The role of a conjunctival biopsy in the diagnosis of eosinophilic granulomatosis with polyangiitis with ocular involvement

Sonali Prasad; Anuj Mehta; Aman Gaur

doi:10.4103/ijo.IJO_1973_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 1 | Page : 161-163

The role of a conjunctival biopsy in the diagnosis of eosinophilic granulomatosis with polyangiitis with ocular involvement

Sonali Prasad, Anuj Mehta, Aman Gaur
Department of Ophthalmology, Vardhaman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Date of Submission	10-Aug-2022
Date of Acceptance	18-Oct-2022
Date of Web Publication	20-Jan-2023

Correspondence Address:
Sonali Prasad
3SBI Colony, Raja Bazar, Patna - 800 014, Bihar
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1973_22

Abstract

A 33-year-old Asian man presented with redness, swelling, and blurring of vision in both eyes for 1 month associated with headache and pain. The patient had a history of allergic rhinitis with a progressive hearing loss for 3 months, followed by bell's palsy. Given his elevated serum cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) and erythrocyte sedimentation rate (ESR) levels as well as computed tomography head, orbit, and peripheral nervous system that showed marked bilateral mastoiditis with opacification of the right middle ear canal and enlargement of the bilateral lacrimal gland, the suspicion of small vessel vasculitis with ocular and ear involvement was high. Because there was no significant lung or kidney involvement, a conjunctival biopsy was attempted, which revealed small capillaries surrounded by eosinophils and plasma cells. Because there are no validated diagnostic tests for eosinophilic granulomatosis with polyangiitis (EGPA), a conjunctival biopsy can be valuable and minimally invasive in the early diagnosis and treatment of small vessel vasculitis.

Keywords: Cytoplasmic anti-neutrophil cytoplasmic antibody, eosinophilic granulomatosis with polyangiitis, small vessel vasculitis

How to cite this article:
Prasad S, Mehta A, Gaur A. The role of a conjunctival biopsy in the diagnosis of eosinophilic granulomatosis with polyangiitis with ocular involvement. Indian J Ophthalmol Case Rep 2023;3:161-3

How to cite this URL:
Prasad S, Mehta A, Gaur A. The role of a conjunctival biopsy in the diagnosis of eosinophilic granulomatosis with polyangiitis with ocular involvement. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:161-3. Available from: https://www.ijoreports.in/text.asp?2023/3/1/161/368196

Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare auto-immune condition that causes disseminated necrotizing vasculitis of small- and medium-sized blood vessels with extravascular granulomas.^[1] The symptoms can vary from non-specific symptoms such as fatigue, fever, rhinitis, weight loss, and abdominal pain to respiratory, neurological, skin, gastro-intestinal, muscular, bone, or heart involvement.^[1],[2] Currently, there is no gold standard diagnostic test for EGPA, and the use of lung or muscle biopsy is quite frequently reported; however, the role of a conjunctival biopsy in the diagnosis of EGPA is a rare entity. We herein report a case of EGPA diagnosed by the biopsy of the inflamed conjunctiva.

Case Report

A 33-year-old Asian male presented with complaints of redness, pain, swelling, and diminution of vision in both eyes for 1 month associated with frontal headache. It was preceded by 3 months history of allergic rhinitis and progressive loss of hearing, followed by right-sided bell's palsy. He also complained of generalized weakness, fatigue, and anorexia since 2 months. The patient had received IV antibiotics from outside with oral prednisolone 50 mg with a tapering dose for 14 days. He also had a past history of asthma and seizure disorder since the age of 5. On examination, the visual acuity was 6/12 in the right eye and 6/9 in the left eye with normal intra-ocular pressure. Proptosis of 24 mm was noted in both eyes on Hertel's exophthalmometry with downward displacement of both the globes, edema, and lateral flaring, leading to S-shaped lid curvature [Figure 1]. A firm, non-tender, non-mobile mass was palpable in the bilateral supero-temporal orbit, which was not reducible and not adherent to the overlying skin. Pupils were round and normal in size and reaction with no afferent pupillary defect. Slit lamp examination showed diffuse conjunctival injection, chemosis with anterior chamber cells (1+), and dilated episcleral vessels with immune infiltrate in the peripheral cornea in both the eyes (right more than left) [Figure 2]a, [Figure 2]b. Dilated fundus examination of the patient was unremarkable. There was significant pre-auricular lymphadenopathy. His blood tests revealed a raised IgE with 190 IU/L, eosinophil count (1.67 × 10⁹/L) (16% times eosinophils), raised erythrocyte sedimentation rate (ESR) (64 mm at 1 hour), marked C-reactive protein (CRP) (63.4 mg/dl), and cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) levels (150 AU/ml) with trace proteinuria and hematuria on urine sedimentation analysis. Computed tomography (CT) head, orbit, and peripheral nervous system (PNS) showed marked bilateral mastoiditis with opacification of the right middle ear canal around the ossicles and symmetrical enlargement of the bilateral lacrimal gland along with mucosal thickening of bilateral frontal, anterior ethmoid, and maxillary sinuses [Figure 3]a, [Figure 3]b. A suspicion of ANCA-associated vasculitis (AAV) was made, and because no significant pulmonary or kidney involvement was noticed, a conjunctival biopsy was attempted. The conjunctiva adjacent to the superior limbus of the right eye was incised and sent for histopathology examination, which revealed small capillaries surrounded by eosinophils and plasma cells [Figure 4]. According to diagnostic criteria by the American Society of Rheumatology based on the history of allergic rhinitis, seventh nerve palsy (neuropathy), paranasal sinus involvement, and extravascular eosinophilia on biopsy, a diagnosis of EGPA was made. The patient was started on anti-inflammatory medication with oral steroids in tapering dose reducing 10 mg every 2 weeks, with topical steroids (prednisolone 1%) in tapering dose over 6 weeks along with a lubricant. The patient was referred to an ENT specialist and a rheumatologist to check for other systemic involvement. The patient was followed up weekly, and a repeat CBC with ESR and CRP was performed at the end of every tapering dose to monitor any signs of a flare-up.

