Churg Strauss Syndrome (Vasculitis) is also called as allergic granulomatosis and allergic angiitis.
Churg Strauss syndrome is a condition characterized by the inflammation of blood vessels, which results in restriction of the flow of the blood to tissues and vital organs of the body. This may also damage vital organs or tissues of the body permanently in few of the cases.
The very common symptom which may directly implicate towards the Churg Strauss syndrome is asthma. Several problems like gastrointestinal bleeding, numbness and severe pain in feet and hands, high fever and rash may also occur in Churg Strauss syndrome. The symptoms of this syndrome often are found to be similar to other diseases which create difficulty in arriving at the diagnosis of the condition.
Treatment for Churg Strauss syndrome is really very difficult as there is no proper cure available to treat the condition. Therefore, its treatment highly concentrates on reducing the symptoms with the help of powerful immunosuppressant drugs and steroids.
Epidemiology of Churg Strauss Syndrome or Allergic Angiitis
Approximately 2.5 cases per 100,000 adults are recorded per year. Churg Strauss syndrome is 2% more than all vasculitic diseases recorded in the United States. Churg Strauss syndrome is estimated to affect 15 to 25 persons in North America every year in a population of a million. Males are more prone to Churg Strauss syndrome when compared to females. The estimated ratio in males and females is about 1.4:1.
Causes and Risk Factors of Churg Strauss Syndrome or Allergic Angiitis
- The exact cause of this syndrome is still not known, but the suspected cause may implicate towards autoimmune disorders, which are usually responsible for forcing the immune system of the body to attack its own healthy tissues leading to inflammation.
- Generally, the immune system of the body acts as a shield in order to protect the cells from the attacks of foreign bodies like viruses and bacteria. In case of this syndrome, the immune system starts producing autoimmune antibodies that attack the healthy tissues of the body itself.
- Changing the modality of medication from low-dose oral systemic steroids to inhaled steroid medications such as for allergy and asthma medication could also be a probable reason for the cause of this disorder.
- Churg Strauss syndrome affects people in the age group of 38 to 52 years. Churg Strauss syndrome is rarely seen in children and adults of older age.
- Having history of asthma, nasal allergies and chronic sinusitis could also be responsible for Churg Strauss syndrome.
Signs and Symptoms of Churg Strauss Syndrome or Allergic Angiitis
- Weight loss.
- Inflammation of nasal passage or sinus in asthma patients.
- Improvement in asthma and intensification of the disease in some other part of the body.
- Shortness of breath, chest pain, and cough could also be noticed due to the lungs being affected by vasculitis.
- Appearance of nodules or skin lumps on the extremities.
- Pain in the stomach and diarrhea is also experienced due to inflammation of blood vessels in the abdomen.
- Inflammation of the prostate gland and urinary bladder.
- Nerve injury due to vasculitis may lead to weakness and numbness of the extremities.
- Seizures or periods of confusion can also be seen if the brain is affected.
Treatment for Churg Strauss Syndrome or Allergic Angiitis
There is no proper cure available for treating Churg Strauss syndrome. Treatment for Churg Strauss syndrome usually concentrates on reducing the symptoms. Starting the treatment as early as possible increases the effectiveness of treatment and decreases the chances of complications. However, relapses are quite common even after resolution of symptoms with drugs.
Although, there is no particular treatment available this disease, given below are few of the treatment modalities which usually help in controlling the symptoms.
Medications Used To Treat Churg Strauss Syndrome or Allergic Angiitis Include As Follows:
- Corticosteroids: Corticosteroids like prednisone are very commonly used for its treatment. Boosting the current dose of corticosteroids may also help to keep the symptoms under control. High doses of corticosteroids if taken for prolonged period carry certain severe side effects such as cataracts, hard-to-treat infections, bone loss and high blood sugar and therefore the dose is gradually tapered down as much as possible. In some patients, low doses could also create problems depending upon their tolerability.
- Other Immunosuppressive Drugs: Immunosuppressants like methotrexate, cyclophosphamide and azathioprine may help in reducing the adverse reactions of the immune system. Immunosuppressive drugs help in impairing the ability of the body to fight against infection. Immunosuppressive drugs carry certain side effects therefore close monitoring during these medications becomes necessary.
- Intravenous Immunoglobulin: Intravenous immunoglobulin is usually used when other medications fail to work satisfactorily. Intravenous immunoglobulin is given as an infusion through a vein. Intravenous immunoglobulin may cause side effects such as flu-like symptoms, which are experienced for a day or two.
Investigations for Churg Strauss Syndrome or Allergic Angiitis
Elevated serum creatinine, elevated ESR and CRP, anemia and eosinophilia being detected on FBC, increased serum IgE levels, hypergammaglobulinemia, proteinuria, microscopic hematuria and red blood cell casts in the urine may all be the indications of this disease. Given below are few other tests that may help in its diagnosis.
- Antineutrophil cytoplasmic antibodies (ANCA).
- Chest x-ray.
- Pulmonary CT scan.
- Other investigations may be indicated depending upon the complications of the disease involving any other specific organ.