What kind of hypersensitivity is Wegener's granulomatosis

Vasculitis (blood vessel rheumatism)

Vasculitis (blood vessel rheumatism) is a rare rheumatic disease and is understood as an inflammation of the blood vessels. According to the German Rheuma League, around 16,500 Germans suffer from vasculitis. It affects people of all ages, women and men equally often.

What is the cause of blood vessel rheumatism?

The immune system has the task of fending off viruses and bacteria. To ensure this, the immune cells have to identify within a very short time whether it is an endogenous cell, a bacterium or a virus. All rheumatic diseases have in common that errors occur in identification and defense. The body's own structures are mistaken for bacteria and attacked by the immune system.

Such incorrect reactions can also attack the walls of blood vessels. Since every human organ is supplied by blood vessels, almost every organ can be damaged as part of such inflammations.

Which blood vessels are affected?

Vasculitis is classified based on the size of the blood vessels involved. When microscopic blood vessels become inflamed, one speaks of Small vessel vasculitis. This means capillaries, arterioles and venules that are not visible to the naked eye. The inflammation of large blood vessels, such as the main artery (aorta), the carotid artery or the temporal artery, is referred to as Large vessel vasculitis.

There are a number of different clinical pictures that are precisely assigned by rheumatologists. A typical small vessel vasculitis is that Granulomatosis with polyangiitis (Wegener's granulomatosis). A typical large vessel vasculitis is Giant cell arteritis (temporal arteritis).

How is the diagnosis made?

The diagnosis of vasculitis is difficult because the courses of the diseases are very different and almost every organ in the human body can be affected.

If a rheumatologist suspects small vessel vasculitis, he will try to obtain a tissue sample from the affected organ. The inflamed blood vessels are so small that they can only be assessed under a microscope.

If the rheumatologist suspects large vessel vasculitis, he will examine the affected blood vessels with ultrasound, magnetic resonance imaging (MRI) or other special procedures.

What complaints do you have?

Every human organ is supplied by blood vessels. If these become inflamed, this can have consequences for the function of the organ. The rheumatologist must thoroughly examine each organ to see if there is any inflammation of the blood vessels in the organ. Vasculitis can cause a variety of conditions:

 

  • General symptoms
    If the diagnosis of vasculitis is confirmed, fever, fatigue, and weight loss may indicate inflammatory activity and a generalized stage.
  • ENT tract, upper respiratory tract
    Inflammation of the blood vessels in the nasopharynx can lead to damage to the nasal septum, tympanic effusions, destruction of the nasal cartilage ("saddle nose") and chronic inflammation of the windpipe. Chronic bloody runny nose and chronic sinus infections can be the result.
  • lung
    An immunologically mediated inflammation of the capillaries of the lungs leads to bleeding into the lungs. An increase in the number of connective tissue fibers increases the stiffness of the lungs (pulmonary fibrosis), which impairs respiratory function.
  • kidney
    Inflammation of the kidney calyx (glomerulonephritis) is one of the most threatening manifestations of small vessel vasculitis and causes deterioration in kidney function.
  • Peripheral nerves
    Peripheral nerves are often affected, and the inflammation can lead to nerve failure. Peripheral nerve failures can develop dramatically within a very short time (“overnight”).
  • eye
    Inflammation of the outer choroid (scleritis) and the middle skin of the eye (uveitis) are possible manifestations of small-vessel vasculitis. Inadequate treatment of both clinical manifestations can lead to deterioration in vision.
  • heart
    Involvement of the heart in the form of muscle inflammation (myocarditis) or pericarditis (pericarditis) is often decisive for the prognosis of the overall course of the disease. It should be borne in mind that patients with vasculitis have an increased risk profile for coronary artery occlusion (coronary artery disease).
  • Joints / muscles
    Muscle pain, joint pain and joint inflammation occur with all vasculitides, but are usually less dangerous than with rheumatic joint diseases.
  • Central nervous system (brain, meninges)
    Involvement of the cerebral nerves or the meninges (meningitis) can be associated with very serious clinical courses, but is rather rare compared to the other organ manifestations. The symptoms can resemble a stroke.
  • skin
    Inflammation of the small blood vessels in the skin is very common. Many red spots a few millimeters in size (petechiae) on the lower legs are typical. Particularly severe inflammation can lead to chronic wounds.
  • Stomach and intestines
    Inflammatory activity of gastrointestinal vasculitis is found in some small vessel vasculitis. It should be borne in mind that problems in the stomach and intestines can also occur in the context of adverse drug effects and must be differentiated from inflammation.
  • bone
    Osteoporosis is a common disease that is important for the rehabilitation process in patients with vasculitis. This is due to the disease activity itself, long-term cortisone administration and disease-related immobility.
  • Mental comorbidity
    Vasculitis is a potentially life-threatening disease that often recurs, resulting in depression and anxiety disorders. In addition, psychological manifestations are possible as adverse drug effects from high-dose cortisone.

