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Churg-Strauss syndrome facts
- Churg-Strauss syndrome is a disease characterized by inflammation of the blood vessels.
- Churg-Strauss syndrome occurs in patients with a history of asthma or allergy.
- Symptoms of Churg-Strauss syndrome include fatigue, weight loss, nasal passage inflammation, numbness, and weakness.
- The ultimate test for the diagnosis is a biopsy of involved tissue.
- Treatment of Churg-Strauss syndrome involves stopping inflammation and suppressing the immune system.
What is Churg-Strauss syndrome?
Churg-Strauss syndrome is one of many forms of vasculitis. Vasculitis diseases are characterized by inflammation of blood vessels. Churg-Strauss syndrome, in particular, occurs in patients with a history of asthma or allergy and features inflammation of blood vessels (also referred to as angiitis) in the lungs, skin, nerves, and abdomen. The blood vessels involved in Churg-Strauss syndrome are small arteries and veins.
What causes Churg-Strauss syndrome?
Churg-Strauss syndrome is rare. The cause of the syndrome is not known, but it involves an abnormal over-activation of the immune system in a person with underlying bronchospastic lung disease (asthma). While Churg-Strauss syndrome has been reported to be associated with certain asthma medications, called leukotriene modifiers, whether they actually cause the disease or whether the patients that take them have more severe asthma that lends a tendency toward the development of Churg-Strauss is not yet clear.
What are symptoms of Churg-Strauss syndrome?
Churg-Strauss syndrome causes fever, weight loss, and sinus or nasal passage inflammation in the patient with asthma. Fatigue is common. Sometimes the asthma actually improves somewhat as the disease intensifies elsewhere. Cough, shortness of breath, and chest pain can occur as the lungs are affected by vasculitis.
Skin lumps, called nodules, can appear on the extremities. Diarrhea and pain in the belly occur due to blood vessel inflammation within the abdomen. The bladder and prostate gland can become inflamed.
How is Churg-Strauss syndrome diagnosed?
Churg-Strauss syndrome is suggested when the symptoms described above occur in a patient with a history of asthma.
Abnormalities of the lungs, skin, and nerves might be noted by the doctor during the examination. Blood pressure can be elevated.
Blood examination generally shows elevated levels of an uncommon white blood cell, called an eosinophil, and other white blood cells are also elevated in number. Kidney function blood tests and urinalysis can be abnormal when the kidneys are affected (which is not common).
The ultimate test for the diagnosis is a biopsy of involved tissue, which demonstrates a characteristic pattern of inflammation visible under a microscope. Eosinophil are also seen accumulated in the abnormal tissue.
How is Churg-Strauss syndrome treated?
The treatment of patients with Churg-Strauss syndrome is directed toward both immediately quieting the inflammation of the blood vessels (vasculitis) and suppressing the immune system. Treatment usually includes high doses of cortisone-related medication (such as prednisone or prednisolone) to calm the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan).
Traditionally, cyclophosphamide has been given for a year or more in patients with Churg-Strauss syndrome. In a research study, Churg-Strauss syndrome patients did equally well if treated with cyclophosphamide for 6 or for 12 months. This study suggests that doctors might now be able to recommend a shorter (and, therefore, less toxic) course of Cytoxan for patients with Churg-Strauss syndrome.
What is the outlook (prognosis) for patients with Churg-Strauss syndrome?
Churg-Strauss syndrome is a serious disease that can be fatal. Untreated it is extremely dangerous and threatens the organs that are affected. With aggressive treatment and monitoring it can be quieted and total inactivation of the disease (remission) is possible.
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King, Talmadge E., MD. "Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)," UptoDate. Updated Jul 8, 2016.
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BronchoscopyBronchoscopy is a procedure during which a patient's lung, airways, voice box, vocal cord, trachea, and many branches of the bronchi may be evaluated. There are two types of bronchoscopies: a flexible fiberoptic bronchoscope and a rigid bronchoscope. Bronchoscopy can be used for diagnosis and treatment. Some indications for bronchoscopy include:
- persistent unexplained cough,
- bloody sputum,
- abnormal chest x-ray,
- evaluation of possible lung infection,
- remove foreign bodies in the airway,
- place a stent to open a collapsed lung,
- or to remove a mass or growth blocking the airway.
