic, University Clinical Center of Kosovo, Pristina. She is Imonologist at University Center Clinic in Kosova.