CHRONIC FATIGUE SYNDROME INFORMATION
We all get tired. Many of us at times have felt depressed. But the mystery known as chronic fatigue syndrome is not like the normal ups and downs we experience in everyday life. The early sign of this illness is a strong and noticeable fatigue that comes on suddenly and often comes and goes or never stops. You feel too tired to do normal activities or are easily exhausted with no apparent reason. Unlike the mind fog of a serious hangover, to which researchers have compared Chronic Fatigue Syndrome, the profound weakness of Chronic Fatigue Syndrome does not go away with a few good nights of sleep. Instead, it slyly steals your energy and vigor over months and sometimes years.
How Chronic Fatigue Syndrome Begins and Its Symptoms
For many people, Chronic Fatigue Syndrome begins after a bout with a cold, bronchitis, hepatitis, or an intestinal bug. For some, it follows a bout of infectious mononucleosis, or mono, which temporarily saps the energy of many teenagers and young adults. Often, people say that their illnesses started during a period of high stress. In others, Chronic Fatigue Syndrome develops more gradually, with no clear illness or other event starting it.
Unlike flu symptoms, which usually go away in a few days or weeks, Chronic Fatigue Syndrome symptoms either hang on or come and go frequently for more than six months. Chronic Fatigue Syndrome symptoms include:
Headache
Tender lymph nodes
Fatigue and weakness
Muscle and joint aches
Inability to concentrate
Who Gets Chronic Fatigue Syndrome?
Chronic Fatigue Syndrome was once stereotyped as a new "yuppie flu" because those who sought help for and caused scientific interest in Chronic Fatigue Syndrome in the early 1980s were mainly well-educated, well-off women in their thirties and forties. Similar illnesses, known by different names, however, date back at least to the late 1800s. The modern stereotype arose. Since then, doctors have seen the syndrome in people of all ages, races, and social and economic classes from several countries around the world.
Still, Chronic Fatigue Syndrome is diagnosed two to four times more often in women than in men, possibly because of biological, psychological, and social influences. For example,
Chronic Fatigue Syndrome may have a gender difference similar to diseases such as systemic lupus erythematosus and multiple sclerosis, which affect more women than men.
Women may be more likely than men to talk with their doctors about Chronic Fatigue Syndrome-like symptoms.
Some members of the medical community and the public do not know about or are skeptical of the syndrome.
An increasingly diverse patient group will likely emerge as more doctors see Chronic Fatigue Syndrome as a real disorder.
How Many People Have Chronic Fatigue Syndrome?
Because there is no specific laboratory test or clinical sign for Chronic Fatigue Syndrome, no one knows how many people this illness affects. CDC estimates, however, that as many as 500,000 people in the United States have a Chronic Fatigue Syndrome-like condition.
What Causes Chronic Fatigue Syndrome?
While no one knows what causes Chronic Fatigue Syndrome, for more than a century, doctors have reported seeing illnesses similar to it. In the l860s, Dr. George Beard named the syndrome neurasthenia because he thought it was a nervous disorder with weakness and fatigue. Since then, health experts have suggested other explanations for this baffling illness.
Iron-poor blood (anemia)
Low blood sugar (hypoglycemia)
Environmental allergy
A body wide yeast infection (candidiasis)
In the mid-1980s, the illness became labeled "chronic EBV" when laboratory clues led scientists to wonder whether the Epstein-Barr virus (EBV) might be causing this group of symptoms. New evidence soon cast doubt on the theory that EBV could be the only thing causing Chronic Fatigue Syndrome. High levels of EBV antibodies (disease-fighting proteins) have now been found in some healthy people as well as in some people with Chronic Fatigue Syndrome. Likewise, some people who don't have EBV antibodies, and who thus have never been infected with the virus, can show Chronic Fatigue Syndrome symptoms.
Chronic Fatigue Syndrome Symptoms
Primary Chronic Fatigue Syndrome Symptoms
As the name chronic fatigue syndrome suggests, this illness is accompanied by fatigue. However, it's not the kind of fatigue patients experience after a particularly busy day or week, after a sleepless night or after a stressful event. It's a severe, incapacitating fatigue that isn't improved by bed rest and that may be exacerbated by physical or mental activity. It's an all-encompassing fatigue that results in a dramatic decline in both activity level and stamina.
