A 25 year old woman, 23 weeks into a twin pregnancy, was admitted to the labour ward with hypertension and proteinuria. She had been taking phenytoin as anticonvulsant prophylaxis since the age of 16 but had been free of any fit and otherwise in good health until eight months earlier, when she began to notice symptoms of asthma. Control had proved difficult, requiring three hospital admissions, though ventilatory assistance had not been needed. During the most recent admission eight weeks earlier a chest X-ray had demonstrated widespread alveolar infiltrates and spirometry a restrictive deficit (FEV, of 1.4 and FVC of 1.7) with a peak flow of 250 L/min. She had also complained of dysaesthetic pain in both feet, and nerve conduction studies had shown changes of mild to moderate peripheral neuropathy. Some weeks later a vasculitic rash appeared at the extremities. Full blood counts showed an increasing eosinophilia count of up to 35%.A diagnosis of Churg-Strauss syndrome was made, and she was maintained on prednisolone (40 mg) once daily with inhaled bronchodilators, but continued to deteriorate, developing mononeuropathies of both hands. A week before admission she began to have persistent hypertension and proteinuria.On admission the blood pressure was 160/100 mmHg, and the JVP visible at 2 cm. There was mild ankle oedema. Coarse crackles were audible throughout both lung fields. A confluent purpuric rash was present over the dorsal aspects of both feet and ankles. Both feet were dysaesthetic, with marked contact hypersensitivity, and ankle dorsiflexion was weak (MRC grade 4).There were signs of left ulnar and right median nerve palsies. High doses of nifedipine and methyldopa were required to bring her blood pressure down to 140/90 mmHg. Azathioprine was added as a steroidsparing measure, and weekly dexamethasone injections administered in anticipation of preterm delivery. Satisfactory blood pressure control and reduction in proteinuria were achieved over the following three weeks, but her weakness and neuropathic pain continued to progress. Pulsed intravenous cyclophosphamide was started in a regime of 1 g/m2 with 0.6 mg of mesna (in divided doses) at monthly intervals, which was followed after two weeks by improved pain control and discharge home. No further progression of the mononeuropathies was observed at follow up two months later.She was readmitted at 30 weeks with preterm prelabour rupture of the membranes, and induction of labour was started that day, with an oxytocin infusion. After three hours she underwent an emergency caesarean section for a compound presentation of the first twin. Two female infants were born with birthweights of 1.1 kg and 1 a09 kg. Twin one was in good condition and had an uneventful postnatal course. Twin two required minimal ventilatory support for 24 hours. Both 746 0 RCOG 1997 British Journal of Obstetrics and Gynaecology
C A S E R E P O R T S 747infants were discharged home after 40 days and were well. The mother made an uneventful recovery with stabilisation of her blood ...