Churg-Strauss syndrome

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Churg-Strauss syndrome, also known as “eosinophilic granulomatosis with polyangiitis” (EGPA), is a rare, serious disease. An excess of certain white blood cells leads to inflammation of the small and medium-sized blood vessels. At first, bronchial asthma and other allergic diseases appear as symptoms. Later, in addition to the lungs, other important organs such as the heart or kidneys are affected by the inflammation. With the right therapy, many sick people lead a normal life. Read more about the causes and treatment options here!

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. M30

Brief overview

Description: Rare, serious illness, initially characterized primarily by allergic diseases such as bronchial asthma, if left untreated, it leads to serious functional disorders of important organs
Causes: Unknown
Prognosis: not curable, but mostly well treatable
Treatment: Treatable with drugs that weaken the excessive reaction of the immune system
Symptoms: asthma, hay fever, nasal polyps, later serious symptoms possible, depending on the organ affected (heart, lungs, kidneys, skin, nerves)
Diagnosis: Based on the symptoms, blood tests, various comprehensive examinations of the affected organs, evidence through tissue samples
Prevention: The Churg-Strauss syndrome cannot be prevented.

What is Churg-Strauss Syndrome?

The Churg-Strauss syndrome is one of the rare diseases. It occurs in about two to four people in a million in Western Europe and the United States. Since 2012, doctors have no longer officially referred to the disease as Churg-Strauss syndrome, Churg-Strauss granulomatosis or Churg-Strauss vasculitis after its discoverers, but as eosinophilic granulomatosis with polyangiitis, or EGPA for short.

Churg-Strauss syndrome is a serious disease in which small and / or medium-sized blood vessels become inflamed. In addition to asthma, those affected have an increase in certain white blood cells - the eosinophils. The syndrome can be treated well with appropriate medication.

The Churg-Strauss syndrome often runs in different phases: First of all, allergic diseases (such as bronchial asthma, allergic runny nose) appear, then certain white blood cells increase, which in turn lead to inflammation of the lungs or digestive organs. Finally, the blood vessels become inflamed, which leads to serious disorders of important organs such as the heart, nerves, kidneys or lungs.

It usually takes some time for doctors to diagnose Churg-Strauss syndrome, an average of ten years. Patients between the ages of 40 and 50 are typically affected. The Churg-Strauss syndrome, like granulomatosis with polyangiitis (GPA), which was previously referred to as Wegener's granulomatosis, is one of the so-called ANCA-associated systemic vasculitides.

What are ANCA-Associated Systemic Vasculitis?

According to the definition, Churg-Strauss syndrome belongs to the group of ANCA-associated systemic vasculitides. This means that it belongs to a group of diseases in which there is vascular inflammation in various organs. The abbreviation ANCA stands for "antineutrophil cytoplasmic antibody" - in English: antineutrophil cytoplasmic antibody. These special antibodies play a role in autoimmune diseases. Autoimmune diseases are diseases in which the immune system attacks the body's own structures (organs, tissues) due to a malfunction.

These ANCA-associated systemic vasculitides include:

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome

What is the cause of Churg-Strauss Syndrome?

The cause of Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis (EGPA) is unknown. Certain genetic factors and asthma are believed to increase the likelihood of developing the disease. Churg-Strauss syndrome can be triggered by infections, allergy-causing substances (allergens) or medication.

Doctors suspect that Churg-Strauss syndrome is an autoimmune disease. This means that there is an excessive reaction of the immune system, with healthy body cells being fought. In a normal immune reaction, the body's own defense is only directed against foreign substances such as pathogens such as bacteria or viruses.

Is there a cure for Churg-Strauss syndrome?

The Churg-Strauss syndrome cannot be cured. With the appropriate therapy, however, symptoms no longer occur in many cases. Without treatment, however, life expectancy decreases and the quality of life is limited by symptoms. If the Churg-Strauss syndrome is treated accordingly in a special clinic, normal life expectancy can be assumed.

Depending on how severe the eosinophilic granulomatosis with polyangiitis (EGPA) is and when the disease is diagnosed, it may attack various organs such as the lungs, digestive organs, heart, nerves or kidneys. In some cases, the drugs that are important for treatment also trigger secondary diseases. The complications that result from this range from diabetes mellitus, obesity, osteoporosis (bone loss) and infertility to an increased risk of bladder cancer. The most common cause of death in Churg-Strauss syndrome is heart disease such as heart failure or myocardial infarction.

How is Churg-Strauss Syndrome treated?