People with Chronic Fatigue Syndrome function at a significantly lower level of activity than they were capable of prior to becoming ill. The illness results in a substantial reduction in occupational, personal, social or educational activities.
A Chronic Fatigue Syndrome diagnosis should be considered in patients who present with six months or more of unexplained fatigue accompanied by other characteristic symptoms. These symptoms include:
cognitive dysfunction, including impaired memory or concentration
post exertional malaise lasting more than 24 hours (exhaustion and increased symptoms) following physical or mental exercise
unrefreshing sleep
joint pain (without redness or swelling)
persistent muscle pain
headaches of a new type or severity
tender cervical or axillary lymph nodes
sore throat
Other Chronic Fatigue Syndrome Symptoms
In addition to the eight primary defining symptoms of Chronic Fatigue Syndrome, a number of other symptoms have been reported by some Chronic Fatigue Syndrome patients. The frequency of occurrence of these symptoms varies among patients. These symptoms include:
irritable bowel, abdominal pain, nausea, diarrhea or bloating
chills and night sweats
brain fog
chest pain
shortness of breath
chronic cough
visual disturbances (blurring, sensitivity to light, eye pain or dry eyes)
allergies or sensitivities to foods, alcohol, odors, chemicals, medications or noise
difficulty maintaining upright position (orthostatic instability, irregular heartbeat, dizziness, balance problems or fainting)
psychological problems (depression, irritability, mood swings, anxiety, panic attacks)
jaw pain
weight loss or gain
Clinicians will need to consider whether such symptoms relate to a comorbid or an exclusionary condition; they should not be considered as part of Chronic Fatigue Syndrome other than they can contribute to impaired functioning.
Further explanation about the symptoms of Chronic Fatigue Syndrome
The severity of Chronic Fatigue Syndrome varies from patient to patient, with some people able to maintain fairly active lives. By definition, however, Chronic Fatigue Syndrome significantly limits work, school and family activities.
While symptoms vary from person to person in number, type and severity, all Chronic Fatigue Syndrome patients are functionally impaired to some degree. CDC studies show that Chronic Fatigue Syndrome can be as disabling as multiple sclerosis, lupus, rheumatoid arthritis, heart disease, end-stage renal disease ,chronic obstructive pulmonary disease (COPD) and similar chronic conditions.
Chronic Fatigue Syndrome often follows a cyclical course, alternating between periods of illness and relative well-being. Some patients experience partial or complete remission of symptoms during the course of the illness, but symptoms often reoccur. This pattern of remission and relapse makes Chronic Fatigue Syndrome especially hard for patients and their health care professionals to manage. Patients who are in remission may be tempted to overdo activities when they're feeling better, which can exacerbate symptoms and fatigue and cause a relapse. In fact, post exertional malaise is a hallmark of the illness.
The percentage of Chronic Fatigue Syndrome patients who recover is unknown, but there is some evidence to indicate that the sooner symptom management begins, the better the chance of a positive therapeutic outcome. This means early detection and treatment are of utmost importance. CDC research indicates that delays in diagnosis and treatment may complicate and prolong the clinical course of the illness.
Chronic Fatigue Syndrome Causes
There is no single known cause for CFS. Studies have pointed to several different conditions that might be responsible. These include:
Viral infections
Chemical toxins
Allergies
Immune abnormalities
Psychological disorders.
Multiple causes for Chronic Fatigue Syndrome
Although the cause is still controversial, many doctors and researchers now think that CFS may not be a single illness. Instead, they think CFS may be a group of symptoms caused by several conditions. One theory is that a microorganism, such as a virus, or a chemical injures the body and damages the immune system, allowing dormant viruses to become active. About 90% of all people have a virus in the herpes family dormant (not actively growing or reproducing) in their bodies since childhood. When these viruses start growing again, the immune system may overreact and produce chemicals called cytokines that can cause flu-like symptoms. Immune abnormalities have been found in studies of people with CFS, although the same abnormalities are also found in people with allergies, autoimmune diseases, cancer, and other disorders.
The role of inflammation in Chronic Fatigue Syndrome
Recent studies have shown that chronic fatigue syndrome may be caused by inflammation of pathways in the nervous system, and that this inflammation may be some sort of immune response or autoimmune process. C.F.S. may occur when a viral illness is complicated by an inadequate or dysfunctional immune response. Other factors (such as age, prior illness, stress, environment, or genetic disposition) may also play a role. C.F.S. most commonly occurs in women ages 30 to 50.
