Churg-Strauss Syndrome

Churg-Strauss syndrome is an extremely rare disease that causes inflammation of your blood vessels. It can damage small and medium-sized blood vessels.

It most often affects your nose, sinuses, lungs, heart, intestines, and nerves. In about one-half to two-thirds of people with the disease, inflammation also may affect the kidneys, muscles, or joints.

Doctors also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis.

Stages of Churg-Strauss

There are three. They don’t always happen in sequence, and you may not get all three.

Allergic: Your doctor may also call it prodromal, which means it happens at the start of a disease. It can last anywhere from months to years. You’ll probably notice:

  • Asthma if you don’t already have it
  • Existing asthma worsening
  • Hay fever
  • Inflamed sinuses
  • Nasal polyps

These symptoms usually come first. They might show up 6 months or 2 decades before vasculitis.

Eosinophilic: Your body makes this type of white blood cell in response to an allergy. In this phase, you have too many. They can build up in various body parts, including your lungs, digestive tract, and skin. Symptoms include:

  • Weight loss
  • No appetite
  • Fever
  • Night sweats
  • Joint pain
  • Asthma
  • Fatigue
  • Cough
  • Belly pain

Vasculitic: Inflammation narrows your blood vessels. That means less blood reaches your body. Watch for signs like:

  • Weakness, fatigue, or general bad feeling
  • Weight loss without trying
  • Swollen lymph nodes
  • Skin sores or rashes
  • Achy, swollen joints
  • Pain, numbness, or tingling in your feet and hands
  • Belly pain
  • Diarrhea, nausea, and vomiting
  • Blood in your pee or poop

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Causes and Risk Factors

Doctors don’t know exactly what causes it. Some experts think allergies play a role. But many people with allergies never get it. Others say it’s a mix of genes and things in your environment, like allergens and medications, that send your immune system into overdrive. Instead of protecting you, it ramps up an immune response that causes inflammation throughout your body.

There’s no proven tie to medications. And while it’s rare, some people did get it after they used an asthma medication called montekulast. Others got it when they switched from oral steroids for allergies and asthma to inhaled versions.

Anyone can get it. Things that make it more likely include:

Age: On average, people are between 30 and 50 when they get diagnosed.

Asthma or nasal problems: Most people who get it have chronic asthma, nasal allergies, or chronic sinusitis.

Complications

This disease affects many organs and systems. Over time, it can cause:

  • Nerve damage: It affects your peripheral nervous system, which runs throughout your body. You might get neuropathy -- tingling, burning, or pain -- in your hands and feet.
  • Scars: Sores on your skin can leave a mark.
  • Pericarditis: The membrane around your heart can become inflamed.
  • Myocarditis: Muscles in your heart can become inflamed.
  • Kidney problems: When they can’t filter, waste builds up in your bloodstream.

Diagnosis

Your doctor will discuss your symptoms. There are six specific ones (she’ll call them criteria) she’s looking for to help her make a diagnosis. She might know it’s Churg-Strauss if you only have two or three, but most of the time doctors are looking for four out of the six:

  • Asthma
  • High white blood cell count
  • Nerve damage
  • Spots or lesions on a chest X-ray
  • Sinus trouble
  • White blood cells outside your blood vessels

There’s no single test to diagnose Churg-Strauss syndrome. Your doctor will look at your medical history and give you a physical exam. She’ll ask you about your symptoms, especially asthma.

You may also get:

  • Blood tests: The doctor will look for proteins in your blood that show inflammation. She’ll also check for extra white blood cells.
  • Imaging tests: These could include an X-ray, CT scan, and MRI to check for problems in your lungs and sinuses.
  • Biopsy: Your doctor will take a small tissue sample from an affected spot, like an area of your skin with a rash, to look for inflamed blood vessels. Or she might do a kidney or lung biopsy.

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Medical Treatment

There’s no cure for Churg-Strauss syndrome. But your doctor can help you manage it.

Medications

You’ll need to take drugs that target your immune system. The goal is to limit or prevent damage to healthy tissues. She’ll talk to you about:

Steroids: These are the most common drugs prescribed for this condition. Most people take prednisone (by mouth) and prednisolone (by IV). If your nervous system, heart, kidneys, or intestines aren’t affected, you’ll probably do well with prednisone alone. Once the disease is under control, your doctor will slowly lower the dose. You may be able to stop it entirely.

Immune system drugs: If steroids aren’t enough, your doctor may also prescribe chemotherapy medications, like azathioprine (Imuran), cyclophosphamide (Cytoxan), or methotrexate. These drugs were created to treat cancer, so you’ll probably take a lower dose for Churg-Strauss. The doctor will do blood tests often to check for side effects.Continue Reading Below

You may be able to ease off this medication after your condition is under control. This could take 6 months or several years.

Intravenous immune globulin (IVIG): You might take this protein cocktail if other treatments don’t work. It’s infused into a vein, usually once a month.

Biologic response modifiers (Biologics): These may be your next step in your treatment plan. Unlike medications that target your entire immune system, biologics focus on a specific part of the inflammation process. You usually get it in an injection or an infusion. These drugs can make you more likely to get an infection.

Lifestyle Changes

Long-term steroid use can cause problems. If you take these medications for Churg-Strauss, a few changes can help you manage the side effects:

Keep your bones healthy: Talk to your doctor about vitamin D and calcium. Ask if you get enough in your diet or if you need supplements.

Exercise: Steroids cause weight gain, so it’s important to stay active. Choose aerobic activity like walking or swimming to get your heart going. These medications also weaken bones, so opt for strength training and weight-bearing exercises to keep you strong.

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Stop smoking: It’s bad for your health all by itself. But it can make steroid side effects worse.

Eat healthy food: Steroids can boost your blood sugar and may lead to type 2 diabetes. Opt for foods that keep your sugar stable, like fruits, whole grains, and veggies.

See your doctor: She’ll want to keep an eye on you during treatment and make sure you don’t relapse. You can expect:

  • Bone scans
  • Eye exams
  • Blood pressure readings
  • Blood sugar tests
  • Cholesterol checks

What’s the Outlook?

Most of the time, symptoms will go away with treatment. Your doctor will call this remission. The best results tend to happen when she knows a lot about the condition and starts treatment ASAP.

If the disease has moved to your organs and systems, you’ll have a tougher time than someone with a milder form. But with prompt treatment, even people with the most severe forms of CSV can expect to get better.

WebMD Medical Reference Reviewed by Nayana Ambardekar, MD on April 03, 2018

Sources

SOURCES:

Churg Strauss Syndrome Association.

The Johns Hopkins Vasculitis Center.

Mayo Clinic: "Churg-Strauss syndrome."

Merck Manual: "Eosinophilic Granulomatosis with Polyangiitis (EGPA)."

National Organization for Rare Diseases: “Churg Strauss Syndrome.”

UpToDate: “Patient education: Intravenous immune globulin (IVIG) (Beyond the Basics).”

Arthritis Foundation: “Biologics Overview.”

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