September is Rheumatic Disease Awareness Month, which makes this a good time to learn about three rare rheumatic diseases: Behcet's disease, Churg-Strauss syndrome and Takayasu's arteritis.
Behcet's disease, also called Behcet's syndrome, is a rare disorder that causes blood vessel inflammation throughout your body. The disease may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells. It's likely that genetic and environmental factors play a role.
Symptoms vary from person to person, can come and go or become less severe over time, and can seem unrelated at first.
Signs and symptoms depend on which parts of your body are affected and can include:
There is no cure for Behcet's disease. Treatment involves medications to reduce the signs and symptoms, and prevent serious complications, such as blindness. If you have a mild form, your health care professional can suggest medications to control the pain and inflammation of flare-ups. You may not need medication between flare-ups. For a severe case, your health care professional can prescribe medications to control the disease throughout your body, in addition to medications for flare-ups.
Churg-Strauss syndrome, also called eosinophilic granulomatosis with polyangiitis, is a rare disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them.
The cause of Churg-Strauss syndrome is unknown. It's likely that a combination of genes and environmental factors, such as allergens or certain medications, triggers an overactive immune system response. Instead of protecting against invading bacteria and viruses, the immune system targets healthy tissue, causing widespread inflammation.
Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications. The syndrome tends to occur in three stages and gets progressively worse. Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells called eosinophils.
Other signs and symptoms can include:
There is no cure for Churg-Strauss syndrome. Treatment with steroids and other powerful immunosuppressant drugs can manage symptoms.
Takayasu's arteritis is a rare type of vasculitis, which is a group of disorders that causes blood vessel inflammation. In Takayasu's arteritis, the inflammation damages the aorta, which is the large artery that carries blood from your heart to the rest of your body, and its main branches. The disease can lead to narrowed or blocked arteries, or to weakened artery walls that may bulge, cause an aneurysm and tear. It also can lead to arm or chest pain, high blood pressure, and eventually heart failure or stroke.
The cause of the initial inflammation in Takayasu's arteritis is unknown. The condition is likely an autoimmune disease where your immune system attacks your own arteries. A virus or another infection may trigger this disease.
The signs and symptoms of Takayasu's arteritis often occur in two stages.
In the first stage, signs and symptoms can include:
Not everyone has these early signs and symptoms. It's possible for inflammation to damage arteries for years before you realize something is wrong.
During the second stage, inflammation causes arteries to narrow so less blood and oxygen and fewer nutrients reach your organs and tissues.
Signs and symptoms in the second stage can include:
There is no cure for Takayasu's arteritis. Treatment focuses on controlling inflammation with medications and preventing further damage to your blood vessels. Takayasu's arteritis can be difficult to treat because the disease may remain active, even if your symptoms improve. It's also possible that irreversible damage has already occurred by the time you're diagnosed. On the other hand, if you don't have signs and symptoms or serious complications, you may not need treatment or you may be able to taper and stop treatment if your health care team recommends it.
Connect with others talking about managing Behcet's disease, Churg-Strauss syndrome, Takayasu's arteritis and other rare rheumatic diseases in the Autoimmune Diseases support group on Mayo Clinic Connect, an online patient community moderated by Mayo Clinic.