Churg–Strauss syndrome, also known as allergic granulomatosis, was first described by Churg and Strauss in 1951.1 It is a systemic allergic disease characterised by eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small to medium-sized vessels. The skin, heart, and gastrointestinal tract are occasionally involved, but ocular involvement is unusual. We present the case of a 54-year-old woman who presented with an aggressive unilateral proliferative retinopathy and was subsequently diagnosed as having Churg–Strauss syndrome.

Case report

A 54-year-old female presented to the Ophthalmology department with a 2-week history of hazy vision in her right eye. There was no past ocular history of note. She had a past medical history of hypothyroidism and asthma. Regular medications included thyroxine and salbutamol and becotide inhalers. She smoked 20 cigarettes per day.

On examination visual acuities were 6/24 in the right eye and 6/5 in the left eye. There was a moderate vitreous haemorrhage in the right eye. The left vitreous was clear. There were poor views of the right fundus, but no tears, holes or detachments were seen. The left fundus was healthy. Blood pressure and blood sugar were normal.

The patient was followed up weekly at the retinal out patient clinic. At 3 weeks following her initial attendance the view of the fundus in the right eye had resolved sufficiently to reveal superotemporal new vessels. Fluorescein angiography confirmed the presence of these new vessels (Figure 1) and demonstrated widespread capillary closure (Figure 2).

Figure 1
figure 1

Fluorescein angiography showing superotemporal neovascularisation.

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Figure 2
figure 2

Fluorescein angiography showing inferonasal capillary closure.

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The patient subsequently underwent right panretinal photocoagulation. Initially, the new vessels appeared to regress. However, 4 months following panretinal photocoagulation she experienced a further vitreous haemorrhage in the right eye. On this occasion the vitreous haemorrhage failed to clear, and therefore 2 months later right vitrectomy combined with endolaser was performed.

The patient was followed up every 2 months in the retinal out patient clinic. At 1 year following her initial presentation, the patient attended clinic complaining of significant systemic symptoms. For the preceding 2 months she had been experiencing dizzy spells and headaches behind her right eye. She complained of lethargy, poor appetite, and had lost a stone in weight. She had noticed progressive numbness in her hands and feet and had developed a nasal discharge. She also described symptoms consistent with episodes of right amaurosis fugax.

On examination she had a mild vitreous haemorrhage in the right eye and had developed further active neovascularisation superotemporally in the right fundus. Left fundus remained healthy. She was noted to have a livedo reticularis rash on her knees and upper thighs. Neurological examination revealed a bilateral glove and stocking peripheral sensory neuropathy.

In view of a suspected vasculitic process, the patient was referred to the rheumatology department. Full blood count revealed a haemoglobin of 104 g/l (normal range 115–165 g/l). Mean cell volume was 72.6 fl (normal range 78–98 fl). There was a hypochromic microcytic blood picture. Eosinophils were elevated at 1.0 × 109/l (normal range 0.0–0.4 × 109/l). ESR and CRP were both normal. Serum ACE was normal. ANCA and ANA were both negative. Chest X-ray did not reveal any focal abnormality. On the basis of a history of asthma, hypereosinophilia, peripheral neuropathy, and paranasal sinusitis, a diagnosis of Churg–Strauss syndrome was made.

The patient was initially treated by the rheumatologists with six cycles of chemotherapy comprising intravenous cyclophosphamide (15 mg/kg) and methylprednisolone (10 mg/kg). She was then commenced on oral methotrexate. Further right vitreous haemorrhage and superotemporal neovascularisation was treated with a repeat vitrectomy and endolaser. At her most recent review 4 years following initial presentation visual acuities were 6/9 right eye and 6/5 left eye and there was no evidence of any active neovascularisation in either eye.

Comment

Churg–Strauss syndrome is a rare disease with an estimated annual frequency of 2.4–6.8 per million patient-years.2 Churg and Strauss reported the cases of 13 patients with severe bronchial asthma and disseminated necrotising vasculitis.1 These patients were found to have fever, eosinophilia, and multisystem involvement, which was associated with a histologic pattern of necrotising arteritis, eosinophilic tissue infiltration, and extravascular granulomata. They entitled the disorder allergic granulomatosis (Churg–Strauss syndrome). To establish a diagnosis of Churg–Strauss syndrome, four of six criteria must be met (the presence of asthma, hypereosinophilia (>10%), mononeuropathy, or polyneuropathy, nonfixed pulmonary infiltrates, paranasal sinus abnormality, and extravascular eosinophil infiltration in biopsy specimens).3

Ocular involvement in the Churg–Strauss syndrome is rare. The reported ophthalmologic manifestations include corneal ulcer, uveoscleritis, conjunctival granuloma, orbital inflammatory pseudotumour, amaurosis fugax, retinal artery occlusion, ischaemic optic neuropathy, oculomotor nerve palsy, and trochlear nerve palsy.4 Takanashi et al4 classified these ocular manifestations into two types: orbital inflammatory pseudotumour and ischaemic vasculitis. They hypothesised that these two groups may represent the two essential characteristics of the disease processes: granulomatosis and angiitis.

Serum ANCA is positive in 70% of patients with Churg–Strauss syndrome.5 In the review of ophthalmic cases by Takanashi et al the presence of ANCA was characterisitic of the ischaemic type. They advised that this may be a risk factor for sudden visual loss and that prophylactic steroids may be advisable in these cases.

Our case was of the ischaemic type but was negative for ANCA. The proliferative retinopathy in this patient was particularly aggressive and had the potential for marked visual loss. However, with appropriate surgical and medical management she has had a relatively good outcome. To our knowledge this is the first reported case of Churg–Strauss syndrome presenting with a proliferative retinopathy.