Allergic angiitis, also called as Churg-Stauss syndrome, is a rare disorder that causes inflammation of the blood vessels and restricts the blood flow to vital organs and tissues. It affects the nose, sinuses, lungs, heart, intestines, and nerves. In some patients, it may also affect the kidneys, muscles, and joints. About 11 to13 people per million are diagnosed with this rare disorder.
Allergic angiitis causes a variety of nonspecific clinical signs and symptoms. Some patients have only mild symptoms, whereas others may experience severe, life-threatening complications. The disease develops into three different stages characterized by different signs and symptoms. However, not everyone develops all the three phases of disease in the same order.
In this stage, the patient may experience different allergic reactions like asthma, allergic rhinitis (affecting mucous membrane of the nose), and sinus pain and inflammation (sinusitis).
In this stage, abnormally high levels of eosinophils (a type of white blood cells) are found in the blood, affecting the immune system. This condition is called hypereosinophilia and causes fever, weight loss, asthma, loss of appetite, abdominal pain, and gastrointestinal bleeding.
The severe inflammation of blood vessels (vasculitis) occurs at this stage. The blood vessels are narrowed resulting in poor blood flow to different organs and tissues. Most of the body parts are affected including kidneys and muscles. You may feel unwell and have unintended weight loss, swollen lymph nodes, weakness, and fatigue.
Depending the organs affected, you may experience rash or skin sores, joint aches and swelling, peripheral neuropathy, shortness of breath, chest pain, irregular heartbeat, blood in the urine.
The exact cause of the disease is still not known. Few experts think that “allergies” play a significant role in causing the disease. Some people developed allergic angiitis after using an allergic medication called “montelukast” or after switching from systemic oral steroids to inhaled steroid medications. However, there is no proper evidence to prove this.
Allergic angiitis is the rare syndrome affecting not more than three people per million. The possible risk factors associated with the disease are:
Age: Most of the people are diagnosed with the disease at the age of 40s.
History of nasal problems: Maximum number of people diagnosed with allergic to angiitis have a history of nasal allergies, sinusitis or asthma which is difficult to control.
Genes: A recent study has proved that HLA–DRB4 gene is the genetic risk factor for allergic angiitis.
It is very important to know that almost everyone diagnosed with allergic angiitis have asthma, but not all asthma patients develop the disease.
The complications of the disease depend on the organs involved such as lungs, skin, gastrointestinal system, joints, and heart. Cardiac and neurological complications are very serious and are mainly observed in patients with a delayed diagnosis.
The signs and symptoms of the disease are similar to other health conditions, and hence diagnosis of allergic angiitis is difficult. The doctor will check your medical history, signs and symptoms, especially asthma. The doctor will recommend you to undergo blood test, X-ray, and urine analysis. If the doctor thinks that you have this condition, a biopsy (a small sample tissue) of an affected area is recommended.
In some cases, kidney or lung biopsy is also recommended to confirm the disease.
The goal of the treatment is to limit or prevent damage to healthy tissues. Medications used to treat the disease are:
Corticosteroids such as prednisone are the commonly prescribed medications for this condition. In some patients with mild symptoms, corticosteroids alone may not be helpful and hence the physicians prescribe chemotherapy medications such as azathioprine, cyclophosphamide or methotrexate.
Immunoglobulin is recommended to patients who have not responded to other treatment options. Once the disease is under control, the doctor may reduce the dose of the drugs and eventually stop it. This may take at least six months or upto several years, depending on the patient’s condition.
Long-term treatment with medications may cause several side effects, but you can manage it efficiently by making certain lifestyle changes.