Depression not a cause for Chronic Fatigue Syndrome
The role of psychological problems in CFS is very controversial. Because many people with CFS are diagnosed with depression and other psychiatric disorders, some experts conclude that the symptoms of CFS are psychological. However, many people with CFS did not have psychological disorders before getting the illness. Many doctors think that patients become depressed or anxious because of the effects of the symptoms of their CFS. One recent study concluded that depression was the result of CFS and was not its cause.
Chronic Fatigue Syndrome Diagnosis
Chronic Fatigue Syndrome is diagnosed by evaluating symptoms and eliminating other causes of fatigue. Doctors carefully question patients about their symptoms, any other illnesses they have had, and medications they are taking. They also conduct a physical examination, neurological examination, and laboratory tests to identify any underlying disorders or other diseases that cause fatigue. In the United States, many doctors use the CDC case definition to determine if a patient has Chronic Fatigue Syndrome.
The health care provider can presume a diagnosis of chronic fatigue syndrome only after ruling out all other known possible causes of fatigue, such as:
infections.
immune or autoimmune disorders.
tumors.
muscle or nerve diseases (such as multiple sclerosis).
endocrine diseases (such as hypothyroidism).
psychiatric or psychological illnesses, particularly depression. Since Chronic Fatigue Syndrome itself may be associated with depression, a diagnosis of depression does not rule out Chronic Fatigue Syndrome but fatigue related to depression alone must be ruled out for CFS to be diagnosed.
drug dependence.
other illnesses (such as heart, kidney, liver diseases).
To be diagnosed with Chronic Fatigue Syndrome Diagnosis, patients must meet both of the following criteria:
Unexplained continuing or recurring chronic fatigue for at least six months that is of new or definite onset, is not the result of ongoing exertion, and is not mainly relieved by rest, and causes occupational, educational, social, or personal activities to be greatly reduced.
Four or more of the following symptoms: loss of short-term memory or ability to concentrate; sore throat; tender lymph nodes; muscle pain; multi-joint pain without swelling or redness; headaches of a new type, pattern, or severity; unrefreshing sleep; and post-exertional malaise (a vague feeling of discomfort or tiredness following exercise or other physical or mental activity) lasting more than 24 hours. These symptoms must have continued or recurred during six or more consecutive months of illness and must not have started before the fatigue began.
Chronic Fatigue Syndrome Treatment
There is no cure for CFS, but many treatments are available to help relieve the symptoms. Treatments usually are individualized to each person's particular symptoms and needs. The first treatment most doctors recommend is a combination of rest, exercise, and a balanced diet. Prioritizing activities, avoiding overexertion, and resting when needed are key to maintaining existing energy reserves. A program of moderate exercise helps to keep patients from losing physical conditioning, but too much exercise can worsen fatigue and other CFS symptoms. Counseling and stress reduction techniques also may help some people with CFS.
Many medications, nutritional supplements, and herbal preparations have been used to treat CFS. While many of these are unproven, others seem to provide some people with relief. People with CFS should discuss their treatment plan with their doctors, and carefully weigh the benefits and risks of each therapy before making a decision.
Drugs us as a Chronic Fatigue Syndrome Treatment
Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen, may be used to relieve pain and reduce fever. Another medication that is prescribed to relieve pain and muscle spasms is cyclobenzaprine (sold as Flexeril).
Many doctors prescribe low dosages of antidepressants for their sedative effects and to relieve symptoms of depression. Antianxiety drugs, such as benzodiazepines or buspirone may be prescribed for excessive anxiety that has lasted for at least six months.
Other medications that have been tested or are being tested for treatment of CFS are:
Fludrocortisone (Florinef), a synthetic steroid, which is currently being tested for treatment of people with CFS. It causes the body to retain salt, thereby increasing blood pressure. It has helped some people with CFS who have neurally mediated hypotension.
Beta-adrenergic blocking drugs, often prescribed for high blood pressure. Such drugs, including atenolol (Tenoretic, Tenormin) and propranolol (Inderal), are sometimes prescribed for neurally mediated hypotension.
Gamma globulin, which contains human antibodies to a variety of organisms that cause infection. It has been used experimentally to boost immune function in people with CFS.
