Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) is an autoimmune disease, i.e. an inflammatory disease in which the immune system attacks the body's own cells. It is a complex disease affecting many organs and systems, and in some cases with serious consequences.

Eosinophilic granulomatosis with polyangiitis( Churg-Strauss syndrome ) is a rheumatic disease, and its causes - like many other diseases this group - are not exactly known. Perhaps the genetic predisposition and genes responsible for the functioning of the immune system play a role here, but it has not been found that sick people are at a higher risk of having sick children.

The main mechanism behind the appearance of eosinophilic granulomatosis is inflammation, sometimes leading to necrosis - typically in small arteries and veins, hence the last part of the disease name. The blood vessels of all organs can be affected, which is why this disease is so dangerous and has so many different symptoms.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - the essence of the disease

As the name implies, its important element is the appearance of an excessive amount of eosinophils (eosinophils). These cells are an essential part of the immune system and are the main line of defense against parasites and are involved in allergic reactions. However, in the case of Churg-Strauss syndrome, too many eosinophils do not imply the presence of parasitic infections or allergies - conditions in which we usually deal with an increased number of them. In this case, for unknown reasons, they multiply excessively and are involved in the ongoing inflammatory reaction that leads to the appearance of granulomas, i.e. specific histopathological changes. They are recognized under the microscope, and the image shows mainly various types of cells of the immune system, with the predominant eosinophils mentioned above.

The incidence of eosinophilic granulomatosis with polyangiitis is approximately 3-38 patients per million inhabitants, so it is quite a rare disease. Its complications can have serious consequences. It is worth mentioning,that this condition has many names, including allergic granulomatous vasculitis, allergic granulomatosis, Churga-Strauss syndrome, CSS, and CS. Their multiplicity corresponds to the variety of symptoms, and the term "allergic" does not imply a mechanism of disease development. It is used because of the current eosinophilia - typical of allergic diseases.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - symptoms

Eosinophilic granulomatosis with polyangiitis has a long-term course and its picture changes over time.

1. The first phase of the disease

The disease begins with the prodromal period, i.e. the initial symptoms - most often in people aged 20-40. The symptoms related to the respiratory system and skin changes are then dominant:

  • hives, changes that look like nettle burns, which are an allergic reaction
  • ulcerative papules
  • itchy skin

However, in this period more typical than skin symptoms are respiratory ailments, which usually occur around the age of 30:

  • allergic rhinitis, i.e. stuffy nose and a runny nose not related to infection
  • polyps in the nose
  • asthma

These symptoms, although all related to sensitization, are not related to the allergen, but to excess eosinophils.

2. The second phase of the disease

The second phase of the disease is the so-called eosinophilic phase. There is an increased amount of them in the blood. Symptoms from the prodromal period persist and, in addition, they add:

  • stomach pains
  • diarrhea
  • cough
  • sometimes hemoptysis

3. The third phase of the disease

The last, third, phase occurs many years after the development of the disease, on average after 3 years, but may appear even after 30 years. This is the proper period of the disease, i.e. the vasculitis phase, and the present symptoms apply to all systems. Most often they do not occur all at once, but rather their combinations of varying severity appear - several symptoms at once or occurring one after another. There are also oligosymptomatic forms, when the symptoms are of low intensity.

The most common symptoms relate to the respiratory system: symptoms persist: asthma (dyspnoea, wheezing), coughing and hemoptysis, moreover, similarly to the initial phase, stuffy and runny nose and polyps in the nose. Occasionally, sinusitis may appear as a symptom of a headache. Slightly less frequent are skin changes: painful lumps - mainly on the elbows, backs of the hands with reddening and sometimes ulcerations, and numerous painful minor hemorrhages under the skin, calledpurpura.

Other systems are not often occupied, but their dysfunctions are the most common cause of death from this disease.

In the case of the cardiovascular system, there is inflammation of the heart muscle, disturbed heart rhyme, the feeling of its rapid or irregular beating, fluid in the pericardium, which significantly disturbs the proper functioning of the heart. Eosinophilic granulomatosis with polyangiitis can eventually lead to heart failure and damage to the coronary arteries, causing a heart attack. It is these diseases that are the most common cause of death.

The nervous system is often involved. Usually it is damage to peripheral nerves, i.e. neuropathy, manifested by disturbances in sensation, shooting pain, tingling, feeling of excessive heat or cold, and it affects two or only one nerves symmetrically.

Involvement of the kidneys is not very common. As a result of renal vascular disease, hypertension, proteinuria, haematuria and glomerulonephritis develop (with histopathological features of necrosis or so-called crescents). Ultimately, eosinophilic granulomatosis leads to renal failure, a common cause of death.

In the case of the gastrointestinal tract, conditions such as gastroduodenitis, as well as intestinal necrosis and perforation, come to the fore. Abdominal pain, diarrhea and bleeding appear.

Rarely, the disease may be accompanied by joint and muscle pain.

General symptoms, such as worse well-being, fever, weakness, weight loss, may occur at any time of the disease.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - diagnosis

Eosinophilic granulomatosis with polyangiitis is a rare disease and its diagnosis is considered only after excluding other causes of the symptoms described, especially since the symptoms are very uncharacteristic and may accompany many other diseases.

Among the auxiliary tests, the basic one is blood count with a smear - eosinophilia and sometimes anemia are observed here. In addition, other tests may show an increase in ESR and CRP, and in urine tests - proteinuria, hematuria, indicative of kidney involvement and impairment of their work.

A more specialized test is the ANCA antibodies - they are present in the blood of approx. 60 percent. sick.

Diagnostics of the respiratory system mainly includes X-ray and computed tomography. They allow to visualize the paranasal sinuses and lungs, and thus to identify sinusitis or alveolar bleeding, which is manifested byhemoptysis.

The most important thing in making a proper diagnosis is the histopathological examination, during which the doctor can see vasculitis and the aforementioned granulomas with the presence of eosinophils. To obtain material for this test, a small piece of tissue should be collected - it may be skin, muscle, nasal mucosa or kidney. The physician decides which organ will be, depending on the predominant symptoms.

Other tests, more specific to other organs, may also be useful in diagnostics, and their performance is decided depending on the clinical picture.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - treatment

Unfortunately, it is not possible to completely cure eosinophilic granulomatosis with polyangiitis. However, complete, permanent remission can be achieved, i.e. inhibition of disease progression and activity, and elimination of its symptoms. However, this cannot be achieved in all patients. If the disease is severe, the effects of treatment may not be satisfactory. Most often, you need to take medication throughout your life, and monitor your he alth frequently to detect and treat any organ damage early. The mainstay of treatment is suppression of the immune system, i.e. immunosuppression. The drugs of first choice are glucocorticosteroids, if they do not bring remission, cyclophosphamide is also used. In addition, depending on the symptoms of other systems, more specific drugs or treatment methods can be used to ensure the proper functioning of the body as long as possible.

Category:

Immune system