Diffuse Granulomatous Conjunctivitis as an Ocular Manifestation of ANCA-Negative Limited Wegener’s Granulomatosis: A Case-Based Literature Review
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Wegener’s Granulomatosis, ANCA-negative, granulomatous conjunctivitis
Abstract
Introduction: Necrotizing granulomatous vasculitis, which is commonly known as Wegener's Granulomatosis (WG), frequently affects small to medium-sized blood vessels and is associated with anti-neutrophil cytoplasmic antibodies (ANCA). Despite ocular manifestations being prevalent in the disease, initial symptoms involving the eyelid and conjunctiva are infrequent. This study reports a case of an 11-year-old girl diagnosed with localized WG in the conjunctiva, with negative ANCA-test, and biopsy strongly suggested WG.
Case Report: An 11-year-old girl presented a red membrane covering the entire ocular surface and diminished vision in the left eye seven months before admission. At presentation, hand motion in the left eye was the best corrected visual acuity. There were granuloma formations in the palpebral and bulbar conjunctival, covering the entire ocular surface in the left eye. An incisional biopsy was performed in the conjunctiva, which revealed an ulcerative mucous membrane, prominent vasculitis, and necrotizing granulomas with giant cells and massive leukocyte infiltrate consistent with WG diagnosis.
Discussion: There is a need to consider the clinical manifestations suggesting the presence of vasculitis, ANCA determination, and histopathological evidence of the compromised organ to confirm the diagnosis. Overall, 82-94% of patients with WG were ANCA positive, leaving approximately 10% who tested negative, particularly those with limited WG. Moreover, a biopsy can confirm the diagnosis, specifically in ANCA-negative cases.
Conclusion: This case illustrated the consideration for WG diagnosis in limited form and negative ANCA-test. The clinical suspicion of WG and alternative diagnostic criteria using tissue biopsy might be helpful in such cases.
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References
Bohm M, Gonzalez Fernandez MI, Ozen S, Pistorio A, Dolezalova P, Brogan P, et al. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatric Rheumatology. 2014;12(1):18.
Cabral DA, Morishita K. Granulomatosis with Polyangiitis in Children Pediatric Rheumatology: A Clinical Viewpoint. Singapore: Springer; 2017. p. 461-73.
Laxer RM, Sherry DD, Hashkes PJ. Vasculitis. Pediatric Rheumatology in Clinical Practice: 2nd Edition. London: Springer; 2016. p. 159-65.
Nejabat M, Mahmoudinezhad G, Shenavandeh S, Ashraf M, Jalalpour M. Conjunctivitis as a manifestation of Wegener's Granulomatosis. Journal of Current Ophthalmology. 2017;30.
Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13(11):1121-5.
Rogaczewska M, Puszczewicz M, Stopa M. Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis – a case report. BMC Ophthalmology. 2019;19(1):139.
Robinson M, Lee S, Sneller M, Lerner R, Langford C, Talar-Williams C, et al. Tarsal-conjunctival disease associated with Wegener's granulomatosis. Ophthalmology. 2003;110:1770-80.
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.
Nejabat M, Mahmoudi Nezhad GS, Shenavandeh S, Ashraf MJ, Jalalpour MH. Conjunctivitis as a manifestation of Wegener’s Granulomatosis. J Curr Ophthalmol [Internet]. 2018;30(3):268–72. Available from: https://doi.org/10.1016/j.joco.2017.11.003
Robinson MR, Lee SS, Sneller MC, Lerner R, Langford CA, Talar-Williams C, et al. Tarsal-conjunctival disease associated with Wegener’s granulomatosis. Ophthalmology. 2003;110(9):1770–80.
Miserocchi E, Waheed NK, Baltatzis S, Foster CS. Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal Wegener granulomatosis. Am J Ophthalmol. 2001;132(6):923–4.
Jordan D, Zafar A, Brownstein S. Cicatricial Conjunctival Inflammation With Trichiasis as the Presenting Feature of Wegener Granulomatosis. Ophthal Plast Reconstr Surg. 2006;22(1):52–3.
Toh T, Cooper RL, Parker A, Vote BJ. Wegener’s granulomatosis presenting with painless bulbar-conjunctival ulcer and central retinal artery occlusion. Clin Exp Ophthalmol. 2006;34(1):51–3.
Eisenberger U, Fakhouri F, Vanhille P, Beaufils H, Mahr A, Guillevin L, et al. ANCA-negative pauci-immune renal vasculitis: histology and outcome. Nephrology Dialysis Transplantation. 2005;20(7):1392-9
Kemna MJ, Damoiseaux J, Austen J, Winkens B, Peters J, van Paassen P, et al. ANCA as a Predictor of Relapse: Useful in Patients with Renal Involvement But Not in Patients with Nonrenal Disease. Journal of the American Society of Nephrology. 2015;26(3):537-42.
Ursea R, De Castro D, Bowen TJ, Chan CC. The role of conjunctival biopsy in the diagnosis of granulomatosis with polyangiitis. J Ophthalmic Inflamm Infect. 2015;5(1):1.
Isa H, Lightman S, Luthert PJ, Rose GE, Verity DH, Taylor SR. Histopathological features predictive of a clinical diagnosis of ophthalmic granulomatosis with polyangiitis (GPA). Int J Clin Exp Pathol. 2012;5(7):684-9.
Guevara D, Cerda F, Carreño M, Piottante A, Bitar P. Actualización en el estudio de Granulomatosis con poliangeitis (Granulomatosis de Wegener). Revista chilena de radiología. 2019;25:26-34.
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