Ampligen, a drug which stimulates the immune system and has antiviral activity. In one small study, ampligen improved mental function in people with CFS.
Treatment of Chronic Fatigue Syndrome with Cognitive Behavioral Therapy (CBT)
Cognitive behavioral therapy, or CBT, is often prescribed to help chronically ill patients cope with illness and develop behaviors and strategies that help alleviate symptoms. It has been successful in helping patients with cardiovascular disease, diabetes and cancer, and recent studies indicate that CBT can be useful in treating some CFS patients.
CBT is frequently prescribed as part of therapeutic process; it help patients learn to manage activity levels, stress and symptoms. Optimally, CBT can help you better adapt to the impact of CFS and improve your level of function and quality of life.
Alternative treatment used for Chronic Fatigue Syndrome
A variety of nutritional supplements are used for treatment of CFS. Among these are vitamin C, vitamin B12, vitamin A, vitamin E, and various dietary minerals. These supplements may help improve immune and mental functions. Several herbs have been shown to improve immune function and have other beneficial effects. Some that are used for CFS are astragalus (Astragalus membranaceus), echinacea (Echinacea spp.), garlic (Allium sativum), ginseng (Panax ginseng), gingko (Gingko biloba), evening primrose oil (Oenothera biennis), shiitake mushroom extract (Lentinus edodes), borage seed oil , and quercetin.
Many people have enhanced their healing process for CFS with the use of a treatment program inclusive of one or more alternative therapies. Stress reduction techniques such as biofeedback, meditation, acupuncture, and yoga may help people with sleep disturbances relax and get more rest. They also help some people reduce depression and anxiety caused by CFS.
CERVICAL SPONDYLOSIS
Cervical spondylosis is a disorder caused by abnormal wear on the cartilage and bones of the neck (cervical vertebrae) with degeneration and mineral deposits in the cushions between the vertebrae (cervical disks). Cervical spondylosis results from chronic degeneration of the cervical spine including the cushions between the neck vertebrae (cervical disks) and joints between the bones of the cervical spine. There may be abnormal growths or "spurs" on the vertebrae (the bones of the spine).
These accumulated changes caused by degeneration can gradually compress one or more of the nerve roots. This can lead to increasing pain in the neck and arm, weakness, and changes in sensation. In advanced cases, the spinal cord becomes involved. This can affect not just the arms, but the legs as well.
A previous neck injury (which may have occurred several years prior) can predispose to spondylosis, but the major risk factor is aging. By age 60, 70% of women and 85% of men show changes consistent with cervical spondylosis on X-ray.
Description of Cervical Spondylosis
As it runs from the brain down the back, the spinal cord is protected by ring like bones, called vertebrae, stacked one upon the other. The vertebrae are not in direct contact with one another, however. The intervening spaces are filled with structures called disks. The disks are made up of a tough, fibrous outer tissue with an inner core of elastic or gel-like tissue.
One of the most important functions of disks is protecting the vertebrae and the nerves and blood vessels between the vertebrae. The disks also lend flexibility to the spinal cord, facilitating movements such as turning the head or bending the neck. As people age, disks gradually become tougher and more unyielding. Disks also shrink with age, which reduces the amount of padding between the vertebrae.
As the amount of padding shrinks, the spine loses stability. The vertebrae react by constructing osteophytes, commonly known as bone spurs. There are seven vertebrae in the neck; development of osteophytes on these bones is sometimes called cervical osteoarthritis. Osteophytes may help to stabilize the degenerating backbone and help protect the spinal cord.
By age 50, 25-50% of people develop cervical spondylosis; by 75 years of age, it is seen in at least 70% of people. Although shrunken vertebral disks, osteophyte growth, and other changes in their cervical spine may exist, many of these people never develop significant problems.
However, about 50% of people over age 50 experience neck pain and stiffness due to cervical spondylosis. Of these people, 25-40% have at least one episode of cervical radiculopathy, a condition that arises when osteophytes compress nerves between the vertebrae. Another potential problem occurs if osteophytes, degenerating disks, or shifting vertebrae narrow the spinal canal. This pressure compresses the spinal cord and its blood vessels, causing cervical spondylitic myelopathy, a disorder in which large segments of the spinal cord are damaged. This disorder affects fewer than 5% of people with cervical spondylosis. Symptoms of both cervical spondylitic myelopathy and cervical radiculopathy may be present in some people.