CAT ScanA CT scan is an X-ray procedure that combines many X-ray images with the aid of a computer to generate cross-sectional and three-dimensional images of internal organs and structures of the body. A CT scan is a low-risk procedure. Contrast material may be injected into a vein or the spinal fluid to enhance the scan.
Chest X-rayChest X-Ray is a type of X-Ray commonly used to detect abnormalities in the lungs. A chest X-ray can also detect some abnormalitites in the heart, aorta, and the bones of the thoracic area. A chest X-ray can be used to define abnormalities of the lungs such as:
- excessive fluid (fluid overload or pulmonary edema),
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cyclophosphamideCyclophosphamide (Cytoxan) is a drug prescribed for the treatment of certain cancers including breast cancer, leukemia, and ovarian cancer. Side effects, drug interactions, patient safety information, and pregnancy and breastfeeding information should be reviewed prior to taking any medication.
prednisolonePrednisolone (Flo-Pred, Pediapred, Orapred, Orapred ODT) is a corticosteroid prescribed to achieve prompt suppression of inflammation due to inflammatory and allergic conditions (for example, rheumatoid arthritis, lupus, gout, ulcerative colitis, Crohn's disease, hay fever, types of dermatitis, and many others. Side effects, drug interactions, dosage, storage, and pregnancy safety information should be reviewed prior to taking this medication.
Prednisone is a drug that belongs to the corticosteroid drug class, and is an anti-inflammatory and immune system suppressant. It's used to treat a variety of diseases and conditions, for example: inflammatory bowel disease (Crohn's disease and ulcerative colitis), lupus, asthma, cancers, and several types of arthritis.
Common side effects are weight gain, headache, fluid retention, and muscle weakness. Other effects and adverse events include glaucoma, cataracts, obesity, facial hair growth, moon face, and growth retardation in children. This medicine also causes psychiatric problems, for example: depression, insomnia, mood swings, personality changes, and psychotic behavior. Serious side effects include reactions to diabetes drugs, infections, and necrosis of the hips and joints.
Corticosteroids like prednisone, have many drug interactions; examples include: estrogens, phenytoin (Dilantin), diuretics, warfarin (Coumadin, Jantoven), and diabetes drugs. Prednisone is available as tablets of 1, 2.5, 10, 20, and 50 mg; extended release tablets of 1, 2, and 5mg; and oral solution of 5mg/5ml. It's use during the first trimester of pregnancy may cause cleft palate. This medicine is secreted in breast milk and can cause side effects in infants who are nursing. You should not stop taking prednisone abruptly because it can cause withdrawal symptoms and adrenal failure. Talk with your doctor, pharmacist, or other medical professional if you have questions about beta-blockers. Talk with your doctor, pharmacist, or other medical professional if you have questions about prednisone.
Relapsing PolychondritisRelapsing polychondritis is an uncommon, chronic disorder of the cartilage that is characterized by recurrent episodes of inflammation of the cartilage of various tissues of the body. Tissues containing cartilage that can become inflamed include the ears, nose, joints, spine, and windpipe (trachea). Tissues that have a biochemical makeup similar to that of cartilage such as the eyes, heart, and blood vessels, can also be affected. Nonsteroidal anti-inflammatory medications (NSAIDs) is used as treatment for mild cases of the disease. Steroid-related medications also are usually required.
Sedimentation RateA sedimentation rate is a common blood test that is used to detect and monitor inflammation in the body. It is performed by measuring the rate at which red blood cells (RBCs) settle in a test tube. The sedimentation rate is simply how far the top of the RBC layer has fallen in one hour, increasing with more inflammation.
VasculitisVasculitis (arteritis, angiitis) is a general term for a group of uncommon diseases which feature inflammation of the blood vessels. Each form of vasculitis has its own characteristic pattern of symptoms. The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.