Churg-Strauss syndrome

Churg Strauss disease is a medical condition that involves small and medium sized arteries and veins and is usually characterized by inflammation of these vessels resulting to blood restriction to important body organs. Most common causes of morbidity and death among patients are myocarditis and myocardial infarction or heart attack. ^{[1] [2]}

Epidemiology

In the United States, 1-3 cases per 100,000 individuals are recorded annually. Globally, the incidence is estimated at about 2.5 per 100,000 adults every year. Males are more frequently affected and the mean age of occurrence is 38 years old. ^[1]

Causes

The exact cause is unknown. Autoimmune and hereditary factors are suggested in the etiology of the disease. Some drugs were implied as inducing agents of the reactions in some cases of Churg Strauss disease. These drugs include mesalazine, freebase cocaine, leukotriene receptor antagonists and anti-hyperthyroidism medications such as propylthiouracil and methimazole. ^[3]

Signs and Symptoms

Since the disease can involve various body systems, a person may experience a wide array of symptoms. The patient may have nonspecific symptoms like fatigue, fever, loss of weight and appetite. One of the cardinal features of the disease is asthma so that shortness of breath and wheezing are common. In peripheral blood smear, result may show an elevated eosinophil count (>10%). Depending on the organ involved, the patient can have allergic rhinitis, nasal polyps, skin rashes, skin nodules, lung infiltrates, hypertension, numbness, tingling sensation and lesions along the heart and gastrointestinal tract. ^{[1] [3] [4]}

Diagnosis

The physician gets a suspicion of Churg Strauss syndrome from medical history and physical examination. The disease is often suspected when a person with asthma experiences other organ involvement and has abnormal blood test i.e. eosinophilia. As the disease may involve different body systems, laboratory tests are requested depending on the manifestations of the disease. Laboratory workups include complete blood count, renal tests, immunologic tests, chest radiography, CT scan, ECG, EMG and gastrointestinal endoscopy.

Treatment

Usual medications are corticosteroids like prednisone. Patients with severe complications may be prescribed with cyclophosphamide or azathioprine. Intravenous immune globulin, interferon-alpha and plasma exchange may also be given depending on the case. If there is no diffuse organ involvement, treatment often is successful. ^[4]

References:

1.      http://emedicine.medscape.com/article/333492-overview

2.      http://www.mayoclinic.com/health/churg-strauss-syndrome/DS00855

3.      http://www.patient.co.uk/doctor/churg-strauss-syndrome

4.      http://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/