Prevention of Cervical Spondylosis
Since cervical spondylosis is part of the normal aging process, not much can be done to prevent it. It may be possible to ward off some or all of the symptoms by engaging in regular physical exercise and limiting occupational or recreational activities that place pressure on the head, neck, and shoulders. The best exercises for the health of the cervical spine are noncontact activities, such as swimming, walking, or yoga. Once symptoms have already developed, the emphasis is on symptom management rather
than prevention.
CERVICAL SPONDYLOSIS DIAGNOSIS
Test used to diagnose Cervical Spondylosis Diagnosis
A spine or neck X-ray shows abnormalities that indicate cervical spondylosis.
A CT scan or spine MRI confirms the diagnosis.
A myelogram (X-ray or CT scan after injection of dye into the spinal column) may be recommended to clearly identify the extent of injury.
An EMG may also be recommended.
More detailed information about Cervical Spondylosis Diagnosis
Cervical spondylosis is often suspected based on the symptoms and their history. Careful neurological examination can help determine which nerve roots are involved, based on the location of the pain and numbness, and the pattern of weakness and changes in reflex responses. To confirm the suspected diagnosis, and to rule out other possibilities, imaging tests are ordered. The first test is an x ray. X rays reveal the presence of osteophytes, stenosis, constricted space between the vertebrae, and misalignment in the cervical spine--in short, an x ray confirms that a person has cervical spondylosis. To demonstrate that the condition is causing the symptoms, more details are needed. Other imaging tests, such as magnetic resonance imaging (MRI) and computed tomography myelography, help assess effects of cervical spondylosis on associated nerve tissue and blood vessels.
An MRI may be preferred, because it is a noninvasive procedure and does not require injecting a contrast medium as does computed tomography myelography. MRIs also have greater sensitivity for detecting disk problems and spinal cord involvement, and they test allows the physician to create permit creating images of a larger area from various angles. However, these images may not show enough detail about the vertebrae themselves. Computed tomography myelography yields a superior image of the bones involved in cervical spondylosis. Added benefits include that it takes less time to perform and tends to be less expensive than an MRI. A good diagnosis may be reached with either a computed tomography myelography or an MRI, but sometimes complementary information from both tests is necessary. Nerve conduction velocity, electromyogram (EMG), and/or somatosensory evoked potential testing may help to confirm which nerve roots are involved.
CERVICAL SPONDYLOSIS SYMPTOMS
neck pain (may radiate to the arms or shoulder)
loss of sensation or abnormal sensations of the shoulders, arms, or (rarely) legs
weakness of the arms or (rarely) legs
neck stiffness that progressively worsens
loss of balance
headaches, particularly in the back of the head
loss of control of the bladder or bowels (if spinal cord is compressed)
Causes of Cervical Spondylosis Symptoms
As people age, shrinkage of the vertebral disks prompts the vertebrae to form osteophytes to stabilize the back bone. However, the position and alignment of the disks and vertebrae may shift despite the osteophytes. Symptoms may arise from problems with one or more disks or vertebrae.
Osteophyte formation and other changes do not necessarily lead to symptoms, but after age 50, half of the population experiences occasional neck pain and stiffness. As disks degenerate, the cervical spine becomes less stable, and the neck is more vulnerable to injuries, including muscle and ligament strains. Contact between the edges of the vertebrae can also cause pain. In some people, this pain may be referred--that is, perceived as occurring in the head, shoulders, or chest, rather than the neck. Other symptoms may include vertigo (a type of dizziness) or ringing in the ears.
General description of Cervical Spondylosis Symptoms
The neck pain and stiffness can be intermittent, as can symptoms of radiculopathy. Radiculopathy refers to compression on the base, or root, of nerves that lead away from the spinal cord. Normally, these nerves fit comfortably through spaces between the vertebrae. These spaces are called intervertebral foramina. As the osteophytes form, they can impinge on this area and gradually make the fit between the vertebrae too snug.
The poor fit increases the chances that a minor incident, such as overdoing normal activities, may place excess pressure on the nerve root, sometimes referred to as a pinched nerve. Pressure may also accumulate as a direct consequence of osteophyte formation. The pressure on the nerve root causes severe shooting pain in the neck, arms, shoulder, and/or upper back, depending on which nerve roots of the cervical spine are affected. The pain is often aggravated by movement, but in most cases, symptoms resolve within 4-6 weeks.
