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Wegener's granulomatosis (WG) is a systemic granulomatous inflammatory disease of unknown origin. It occurs at any age, with the peak incidence in the third and fourth decades. The classic diagnostic triad of WG is necrotising granuloma of the upper or lower respiratory tract, vasculitis, and nephritis. Identifying a raised ANCA titre is virtually diagnostic of the disease, especially with the classic granular cytoplasmic staining pattern of neutrophils (C-ANCA).1 This disease is usually fatal if untreated; however, early diagnosis and instigation of immunosuppressants such as cyclophosphamide can induce long term remission.2
A 79 year old woman presented with a 2 week history of a painful, red left eye. She had mild arthritis and was also noted to be profoundly deaf, requiring hand written notes for communication. There was no history of sinus problems.
Her best corrected visual acuities were 6/18 in the right eye and 6/12 in the left eye. The left peripheral supratemporal cornea showed three areas of 80% corneal thinning, 1 × 1 mm in size, with an overlying epithelial defect (Fig 1). There was adjacent conjunctival injection and moderate anterior chamber cellular activity. The other eye was normal with no signs of inflammation. Examination of her hands showed no obvious rheumatoid abnormalities. Initial investigations performed were full blood count, electrolytes, C reactive protein, erythrocyte sedimentation rate (ESR), and rheumatoid factor. Corneal scraping and cultures were also undertaken to exclude an infective cause. The only significant test result was a raised ESR of 94, with other tests including creatinine being within the normal range. A provisional diagnosis was made of rheumatoid corneal melt and treatment was commenced with topical dexamethasone 0.1% non-preserved hourly and oral prednisolone at 40 mg/day.
After 5 days of steroid treatment, the corneal thinning was resolving and the conjunctival injection settling (Fig 2). Also by the fifth day, the patient's hearing had dramatically improved. She could now conduct a conversation at normal volume. On further questioning, she said her hearing had deteriorated only over the past few months and had ascribed it to “natural” old age deterioration. This suggested a link between her corneal changes and hearing loss and she was investigated for Wegener's granulomatosis (WG). Her serum antineutrophil cytoplasmic antibodies (cANCA) titre was found to be raised (>160). Rheumatoid factor was negative.
Oral cyclophosphamide was commenced on diagnosis of WG and the oral prednisolone slowly tapered. Her ocular inflammation, hearing, and arthritis continued to improve on treatment and at the 2 month follow up, there was minimal peripheral corneal thinning and no inflammation. Best corrected visual acuity in the left eye remained at 6/12. Examination by an otolaryngologist found mild hearing impairment and no significant sinus pathology. Rheumatological review found only mild arthropathy.
The majority of the ocular involvement in WG is caused by focal vasculitis, involving the anterior and/or posterior segment of the eye and corneal melting has been reported in 11–16% of patients with WG.3,4 Profound sensorineural deafness is a less common feature, occurring in about 6% of patients on presentation.2,5 It occurs either secondary to inflammation of the cochlear vessels or serous otitis media.4 Other important systemic features include pyrexia, weight loss, recurrent epistaxis, sinus discharge, haemoptysis, peripheral neuropathy, cerebral vasculitis, and renal failure (major cause of death). Fortunately in this case, the patient had the limited form of WG, with no renal involvement.
This patient presented with an uncommon combination of corneal melt and profound deafness. As deafness is a relatively common problem among the elderly patient population6 it may be overlooked as an important symptom, especially if ocular pathology is the main feature at presentation. It was not until the patient's hearing improved on treatment with oral prednisolone that its significance became apparent. WG is a rare condition and early recognition and treatment are essential as the disease carries a significant risk of serious complications. A full systemic history in patients with corneal melt is important in formulating the correct diagnosis and thereby expediting the appropriate treatment.