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CHURG-STRAUSS SYNDROME

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CHURG-STRAUSS SYNDROME

Churg-Strauss syndrome is a rare autoimmune disorder marked by inflammation of small-to-medium sized blood vessels.

  • This inflammation can restrict blood flow to vital organs and tissues, permanently damaging them.
  • The most commonly involved organ is the lungs, followed by the skin.
  • However, the condition can affect other organs including the nose, sinuses, joints, skin, intestinal tract, kidneys, heart, and nerves outside the brain and spinal cord.
  • The condition which is now called eosophinolic granulomatosis with polyangiitis (EGPA) has 2-5 new cases a year per 1 million people.
  • The condition affects male and female in equal numbers and can affect individuals of almost any age, although, most cases happen in individuals between 30 to 50 years of age.
  • A main feature of the condition is that all affected individuals have a history of asthma and/or allergies.
  • While some affected individuals may develop life-threatening or serious complications such as heart disease, others individuals are mildly affected with nasal polyps or skin lesions.

CAUSES

The exact cause of the condition is unknown but it is classified as an autoimmune disorder.  It is believed that several different factors including genetics and environment play a role in the development of the Churg-Strauss syndrome.

An autoimmune disorder occurs when the body’s natural defense against foreign organisms such as bacteria and virus starts to attack healthy tissues for no reason, causing widespread inflammation.

The condition is not contagious and does not run in families.

SYMPTOMS

There are three stages or phases each with its own symptoms. Not all affected individuals develop all three stages or progress from one stage to another in the same order.

THE ALLERGIC STAGE

This is the first stage of the condition and it usually the early stage. This stage is characterized by a number of reactions including:

  • Almost all patients experience asthma. Asthma may be newly developed, or a worsening pre-existing one. Asthma develops on average three to nine years before other symptoms appear.
  • Hay fever (rhinitis), causing an itching, runny and stuffy nose
  • Sinus pressure and pain that can be associated with nasal polyps.
THE EOSOPHILONIC STAGE

This is the second stage and it is marked by an abnormally high amount of eosinophil in the blood and tissues. An eosinophil is a type of white blood cell that helps the immune system fight certain infections. Normally, eosinophil makes up a smaller percentage of white blood cells but a high percentage of it in the blood and tissues as seen in churg-Strauss disease can cause serious damage.

The symptoms depend on the part of the body affected, although the lungs and digestive tract including the stomach and esophagus are mostly involved.

Symptoms usually include:

  • Fever
  • A cough
  • Asthma
  • Night Sweats
  • Loss of appetite and weight
  • Joint pain
  • Fatigue
  • Abdominal pain and gastrointestinal
THE VASCULITIS STAGE

This is the last and final stage and is characterized by the inflammation of the blood vessels. This leads to the reduction of blood flow to various organs and tissues throughout the skin, heart, peripheral nervous system, muscles, bones, and digestive tract. The kidney can also be affected.

Depending on the organ affected, symptoms may include:

  • Unintended weight loss
  • Swollen lymph nodes
  • A cough
  • Chest pain
  • Irregular heartbeat
  • Blood urine
  • Diarrhea, nausea, and vomiting
  • Irregular heartbeat
  • Blood in the urine
  • Severe abdominal pain
  • Rash or skin sores
  • Joint aches and swelling
  • Severe pain, numbness, and tingling in your hands and feet

DIAGNOSIS AND TREATMENT

No test can confirm the churg-Strauss syndrome. The American College of Rheumatology has established criteria to identify the condition.

The disease is considered present if four or six of this criteria is found in an individual. This may include:

  • Asthma which may be chronic or severe
  • Higher than normal count of eosinophil
  • Sinus problems
  • White blood cells outside your blood vessels (extravascular eosinophil)
  • Presence of mononeuropathy or polyneuropathy
  • Pulmonary Infiltrates

Several tests such as blood tests, imaging scans, biopsy of affected tissues may be requested by your doctor to help determine if you meet these criteria.

TREATMENT

No cure exists for this condition but certain medications may help manage your symptoms.

Medications used includes:

  • Corticosteroids
  • Other immunosuppressive drugs such as azathioprine, methotrexate.
  • Immune globulin
  • Biologic medications such as rituximab.