Cervical Spondylosis Symptoms - cervical spondylitic myelopathy
Cervical spondylosis can cause cervical spondylitic myelopathy through stenosis- or osteophyte-related pressure on the spinal cord. Spinal stenosis is a narrowing of the spinal canal-- the area through the center of the vertebral column occupied by the spinal cord. Stenosis occurs because of misaligned vertebrae and out-of-place or degenerating disks. The problems created by spondylosis can be exacerbated if a person has a naturally narrow spinal canal. Pressure against the spinal cord can also be created by osteophytes forming on the inner surface of vertebrae and pushing against the spinal cord. Stenosis or osteophytes can compress the spinal cord and its blood vessels, impeding or choking off needed nutrients to the spinal cord cells; in effect, the cells starve to death.
With the death of these cells, the functions that they once performed are impaired. These functions may include conveying sensory information to the brain or transmitting the brain's commands to voluntary muscles. Pain is usually absent, but a person may experience leg numbness and an inability to make the legs move properly. Other symptoms can include clumsiness and weakness in the hands, stiffness and weakness in the legs, and spontaneous twitches in the legs. A person's ability to walk is affected, and a wide-legged, shuffling gait is sometimes adopted to compensate for the lack of sensation in the legs and the accompanying, realistic fear of falling. In very few cases, bladder control becomes a problem
CERVICAL SPONDYLOSIS TREATMENT
When possible, conservative treatment of symptoms is preferred. Conservative treatment begins with rest--either restricting normal activities to a less strenuous level or bed rest for 3-5 days. If rest is not adequate to relieve symptoms, a cervical orthosis may be prescribed., such as a soft cervical collar or stiffer neck brace to restrict neck movement and shift some of the head's weight from the neck to the shoulders. Cervical traction may also be suggested, either at home with the advice of a physical therapist or in a health-care setting.
Drugs used to as a Cervical Spondylosis Treatment
Pain is treated with nonsteroidal anti-inflammatory drugs, such as aspirin or ibuprofen. If these drugs are ineffective, a short-term prescription for corticosteroids or muscle relaxants may be given. For chronic pain, tricyclic antidepressants can be prescribed. Although these drugs were developed to treat depression, they are also effective in treating pain. Once any pain is resolved, exercises to strengthen neck muscle and preserve flexibility are prescribed.
If the pain is severe, a short treatment of epidural corticosteroids may be prescribed with discretion. A corticosteroid such as prednisone can be combined with an anaesthetic and injected with a long needle into the space between the damaged disk and the covering of the nerve and spinal cord. Injection into the cervical epidural space relieves severe pain that is not managed with conventional treatment. Frequent use of this treatment is not medically recommended and is used only if the more conservative therapy is not effective.
Surgery as a Cervical Spondylosis Treatment
If pain is continuous and does not respond to conservative treatment, surgery may be suggested. Surgery is usually not recommended for neck pain, but it may be necessary to address radiculopathy and myelopathy. Surgery is particularly recommended for people who have already developed moderate to severe symptoms of myelopathy, although age or poor health may prohibit that recommendation. The specific details of the surgery depend on the structures involved, but the overall goal is to relieve pressure on the nerve root, spinal cord, or blood vessels and to stabilize the spine.
CHURG-STRAUSS SYNDROME
SYMPTOMS OF CHURG-STRAUSS SYNDROME
Churg-Strauss Syndrome (CSS) is a systemic vasculitis. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of "asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems". CSS shares many of the clinical and pathological features of polyarteritis nodosa ("PAN", another type of vasculitis). Churg and Strauss discovered that the presence of granulomas as well as the abundance of eosinophils distinguished this disease from PAN. Another name for Churg-Strauss Syndrome is Allergic granulomatosis
Asthma is one of the cardinal features of CSS. Asthma symptoms may begin long before the onset of vasculitis – e.g., many years before any other symptoms of CSS arise, and long before the diagnosis of CSS is made. Other early symptoms/signs include nasal polyps and allergic rhinitis.
The next phase of the disease is often marked by eosinophilia, the finding of an excessive number of eosinophils in the blood or in tissues. An eosinophil is one subtypes of white blood cell. Normally, eosinophils comprise 5% or less of the total white blood cell count. In CSS, the percentage of eosinophils may reach as high as 60%. In the picture below, the eosinophils are shown by the dark pink stain.
