Cardiovascular magnetic resonance imaging detects cardiac involvement in Churg-Strauss syndrome.

BACKGROUND: Cardiac involvement in Churg-Strauss vasculitis worsens the prognosis. Early detection is, therefore, warranted. Cardiac magnetic resonance (CMR) can visualize various forms of inflammatory changes in the myocardium. We tested whether CMR could elucidate cardiac damage in patients with biopsy-proven Churg-Strauss syndrome and clinical evidence of cardiac involvement. METHODS AND RESULTS: Eleven patients underwent a CMR protocol including cine imaging for left ventricular function in long axes, T2-weighted imaging for edema detection, and contrast-enhanced T1-weighting for early and late gadolinium enhancement. CMR detected various form of myocardial injury in all patients. Systolic left ventricular function was impaired in 6 patients. Mean left ventricular ejection fraction was 45 +/- 15%. Left ventricular size was mildly enlarged (left ventricular end-diastolic volume index 94 +/- 23 mL/m(2)). Edema was present in 4 cases; 7 patients had pericardial effusion. Six patients had increased early contrast uptake. CMR detected late enhancement lesions in 9 of 11 patients, even in those with normal left ventricular size and function. CONCLUSIONS: CMR has the potential to detect myocardial injury in Churg-Strauss syndrome even when left ventricular function appears normal.

Prolonged treatment of refractory Wegener's granulomatosis with 15-deoxyspergualin: an open study in seven patients.

BACKGROUND: A subset of patients with Wegener's granulomatosis does not respond to daily oral cyclophosphamide (CYC) plus corticosteroids or suffers from intolerable side effects. A 6 month course of the immunosuppressant 15-deoxyspergualin (DSG) has previously been employed successfully in these refractory cases. However, there are no reports on long-term treatment with DSG. METHODS: To document the effects of prolonged DSG treatment, this study reports on seven patients suffering refractory Wegener's granulomatosis, who were successfully treated with DSG over an average of 26.5 months (range: 11-55.5 months). RESULTS: Before administration of DSG, patients had experienced an average of 6.6 relapses (range: 3-12) under an average of 5.4 (range: 2-11) different therapeutic approaches, which included CYC in all cases. All suffered active disease when DSG was initiated. Four were unresponsive to CYC and three did not tolerate it. DSG (0.5 mg/kg/day subcutaneous) was given for 2-3 weeks until the leukocyte count dropped to 3000/microl, followed by a rest until a leukocyte count of 4000/microl was reached again. No other immunosuppressants besides corticosteroids were given. All patients showed a long-lasting, favourable response to DSG with complete (n = 5) or partial (n = 2) remission. Only one case relapsed while being treated with DSG. Termination/interruption of DSG was followed by relapse in four of five occasions. Resumption of DSG led to complete remission. Currently, five of the seven patients are still treated with DSG and are in remission. Infections, mainly of the respiratory tract, were observed in five cases and resolved after treatment. One case developed a third-degree heart block that required pacing. CONCLUSIONS: In patients with refractory Wegener's granulomatosis, prolonged treatment with DSG seems safe and successful.

15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy.

The combination of cyclophosphamide (CYC) and oral corticosteroids is effective in the majority of patients with antineutrophil cytoplasmic antibody-associated vasculitis (AASV), but it carries substantial risk of drug-related morbidity and mortality. New regimens are desired, especially in refractory cases. The immunosuppressant 15-deoxyspergualin (DSG) is effective in experimental autoimmune disease and transplantation as well as in acute kidney transplant rejection in humans. To assess the efficacy and safety of DSG, an open label multicenter trial was conducted in patients with AASV who were either unresponsive or had contraindications for standard immunosuppressants. Included were 19 cases of Wegener granulomatosis and one case of microscopic polyangiitis. Nine of them had received CYC shortly before study entry without apparent therapeutic success. DSG (0.5 mg/kg per d) was given for 2 to 3 wk until the WBC count dropped to 3000/ micro l followed by a rest until at least a WBC of 4000/ micro l was reached again. This was repeated up to six cycles. During the study, no other immunosuppressants besides steroids were allowed. Disease improvement during treatment with DSG was achieved in 70% of cases (six cases of complete remission; eight cases of partial remission). Leucopenia occurred in each patient in a regular pattern during the cycles and was transient without exception. No mortality or septicemia was observed. Mild to moderate infections mainly in the respiratory tract were observed but resolved under adequate treatment without sequel. It is concluded that treatment with DSG is successful in patients with refractory Wegener granulomatosis under careful monitoring of WBC count.