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Pictures, Symptoms, Life Expectancy, and Causes of Churg Strauss Syndrome

Updated on April 27, 2016

Churg-Strauss syndrome Pictures

What is Churg-strauss syndrome?

Churg-Strauss syndrome is a rare disorder that involves the small to medium arteries and veins of the body. This also referred as allergic granulomatous angiitis. CSS is related to vasculitic syndromes or systemic vasculitis. The pioneers for this disease are Doctors Jacob Churg and Lotte Strauss, who described this disease in year 1951.

Symptoms

The symptoms of this condition can be classified into three stages:

First stage – The patients’ sinuses are involved. Sinus pain or sinusitis development is due to chronic inflammation. There is onset of allergies that were not previously experienced by the patient. Cases present that the allergies are from pre-existing ones. Allergic rhinitis can also be manifested by the patient that involves a runny nose, sneezing and itching.

Second stage – the patient would develop asthma. It could be pre-existing or not. Asthma is a primary sign for CSS. This would develop before other signs and symptoms appear. But others will also experience late-onset asthma.

Third stage – this is considered the most painful stage. Pain is experienced by the patient on his/her legs, arms and hands. This is explained by the severe blood vessel inflammation or known as vasculitis. Sores and bruises are expected to appear in patients on the third stage. In this level, there is involvement of various organs such as the heart, lungs, kidneys, liver and digestive system.

Other presenting symptoms are the following:

  • Fever
  • Weight loss
  • Sinus and nasal inflammation.
  • Easy fatigability
  • Cough
  • Shortness of breath.
  • Skin lumps/nodules in extremities.
  • Diarrhea appears when there is involvement of the digestive system.

Once this syndrome is not treated properly, complications may arise. This may involve various organs such as the following:

Peripheral neuropathy – also known as peripheral nerve damage. This condition can spread throughout the body even to your spinal cord and brain. This damage is thought to be life-lasting and can cause loss of function due to numbness and burning sensations.

Skin scarring – sores that arise from inflammations can leave out scars as they heal off.

Heart disease – this includes inflammation of the vital heart muscles such as the membrane that surrounds the heart causing pericarditis, muscle layer of the heart wall (myocarditis). Heart attack and heart failure are inevitable events too.

Kidney damage – the kidneys primary function of filtering our wastes is hindered thus leading to a buildup of waste products in the bloodstream. This complication is called glomerulonephritis.

Causes

This rare disease has an unknown cause, but according to study this condition involves an abnormal over activation of the immune system in a person with underlying lung disease such as asthma. The role of the immune system is to fight off invading organisms such as bacteria and viruses, but instead it overreacts by targeting our own normal and healthy tissues, thus causing inflammation.

CSS has been reported to develop after using a particular drug. This drug is the one being taken when you have asthma or an allergic reaction such as the group of leukotriene modifiers. But the role of this medication has no direct link to causing Churg-Strauss syndrome according to researchers. It has been known to trigger the symptoms that CSS presents.

People are at risk for this disease and rarely develop the condition. The risk factors for this syndrome include:

Age – diagnosis of this condition is common to those aged 38-52. Elderly and children are not known to acquire such condition, only in rare cases.

Asthma – those with a medical history of asthma or nasal problems are quite at risk for this disorder.

Treatment

Since there is no direct cure for this condition, giving medications that can assist in relieving patients symptoms were developed. With these medications, a good outcome can be attained and reduction from developing complications can be attained.

Corticosteroids such as prednisone are the widely used and prescribed drug for CSS. This helps in controlling the patient’s manifestations of the disorder. Immunosuppressive drugs are given to clients with mild symptoms. These drugs would include cyclophosphamide, azathioprine, or methotrexate. Intravenous immunoglobulin is given in a monthly basis. This has shown helpful effects to people who don’t respond to other medications.

Lifestyle modification is a need to patients with CSS. Smoking cessation is a must and alcohol drinking too. Have a healthy diet. Adhere to what the doctor say that should be avoided while in medication and therapy. Having a regular routine exercise is a must because of the side effect of corticosteroids which is weight gain. This can also add up in protecting your bones. Bone protection is needed for cortisone drugs can predispose you to bone loss and possibly bone fractures. By exercising, you can facilitate bone strengthening. Having enough vitamin D in the diet is a must too, for it improves bone health.

Lastly, monitoring one’s heath by attending the physician’s appointments is a sort of treatment. By this, you have complete control of the condition.

Prognosis

Remission is indeed possible for this disorder despite the fact that it has no cure. With the advancement of today’s medical research, a good prognosis can be attained. But there as instances where a “flare” occurs. This is the new occurrence of CSS symptoms during the remission period. Once this is experienced, treatment should be done rapidly.

Knowing that this disease can be a fatal one, helping oneself to remission is greatly possible. Prompt treatment that is aggressive and constant in monitoring of the case is a good way to attain desirable results.

Once you are in remission, the possibility of recurrence would really bother the affected. The thought of the long-term damage inflicted to the other organs of the body would greatly bother the patient too. But in order to cope with this condition, the affected should act on it. Educating oneself with Churg-Strauss syndrome is necessary. By this action, preparedness can make you ahead of this disorder. Possible complications can be avoided as you learn about this condition. Talking to your doctor about this condition is an aid in attaining good prognosis for this condition.

The role of the family and friends is essential in assisting the client to remission. This support system can be vital especially at time when the patient is in need of advice and understanding of his/her condition. Joining support groups can be a help too.

Life Expectancy

After years of discovering this syndrome, it has been noted that the people who have it has gradually increased their life expectancy over the years. This marked improvement has greatly proven the advancement of our medical research.

In addition, the French Vasculitis Study Group presented a five-point score that predicts the risk of death on Churg-Strauss syndrome. They presented a criterion that can be a basis for the mortality rate of this condition. With the presence of the following conditions/complications will now indicate the life expectancy of the patient:

  • Reduced renal function.
  • Proteinuria (presence of protein in the urine).
  • Gastrointestinal hemorrhage.
  • Central nervous system involvement.
  • Cardiomyopathy

Presence of 1 of these conditions indicates a severe disease, having a 5-year mortality rate of 26%. A very severe disease is indicated if the person has 2 or more of the above conditions, giving them a 46% mortality rate. Only an 11.9% case of mortality has been noted to patients who have none of the above conditions.

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      john anthony 

      12 months ago

      someone with a slightly better grasp of English should review this translation. It detracts from the authenticity of the post . I am concerned that something important may have been lost in translation from original

      .

    • profile image

      Sandy Bird 

      4 years ago

      I have MCTD, Positive ANA - titre 320 coarse speckled indicating SLE and overlapping syndromes of which CSS is one. Flixotide according to NZ Medsafe was too potent with dose inconsistencies and I had a reaction resulting in problems now with all organs, including eyes. NZ Ministry of Health have not wanted to deal with this, shutting down my attempts at trying to get help, or compensation. I have been told to walk on to another Medical center with chest pains, told to find another doctor when I questioned CSS. Told no CSS in NZ and yet documented by a doctor on Patientville/Flixotide site. This now closed but researching FDA/Flixotide allowed me to find again. So many lies told, even my medical records altered. Who will help me?

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