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Churg-Strauss syndrome (CSS) is an eosinophilic granulomatous vasculitic condition associated with asthma or allergic rhinitis, or both.1 Established management of severe disease includes high dose corticosteroids and intravenous cyclophophamide therapy.2 We present a patient with CSS who was resistant to treatment with pulse intravenous cyclophosphamide but responded to cyclosporin therapy.
A 31 year old white woman was admitted to hospital with a 10 day history of pain and numbness in the left foot, an erythematous rash on her chest and lower legs, generalised myalgia, polyarthralgia, and fevers. She had a eight year history of asthma that had deteriorated over the previous 12 months. Examination revealed a vasculitic rash on the chest and lower legs and a peripheral sensory neuropathy affecting the left foot. She was found to have an increased erythrocyte sedimentation rate of 58 mm 1st h, C reactive protein 48 mg/l (normal up to 10 mg/l), eosinophilia 10.70 × 109/l and echocardiography revealed a small pericardial effusion. The diagnosis of CSS was made and she was given prednisolone 40 mg daily with partial symptomatic improvement and lowering of the eosinophil count. The prednisolone was slowly reduced but on reaching 15 mg/0 mg alternate days the neuropathy worsened, the rash returned, and the eosinophil count rose. Azathioprine was added but despite 200 mg daily, giving a neutrophil count of 3.74 × 109/l, the neuropathy, fever, rash and eosinophilia recurred each time the prednisolone dose was reduced below 15 mg/0 mg alternate days. After more than 12 months from initial diagnosis she was referred to our unit for further treatment.
On initial presentation to our unit, she was grossly cushingoid (weight 80 kg) and still complained of numbness in her foot, fatigue, and myalgia. She was started on weekly cyclophosphamide infusions (1 g), the first three infusions with methylprednisolone. Despite weekly cyclophosphamide infusions the neutrophil count remained above 3.5 × 109/l so we were able to increase the cyclophosphamide dose to 1.25 g weekly and then 1.5 g weekly, which brought the pre-treatment neutrophil count down to 1.9 × 109/l. However, all attempts to reduce the prednisolone dose to 5 mg daily precipitated a flare of disease with recurrent rash, fatigue, and neuropathy. After 12 months on pulse cyclophosphamide (total dose 44.75 g) she was still requiring oral prednisolone to control the rash, fatigue, neuropathy, and eosinophilia and her asthma had again deteriorated. The cyclophosphamide was therefore stopped and she was immediately given cyclosporin (Neoral, Sandoz) 150 mg 12 hourly (3.5 mg/kg per day) giving trough blood values of 117 ng/ml. At the time of writing, she has been receiving this treatment for five months, has been asymptomatic from CSS and her asthma since starting the cyclosporin and managed to reduce and stop oral prednisolone without recurrence of symptoms for the first time in 2.5 years. The only side effects experienced are the “burning” sensation in her hands and feet and intermittent mild nausea.
To our knowledge, this is the first report of severe CSS being treated with cyclosporin. Despite 44.75 g cyclophosphamide in 12 months our patient’s disease flared each time the oral prednisolone dose was reduced. Clearly, in this patient corticosteroids and pulse cyclophosphamide therapy failed to control the disease although these remain the drugs of choice in severe CSS. Plasma exchange and interferon γ have been used separately in resistant cases of CSS but the role of these treatments are still being evaluated.2 3 There are a few reports of cyclosporin used in Wegener’s granulomatosis with response in doses 5–10 mg/kg per day.3 Promising results have recently been seen with cyclosporin in the treatment of corticosteroid resistant asthma although larger controlled studies are still awaited.4Troublesome residual asthma in CSS may necessitate continuing oral prednisolone even when the vasculitic disease has abated.5Cyclosporin therefore, may be a useful treatment in cases of resistant CSS controlling both the vasculitic process and the residual asthma.
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