Churg-Strauss Syndrome: What Is, Treatment & How It Affects Organs

What is Churg-Strauss Syndrome?

Churg-Strauss Syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune condition characterized by inflammation of small to medium-sized blood vessels. This inflammation can affect various organs in the body, including the lungs, skin, nerves, and gastrointestinal tract.

The exact cause of Churg-Strauss Syndrome is unknown, but it is believed to involve an abnormal immune response leading to blood vessel inflammation and the accumulation of eosinophils (a type of white blood cell) in tissues.

Symptoms of Churg-Strauss Syndrome can vary widely but may include asthma, sinusitis, fever, weight loss, fatigue, skin rashes, nerve damage, and gastrointestinal problems. The condition can progress rapidly and may be life-threatening if not treated promptly.

Treatment typically involves medications to suppress the immune system and reduce inflammation, such as corticosteroids and immunosuppressants. In some cases, additional therapies such as plasmapheresis (a procedure to remove harmful antibodies from the blood) may be recommended.

Early diagnosis and treatment are essential in managing Churg-Strauss Syndrome and preventing complications. Patients with this condition often require ongoing medical care and monitoring to manage symptoms and prevent relapses.

Treatment of Churg-Strauss Syndrome

Churg-Strauss syndrome causes inflammation all over the body. Because this disease affects so many organs, you may need to see a few specialists to treat it.

Churg-Strauss syndrome is also called eosinophilic granulomatosis with polyangiitis, or EGPA. There’s no cure, but steroids and other medications can help control symptoms.

These medicines quickly bring down swelling and stop your immune system — your body’s defense against germs — from attacking your organs and tissues. Which type of medication you get depends on whether your disease is mild or severe.

Glucocorticoids

Glucocorticoids — a type of “steroid” — are man-made versions of hormones your body makes. Prednisone and other steroid drugs bring down inflammation in your body quickly.

Your doctor will likely start you on a high-dose prednisone pill — 40 to 60 milligrams a day. This medicine can put your disease into remission, which is when you no longer have any symptoms or signs of Churg-Strauss syndrome.

If your symptoms are severe or steroid pills don’t improve them, you may get very high doses of steroids through a vein (IV). Nasal and inhaled steroids are helpful for sinus problems and asthma symptoms.

Your doctor will monitor your disease with regular lab tests and checkups. Once your symptoms have improved, you’ll taper off the steroids. Your doctor will lower your dose slowly over a few months to ease your body off the medicine.

Steroid drugs can cause side effects such as:

• Weak bones
• Weight gain
• High blood sugar
• Cataracts
• Greater chance of infections

To help prevent these side effects, your doctor will give you the lowest steroid dose possible to control your symptoms.

Glucocorticoid Sparing Therapy

If a steroid alone doesn’t control your symptoms, you may need to take medications known as “glucocorticoid sparing therapy” along with it. Like steroids, these medications are “immune-suppressing drugs.” Also called immunosuppressants, they calm your immune system to stop it from making chemicals that damage your organs and tissues.

You may get one of these drugs along with a steroid:

• Azathioprine (Azasan, Imuran)
• Cyclophosphamide
• Methotrexate (Trexall)
• Mycophenolate mofetil (CellCept)
• Rituximab (Rituxan)

These medicines can make it harder for your body to fight infections. Your doctor will keep close tabs on you while you take them.

Mepolizumab (Nucala)

In 2017, the FDA approved the first drug specifically to treat Churg-Strauss syndrome. Mepolizumab (Nucala) is a biologic drug. It targets the parts of your immune system that cause inflammation. It’s also approved to treat eosinophilic asthma, a severe form of asthma that’s linked to Churg-Strauss.

Mepolizumab lowers the number of infection-fighting white blood cells called eosinophils in the body. People with Churg-Strauss syndrome make too many eosinophils, which cause inflammation and damage.

Mepolizumab may cut down your need for steroid drugs and help you stay in remission longer.

The medicine comes as a shot that a doctor gives you in your arm, thigh, or belly once a month.

Mepolizumab can cause side effects like:

• Headache
• Back pain
• Tiredness

Immune Globulin

In Churg-Strauss syndrome, your immune system makes chemicals that damage your organs and tissues. Immune globulin prevents this immune attack. You might get this treatment if steroids and other medicines haven’t controlled your symptoms.

