A Case Report of Churg-Strauss Syndrome Presenting With Cardiogenic Shock Treated With Extracorporeal Membrane Oxygenation.

Churg-Strauss Syndrome (CSS) complicated with cardiogenic shock is rare. Few case reports have described successful treatment of this rare disease. However, no one has reported on the application of mechanical life support with extracorporeal membrane oxygenation (ECMO) to treat this life-threatening disease.A 36-year-old female with limb numbness for >10 days, chest tightness for 2 days, and worsening dyspnea for 5 h presented in the emergency room. Vital signs showed a low blood pressure (104/60 mm Hg), increased heart rate (158 bpm), and respiration rate (28 bpm). Laboratory tests revealed that eosinophil was significantly increased (WBC: 34.46 × 10/L, neutrophil: 7.56 × 10/L[21.9%], eosinophil: 23.84 × 10/L[69.2%]), and serum myocardial enzymes was abnormal (CK 1049U/L, CKMB-mass 145.1 µg/L, cTnI 16.24 µg/L). Myocardial injury (tachycardia with ST elevation) and poor heart function (LVEF 31%) were found by electrocardiogram and transthoracic echocardiography. On the next day, cardiogenic shock had been developed as demonstrated by deteriorating the perfusion index.Churg-Strauss Syndrome with cardiogenic shock.A series of conservative therapy with drugs such as corticosteroids, anticoagulant, antiplatelet, nitrates, calcium antagonists, inotrope, and vasopressors were initiated on the day of admission. The treatment was ineffective and a cardiogenic shock developed on the next day. Thus, ECMO was initiated immediately to stabilize circulation and perfusion. At the same time, high-dose corticosteroids combined with immunosuppressive therapy were continuously used.Symptoms of cardiogenic shock were gradually improved after ECMO treatment. Elevated values of cardiac enzymes were decreased and the dose of vasoactive drugs was reduced. Extracorporeal membrane oxygenation was discontinued after 8 days, and the patient was eventually weaned off the ventilator. The patient was discharged after 40 days treatment.Once a CSS develops into a cardiogenic shock, the ECMO should be considered as an alternative therapeutics in that it stabilizes hemodynamic status, maintains effective tissue perfusion, and provides an opportunity for the recovery of cardiac function.

Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin.

BACKGROUND: In some patients with Churg-Strauss syndrome (CSS), especially those with myocardial or neural involvement, conventional treatment with corticosteroids with or without cyclophosphamide is not effective. OBJECTIVE: To examine the effects of intravenous high-dose immunoglobulin (IVIG) in patients with CSS who showed poor responsiveness to conventional treatment. METHODS: We consecutively selected patients with CSS who showed any organ involvement despite corticosteroid treatment with or without cyclophosphamide. The diagnosis was based on the classification criteria of the American College of Rheumatology. IVIG therapy was performed with a dose of 400 mg/kg of immunoglobulin daily for 5 days. Neuropathy was evaluated with the manual muscle strength test and by the skin temperature of affected sites. Cardiac function was examined with ejection fraction by echocardiography and 2 imaging tests of myocardium (iodine 123 metaiodobenzylguanidine and thallium 201). RESULTS: The manual muscle strength test results were improved, and the skin temperature of both hands and legs was increased by IVIG therapy. In 5 patients with heart failure, the mean +/- SD ejection fraction of the left ventricle increased from 35.2% +/- 13.9% to 61.0% +/- 10.1% (P < .02). The uptake of iodine 123 metaiodobenzylguanidine of the myocardium increased, indicating that the myocardial viability was improved. The thallium 201 images revealed the presence of perfusion defects, which were improved by IVIG therapy. CONCLUSIONS: Patients with CSS who are resistant to corticosteroid treatment with or without cyclophosphamide may be treated effectively with IVIG therapy.

[Churg-Strauss syndrome with rectal localization and exudative enteropathy]. / Syndrome de Churg et Strauss avec localisation rectale et entéropathie exsudative.

The authors report the case of a 61-year old patient with a Churg-Strauss syndrome revealed by abdominal pain. Investigations showed a diffuse inflammatory digestive tract involvement, documented by endoscopy associated with protein-losing enteropathy. Deep rectal biopsy revealed vasculitis in an otherwise macroscopically normal rectum. Faced with acute and life-threatening course of disease, emergency medical treatment with steroids and cyclophosphamide led to rapid regression of clinical, biological and radiological abnormalities.