Figure 1: Erythema and edema of both upper lids and lateral flaring leading to S-shaped lid curvature

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Figure 2: (a) Dilated episcleral vessels and immune infiltrate in the peripheral cornea were in the left eye. (b) Dilated episcleral vessels and immune infiltrate in the peripheral cornea were seen in the right eye

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Figure 3: (a) Enlargement of the bilateral lacrimal gland. (b) Mucosal thickening of bilateral frontal, anterior ethmoid, and maxillary sinuses

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Figure 4: Histopathology examination shows small capillaries surrounded by eosinophils and plasma cells

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The patient's condition started improving from the second week onward but developed a recurrence of bell's palsy grade III involving the lower motor neuron in the fourth week. There was also a mild flare-up of ocular redness and an inability to close the left eye. The dose of systemic steroid 50 mg and topical steroid QID was hence continued for another 1 week, followed by the usual tapering schedule. Intense lubrication was given to the left eye along with overnight patching with eye ointment to avoid exposure keratopathy.

Discussion

Eosinophilic granulomatosis with polyangiitis, previously known as Churg–Strauss syndrome (CSS) is a rare multi-systemic disease that manifests as necrotizing eosinophilic-rich granulomatous inflammation of medium/small-sized vessels often involving the respiratory tract and kidney.^[1],[2]

Because ANCA positivity has been reported in only a small proportion of EGPA (40%), they have been included in the spectrum of AAV along with microscopic polyangiitis and granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis).^[2]

As there is no gold standard test for EGPA, establishing a definitive diagnosis usually becomes challenging. Several criteria have been proposed for the diagnosis of auto-immune vasculitis including EGPA.^[3],[4],[5] American college of rheumatology (ACR) criteria (1990) is the most widely used set of guidelines that distinguishes EGPA from other causes of vasculitis based on six criteria – asthma, eosinophilia > 10%, neuropathy, mononeuropathy, or polyneuropathy, pulmonary infiltrates, paranasal sinus abnormalities, and extravascular eosinophils on biopsy.^[6] When four or more of these criteria are met, vasculitis can be classified as EGPA with a sensitivity of 85% and a specificity of 99.7%.

Our patient met three out of six criteria with asthma, paranasal sinus involvement, and eosinophilia > 10%. Hence, a biopsy was needed to support the diagnosis as per ACR criteria. Although the lung and kidney are commonly used sites for taking biopsy with a sensitivity of 90%, we however preferred conjunctival biopsy because of significant conjunctival inflammation and negligible pulmonary or renal involvement.

Few cases regarding the role of a conjunctival biopsy in GPA (Wegener's granulomatosis) have been reported, but there is no literature available on the role of a conjunctival biopsy in EGPA patients.^[7],[8],[9]

Karakousis et al.^[7] diagnosed a case of GPA in a 19-year-old boy with scleritis, refractory sinusitis, and elevated C-ANCA titer. Toh et al.^[8] diagnosed a case of GPA using conjunctival biopsy in a 61-year patient with proptosis, conjunctival ulceration, and central retinal artery occlusion. Jordan et al.^[9] confirmed two cases of GPA with conjunctival biopsy. Similarly, we also attempted a conjunctival biopsy in our patient which revealed eosinophilic predominant granulomatous necrosis, suggesting EGPA.

Conclusion

According to the European League Against Rheumatism (EULAR), a positive biopsy is strongly supportive of vasculitis. Conjunctival biopsy being relatively simpler and minimally invasive can play an important role in supporting the diagnosis of eosinophilic granulomatosis with polyangiitis.^[10] It can be considered as a possibility in patients that present with ocular involvement in EGPA and in which the biopsy from other organs such as the lung, kidney, and nasal passage is contraindicated. The conjunctival biopsy solely may not fulfill the classical triad, but it can play a supportive role when considered in combination with clinical and hematological findings.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

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