You get immune globulin through an IV. The treatment can have flu-like side effects that include:

• Fever
• Chills
• Flushed skin
• Muscle and joint pain
• Tiredness
• Nausea and vomiting

Who Treats Churg-Strauss Syndrome

Depending on the symptoms you get, you may need a variety of doctors on your medical team, such as:

• Rheumatologist, who treats autoimmune diseases like Churg-Strauss syndrome
• Cardiologist, who treats diseases of the heart and blood vessels
• Allergist, immunologist, or pulmonologist, who treat asthma and allergies
• Otolaryngologist, or ear-nose-throat (ENT) specialist, who treats problems with the sinuses
• Dermatologist, who treats rashes, bumps, and other skin problems
• Gastroenterologist, who treats problems with the digestive system, which includes the stomach and intestines
• Neurologist, who treats problems with the brain and spinal cord, including nerve problems

What to Expect

Churg-Strauss syndrome used to be hard to treat. But today, steroids and other immune-suppressing medicines can help you live longer, and feel better.

The goal in treating Churg-Strauss syndrome is to put your disease into remission. You may need to take steroids or other medicines for a year or more before your symptoms get better. How long your treatment lasts depends on how severe your disease is, and how quickly it improves with treatment.

How Churg-Strauss Syndrome Affects Organs

Churg-Strauss syndrome is a rare disease that causes inflammation of small and medium-sized blood vessels. This inflammation can slow the flow of blood to your organs.

Another name for Churg-Strauss syndrome is eosinophilic granulomatosis with polyangiitis (EGPA). If you have it, you may have high numbers of white blood cells called eosinophils, which normally help your immune system fight germs. But in this case, eosinophils collect and form lumps called granulomas in different parts of your body.

Which organs Churg-Strauss syndrome affects may be different for each person.

Heart

Swelling in the blood vessels of the heart is called vasculitis. Sometimes granulomas form inside the heart, which can lead to a few different types of heart problems.

The membrane around the heart (pericardium) may swell, a problem known as pericarditis. The pericardium protects your heart and prevents it from expanding too much. Pericarditis affects the heart’s rhythm and how well it works. Sometimes it can cause serious problems with the heart.

The muscular wall around the heart itself can also swell. When the heart swells, it may not be able to pump as well as it should. Sometimes it pumps too quickly or out of its normal rhythm.

Some people with Churg-Strauss syndrome can have a heart attack from a blockage in blood flow to their heart.

Your heart muscle needs a steady flow of blood to work right. The heart may get so damaged that it can’t pump well enough to send out enough blood to meet your body’s needs. This is called heart failure.

Lungs

The lungs are one of the most common organs this disease affects. Almost everyone with Churg-Strauss syndrome has asthma — a swelling of the airways in their lungs. The swelling narrows the airways and makes it hard to breathe. Asthma also causes symptoms like wheezing and coughing.

The type of asthma people with Churg-Strauss syndrome get is eosinophilic asthma. It’s a more severe form that happens when the extra eosinophils that your body makes build up in the lungs and cause them to swell.

Kidneys

Your kidneys filter waste out of your blood to make urine. Tiny clusters of blood vessels called glomeruli filter the blood as it flows through the kidneys.

When you have Churg-Strauss syndrome for a long time, it can damage these tiny filters, leading to a condition called glomerulonephritis.

Damaged kidneys can’t remove as much waste and fluids, which then build up in your blood. Rarely, the kidneys can fail completely and stop filtering.

Nose and Sinuses

Churg-Strauss syndrome can cause swelling in the spaces behind your nose, called sinusitis. You may notice pain around your eyes and cheeks, a stuffed nose, and thick discharge from your nose.

Some people have polyps — painless growths inside the nose. Polyps aren’t dangerous, but they can eventually block your nasal passages and make it hard for you to breathe.

Digestive System

Up to half of people with Churg-Strauss syndrome have problems with their digestive tract. You might get colitis, which is inflammation in the inner lining of the colon — the bottom part of the large intestine. Belly pain is one possible symptom.

Skin

Churg-Strauss syndrome affects the skin in about two out of every three people who have it. Eosinophils normally help your body fight infections, but when you have too many of them, they can build up in the blood vessels under your skin.

Sometimes the blood vessels leak blood, which forms purple-colored spots called purpura on your skin. Some people get a rash. Others have bumps on their elbows or other places. Sores may form on the skin. When those sores heal, they can leave behind scars.

Nerves

Inflammation also damages nerves. Because nerves deliver feeling to your body, damage to them can cause numbness, pain, tingling, or burning, especially in your hands and feet.