Churg-Strauss syndrome (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Eosinophilic granulomatosis with polyangiitis previously called Churg-Strauss syndrome involves primarily small and medium sized blood vessels resulting in vessel inflammation. The exact cause of the disease is not known. However, various environmental factors, allergens, genetics, and drugs may play a role in triggering disease process by activating different types of white blood cells. The disease is characterized by the presence of asthma, peripheral eosinophilia, rhinosinusitis, tingling and numbness of hands and feet (peripheral neuropathy) and multiple organ involvements including skin, GI tract, and kidney.

What are the symptoms of Churg-Strauss syndrome

Symptoms of eosinophilic granulomatosis with polyangiitis typically develops through three phases, include prodromal phase, eosinophilic phase, and vasculitis phase. Although these phases cannot be clinically distinguishable.

• Prodromal phase: Most common manifestations include
  • Runny nose
  • Sneezing
  • Nasal polyps
  • Sinusitis
  • Asthma
  • Ear pain and discharge
• Eosinophilic phase: This phase is characterized by eosinophilia and accumulation of eosinophils in various organs. Most commonly involved organs are lung and GI tract.
  • Asthma
  • Cough
  • Shortness of breath
  • Abdominal pain
  • GI bleeding
  • Fatigue
• Vasculitic phase:  Symptoms depending on organ system involvement. Most commonly involved organs are lungs, kidneys, skin, heart, and peripheral nerves.
  • Weight loss
  • Generalized weakness
  • Fatigue
  • Skin rash and nodules
  • Numbness and tingling sensation of hands and feet
  • Abdominal pain
  • Diarrhea
  • Nausea and vomiting
  • Shortness of breath
  • Hearing loss
  • Chest pain
  • Blood in urine

What causes Churg-Strauss syndrome?

There are no established causes for eosinophilic granulomatosis with polyangiitis. However, various allergens, infections, vaccinations and drugs may act as a triggering agents, and are responsible for developing disease. Genetics may also play a role.

When to seek urgent medical care?

You should see the doctor if you experience any of the following:

• Worsening asthma
• Severe breathing difficulties
• Worsening sinusitis, facial pain and rhinitis
• Severe abdominal pain
• Blood in urine
• severe urticarial rash

The above symptoms may also present in association with other diseases, but they have to be evaluated properly by medical professional.

Treatment Options

• Your doctor may suggest treatment with glucocorticoids (eg, predisone) and immunosuppressants that slow down the immune response.
• Medicines used to treat eosinophilic granulomatosis with polyangiitis include:
  • Prednisone
  • Cyclophosphamide
  • Azathioprine
  • Methotrexate
  • Rituximab
  • Interferon alpha
• These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor. Other medicines may be prescribed, including:
  • Bisphosphonate: Prevent bone loss caused by prednisone
  • Folic acid: If you are taking methotrexate
  • Trimethoprim/sulfamethoxazole: Lung infections
• Some patients may be considered for surgical therapy to relieve symptoms of persistent sinusitis and hearing loss.

Prevention

Currently, there are no established preventive measures for eosinophilic granulomatosis with polyangiitis.

What to expect (Outlook/Prognosis)?

• Without treatment, people with eosinophilic granulomatosis with polyangiitis can die within a few months.

• With treatment, the outlook for most patients is good.
• Studies have shown that most people who receive and cyclophosphamide are doing much better. However, the disease relapses are common.

Possible Complications

Without treatment, eosinophilic granulomatosis with polyangiitis can result in the following complications. Complications depend on organ system involvement.

• Heart:
  • Pericarditis
  • Myocarditis
  • Pericardial effusion
  • Myocardial infarction
  • Congestive heart failure
• Lung:
  • Pleural effusion
  • Pulmonary hypertension
  • Status asthmaticus
• Kidney:
  • Glomerulonephritis
  • Renal failure
• GI tract:
  • Gastrointestinal bleeding
  • Acute abdominal pain or intestinal angina
• Neurologic:
  • peripheral neuropathy
  • Cerebral hemorrhage
• Skin:
  • Purpura
  • Petichiae
  • Noduluar lesions on extensor surfaces