Basically, EGPA is treated with drugs that weaken the excessive reaction of the immune system (immunosuppressants). Doctors usually follow appropriate guidelines for treatment. These are summaries by doctors and for doctors on individual diseases, in which current recommendations for treatment and diagnosis are listed.

Therapy for Churg-Strauss syndrome depends on the severity of the disease and whether organs are affected or not. If this is not the case - the disease is easier - the doctor will prescribe glucocorticoids and methotrexate. Both remedies have a strong effect against inflammatory processes in the body. If organs are affected or if the syndrome is already life-threatening, doctors use other immunosuppressants.

If the disease recedes and symptoms improve, doctors refer to this as remission. If this occurs in Churg-Strauss syndrome (usually after about three to four months of treatment), doctors adjust the medication and its dosage over the next two years. If the condition remains stable, reduce the dose slowly, if possible.

If the symptoms reappear or if the condition worsens, the doctor will adjust the medication accordingly. Whether or not rehab for eosinophilic granulomatosis with polyangiitis (EGPA) supports the recovery must be discussed individually with the attending physician.

The drugs used to treat Churg-Strauss syndrome may lead to complications and secondary diseases. For this reason, it is advisable to keep your doctor's appointments regularly.

What are the symptoms of Churg-Strauss syndrome?

The symptoms that Churg-Strauss syndrome causes depend on the severity of the disease. Eosinophilic granulomatosis with polyangiitis (EGPA) often proceeds in different phases.

Allergic phase of Churg-Strauss syndrome

At the onset of the disease, allergic diseases in particular appear, including:

bronchial asthma
Allergic rhinitis (hay fever)
Allergic sinusitis (allergic inflammation of the sinuses)
Polyps in the nose

This phase usually lasts for several years.

Eosinophilic phase of Churg-Strauss syndrome

In the eosinophilic phase of Churg-Strauss syndrome, the so-called eosinophils rise massively in the blood. Eosinophils or eosinophils are white blood cells and indicate allergies. In this phase, these may also be deposited in the tissue and lead to inflammation there, for example in the lungs or in the digestive organs. The symptoms are very different and range from a dry cough or shortness of breath to various gastrointestinal complaints and general symptoms such as unwanted weight loss, fatigue or night sweats.

Vasculitic phase with organ involvement in Churg-Strauss syndrome

In the last phase of Churg-Strauss syndrome, the small and / or medium-sized blood vessels become inflamed and lead to serious disorders of important organs. Depending on which organ is affected, the following, often serious symptoms appear:

Lungs: Often affected, the main symptoms are asthma
Heart: Involved in up to 45 percent of patients in the context of the disease, there are various heart diseases. The most common are inflammation of the pericardium (pericarditis) and various heart muscle diseases (cardiomyopathies).
Kidneys: Affected in up to 26 percent of patients, impaired kidney function or even kidney failure possible (Pauci immune glomerulonephritis)
Skin: About half of the patients show small, dot-shaped hemorrhages (petechiae). Other skin changes such as nodules also occur in about every third person affected.
Nerves: Frequently affected, the inflammation damages the nerves, weakness and pain occur in the arms and / or legs, symptoms of paralysis are possible

How is Churg-Strauss Syndrome diagnosed?

The diagnosis of EGPA is very comprehensive. First, the doctor asks about the symptoms. The focus here is mostly on allergic diseases. He also examines the patient physically. The attending physician may perform neurological examinations to check for nerve damage.

On the basis of various laboratory tests, especially of the blood, but also of the urine, indications of the presence of Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis (EGPA) can be found. The blood tests are mainly laboratory values that provide information about inflammation, as well as specific antigen tests.

A comprehensive check-up is carried out to clarify how far the disease has progressed and whether important organ functions are restricted. The doctor examines the heart, lungs, kidneys, and possibly also the stomach and intestines, and looks for abnormalities or functional disorders.

The heart function is checked by means of an electrocardiogram (EKG) and / or a heart ultrasound. A pulmonary function test and a chest x-ray can tell doctors about the condition of the lungs. Computed tomography (CT) and magnetic resonance imaging (MRI) may also be performed.

If necessary, the doctors do an endoscopy (gastrointestinal mirroring) to examine the stomach and intestines for indications. The doctor confirms the diagnosis of Churg-Strauss syndrome by examining a tissue sample. The tissue sample is taken as part of certain examinations, such as a gastrointestinal mirror. In most cases this examination is painless and the patient is unaware of the removal.