The third phase of the illness is a vasculitis, which involves the skin, lungs, nerves, kidneys, and other organs. Particular mention should be made of the frequent devastating involvement of the nerves (called mononeuritis multiplex), which produces severe tingling, numbess, shooting pains, and severe muscle wasting/power loss in the hands or feet. The list below contains the organs commonly involved by CSS and the specific disease manifestation(s) in each organ.
nose
Sinusitis, including allergic rhinitis
Nasal polyps
Lung
Pulmonary infiltrates (only one-third of all patients)
Bleeding into the lungs (occasionally)
Diffuse interstitial lung disease (rarely)
Skin
Rashes
Palpable purpura
Nodules (above or below the skin), often at sites of pressure, such as the elbows
Kidney
Glomerulonephritis (inflammation in the small units of the kidney that filter blood) Hypertension
Gastrointestinal
Lesions (vasculitic) are occasionally found in the GI tract
Granuloma sometimes found in spleen
Nerve
Peripheral nerve involvement including pain, numbness, or tingling in extremities (neuropathy/mononeuritis multiplex)
Cause of Churg-Strauss Syndrome
The cause of CSS is unknown but is probably multi-factorial. Genetics may play a small role in the disease, but CSS is almost never seen in two members of the same family. Environmental factors such as exposure to industrial solvents may play a role in susceptibility to this disease, but this is largely speculative. Infections may be the inciting event(s), but to date there is no definitive evidence of this.
Diagnosis of Churg-Strauss Syndrome
Among all of the vasculitides, asthma is a distinctive feature of CSS alone. However, not all patients with asthma have vasculitis – only a tiny minority do, in fact. It is the specific combination of symptoms and signs, the pattern of organ involvement, and the presence of certain abnormal blood tests (eosinophilia, in particular) that help the doctor make the diagnosis. In addition to a detailed history and physical examination, blood tests, chest X-rays and other types of imaging studies, nerve conduction tests, and tissue biopsies (e.g., of lung, skin, or nerve) may be performed to help diagnose CSS.
The American College of Rheumatology (ACR) has established criteria that must be fulfilled in order to classify a patient as having CSS. These criteria were intended to distinguish CSS from other forms of vasculitis (for the purposes of research studies). Not all patients meet every criterion. Some, in fact, may have only 2 or 3 criteria, yet their physicians are still comfortable classifying their disease as CSS. The ACR selected 6 disease features (criteria) as being those that best distinguished CSS from other vasculitides. In order to be classified as a CSS patient in a research study, therefore, a patient should have at least 4 of the 6 ACR criteria.
These criteria include: 1) asthma; 2) eosinophilia [>10% on differential WBC count]; 3) mononeuropathy; 4) transient pulmonary infiltrates on chest X-rays; 5) paranasal sinus abnormalities; and 6) biopsy containing a blood vessel with extravascular eosinophils.
Treatment of Churg-Strauss Syndrome
CSS usually responds to prednisone. Initially, high doses of oral prednisone are used in an attempt to get the disease into remission as quickly as possibly (e.g., using oral prednisone 40-60 mg/day). After the first month or so, this high dose of prednisone is gradually tapered down over the ensuing months. Other immunosuppressive drugs, such as azathioprine, cellcept, methotrexate, or cyclophosphamide may be used in addition to prednisone. High doses of intravenous steroids (usually methylprednisolone) maybe useful for those patients with severe disease or for those who are unresponsive to the combination of oral prednisone used with other immunosuppressive medications.
Prior to the advent of prednisone, CSS was often a fatal disease. The majority of patients died from rampant, uncontrolled disease. With present therapy, constitutional symptoms begin to resolve quite quickly, with gradual improvement in cardiac and renal function, as well as improvement in the pain that results from peripheral nerve involvement. The course of therapy can last for 1 to 2 years, although the length and type of treatment depend on the severity of disease and the organs involved. The patient's response to treatment and the continuation of disease control during lowering of the prednisone dose are the primary determinants of how long therapy is continued. Laboratory monitoring of blood tests is very helpful in gauging the activity of disease. Some of the most useful laboratory tests are the erythrocyte sedimentation rate (ESR) and the eosinophil count.
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