What Are the Signs and Symptoms of Churg-Strauss Syndrome?

Allergic rhinitis is a symptom of Churg-Strauss syndrome.

Churg-Strauss syndrome is the traditional name for eosinophilic granulomatosis with polyangiitis (EGPA), a rare rheumatologic disease that affects multiple organ systems because it causes inflammation in the small blood vessels (vasculitis) throughout the body. This can also result in asthma and blood cell abnormalities.

Because Churg-Strauss syndrome can affect any of the body's tissues, the signs and symptoms the disease vary widely.

Signs and symptoms of Churg-Strauss syndrome include the following:

• Asthma
  • 97% of patients have this symptom
  • May be severe
• Allergic rhinitis (runny nose)
• Sinus infection (sinusitis)
• Feeling unwell (malaise)
• Fatigue
• Flu-like symptoms
• Weight loss
• Fever
• Body aches/muscle pains
• Cough, including coughing up blood
• Skin rashes, including blisters and hives
• Abdominal pain
• Diarrhea
• Joint pain
• Numbness and tingling in extremities (peripheral neuropathy)
• Vision problems
• Heart problems, including heart attack and heart muscle inflammation

The cause of Churg-Strauss (eosinophilic granulomatosis with polyangiitis syndrome) is unknown. It is thought to be a result of an immune dysfunction or an autoimmune reaction, in which the body mistakenly attacks its own tissues. It is unclear what causes the autoimmune reaction, as to whether it is a type of allergic reaction or if it is triggered by the environment or certain medications.

Genetic factors may also play a role.

Some medications that are thought to be associated with Churg-Strauss syndrome include the following:

• Leukotriene modifying agents
• Glucocorticoids
• Omalizumab
• Cocaine

A patient must meet at least four out of six criteria to diagnose Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis syndrome):

1. Asthma
2. Abnormal blood tests, including a type of high white blood cells (eosinophilia)
3. Sinus infection (sinusitis)
4. Pneumonia
5. Biopsy that shows vasculitis with eosinophils
6. Evidence of nerve dysfunction

Tests that may be indicated to help diagnose Churg-Strauss syndrome include the following:

• Complete blood count (CBC)
• Peripheral blood smear
• Blood tests for
  • certain infections, including HIV, Aspergillus, and toxocariasis;
  • tryptase;
  • vitamin B12 levels;
  • erythrocyte sedimentation rate (ESR);
  • C-reactive protein (CRP) levels;
  • blood urea nitrogen (BUN) and serum creatinine levels;
  • IgE levels;
  • eosinophil cationic protein (ECP);
  • soluble interleukin-2 receptor (sIL-2R); and
  • soluble thrombomodulin (sTM).
• Antineutrophil cytoplasmic antibody (ANCA)
• Chest X-ray
• CT scan
• Biopsy of an affected tissue, such as lung, skin, or nerve
• Bronchoalveolar lavage (BAL)
• Gastrointestinal endoscopy
• Urinalysis
• Electromyelography (EMG) and nerve conduction studies
• Electrocardiogram (ECG) for cardiac manifestations

Treatment for Churg-Strauss syndrome includes the following:

• Systemic steroids
• Rituximab
• Cyclophosphamide followed by oral mycophenolate, azathioprine, or methotrexate for maintenance therapy
• Mepolizumab
• Plasma exchange therapy may be indicated in some cases

Because Churg-Strauss (eosinophilic granulomatosis with polyangiitis syndrome) syndrome can affect every system and organ in the body, there are many potential complications. Some of the most common complications of Churg-Strauss syndrome include the following:

• Nerve damage
• Heart problems
• Breathing or lung problems
• Skin issues
• Kidney problems
• Gastrointestinal dysfunction
• Pregnancy complications
• Fetal death rate may be slightly increased

Churg-Strauss syndrome has three phases:

1. The first phase consists of asthma with associated with allergic rhinitis (runny nose) and sinus infection (sinusitis).
2. The second phase involves lung and gastrointestinal dysfunction due to high levels of eosinophils (a type of white blood cell) in the tissues and organs.
3. The third phase is the vasculitis phase, which usually develops within 3 years of disease onset and can affect any of the body's tissues or organ systems, causing most of the complications.

In the past, Churg-Strauss syndrome was fatal within months of 70%-90%. This means that 5 years following diagnosis, 70%-90% of patients are still alive.

Patients over age 65 have a poorer prognosis. If the vasculitis affects critical organ systems, such as the gastrointestinal system, the heart, kidneys, or the brain, the prognosis is fair to poor and most deaths are due to complications of the vasculitis phase of the disease.