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.