How is therapy carried out in rehabilitation?

As part of inpatient rehabilitation, patients are treated with a variety of therapies that complement each other and are coordinated:

  • Information and training
    It is known that patients who have a good knowledge of their diseases are much better able to deal with disease symptoms. The information is conveyed through training courses, books and information brochures. The exchange between the patients is also important. Since the diseases are very rare overall, it makes sense to concentrate patients from all over Germany at a few specialized centers.
  • Exercise therapy / sports therapy
    Exercise therapy (Nordic walking, water aerobics, functional training, ergometer therapy) is not just about improving the mobility of the joints. Rather, targeted applications can reduce inflammatory reactions.
  • nutrition
    As part of rheumatological rehabilitation, patients receive lectures on nutrition and cook together in the teaching kitchen. Diet can have a positive effect on rheumatic inflammation.
  • job
    The professional situation is a central issue in rehabilitation. It is important to find career prospects again after a serious, possibly life-threatening episode of illness.
  • Motivation to participate in self-help groups
    Participation in a self-help group can help to maintain the successes achieved in rehabilitation in the long term. Due to the rarity of the clinical pictures, it can be difficult to find specific self-help groups on site. One solution could be regional offers from the German Rheumatism League on the subject of vasculitis.
  • Preventing coronary artery disease and stroke
    Due to the chronic inflammatory situation, there is a significantly increased risk profile for non-inflammatory diseases of the coronary arteries and strokes. General aerobic cardiovascular training through exercise therapy is indicated for all forms of vasculitis. Depending on the resilience, Nordic walking, walking, ergometer training and water aerobics can be used. Influenceable cardiovascular risk factors should be reduced. This includes, among other things, targeted smoking cessation, weight reduction in the case of obesity and knowledge of a healthy diet (teaching kitchen, nutritional lectures, individual advice).
  • Improve lung function
    Strengthening the auxiliary breathing muscles through breathing exercises. General aerobic exercise.
  • Preventing osteoporosis with vertebral fractures
    The risk of osteoporosis is significantly increased. All patients with vasculitis should be examined by a bone specialist (osteologist DVO) and the risk of bone fractures should be assessed by him (structured osteological assessment).
  • Stabilization of the psyche
    Group applications are about coping with psychological problems as a result of the serious underlying illness. One-on-one psychological interviews should be conducted by a therapist who has specific knowledge of the clinical picture at hand.
  • Protection of the kidney
    When the inflammation in the kidney has been completely suppressed, it is important to switch off all other potentially kidney-damaging accompanying factors (high blood pressure, medication, etc.).
  • Hand coordination
    Active thermal applications, e.g. hand exercises in warm rapeseed, improve the well-being and fine motor skills of the hands.

Center formation in rehabilitation

Vasculitis is rare, difficult to diagnose, and dangerous. Therefore, patients with vasculitis in acute medicine are treated in specialized centers. This development is also useful for rehabilitation.

A therapy team that has extensive experience in the treatment of these diseases is crucial for establishing a center for the rehabilitation of patients with vasculitis. Alarm signals must be interpreted correctly. Close cooperation between the rehabilitation clinic and an acute medical vasculitis center is essential in the event of acute deterioration. Training courses for the entire therapy team must take place regularly. Therapeutic applications on the level of the body (physiotherapy, occupational therapy and sports therapy) and on the level of the psyche (psychological training) can only be successful if the therapist has detailed knowledge of the disease and its functional disorders.

Conclusion

Vasculitis are diseases with a potentially threatening course that can affect almost any organ system. Rehabilitation should take place in specialized centers in order to ensure the highest possible quality of care.

last changed on: 06/24/2019

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