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ANCA-associated vasculitis

Understanding causes

ANCA-associated vasculitis are vascular inflammations characterized by autoimmunity and anti-neutrophilic cytoplasmic antibodies (ANCA). Although the ANCA-associated vasculitides define ANCA, their role in the development of these vascular inflammations is not fully understood.  

ANCA-associated vasculitides are systemic diseases that affect almost all organ systems and can have severe clinical consequences. Three partly quite different diseases are classified as ANCA-associated vasculitis:

  • Eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome) is characterised by an asthmatic component and a vasculitic component. The asthmatic symptoms are often difficult to control, especially compared to seasonal bronchial asthma. The vasculitic changes mainly affect the skin, nervous system, heart and other organs.

  • Granulomatosis with polyangiitis (GPA, former Wegener's disease) often affects the respiratory tract and the kidneys. Especially the changes in the upper respiratory tract (nose, paranasal sinuses, larynx) can be characteristic for the GPA.

  • Microscopic polyangiitis (MPA) is closely related to GPA. The main distinguishing criterion is granuloma (a special inflammatory formation), which is not observed in MPA. In addition, the infestation of the upper respiratory tract is rather rare in comparison to GPA, whereas disease symptoms occur in the kidneys and lungs.

Facts and figures around the disease

  • Estimates of the incidence of ANCA-associated vasculitis vary widely by entity (complexity of symptoms). The GPA is the most common ANCA-associated vasculitis in Western Europe, with 6-12 new cases per million people per year.

  • MPA and EGPA, on the other hand, are less common, with only 0-3 new cases per million people per year.

  • The average age at which the disease occurs is between 60 and 70 years, even if the disease can occur at a very young age.

  • The life expectancy of patients with ANCA-associated vasculitis has continuously improved since the introduction of immunosuppressive therapies, so that today the quality of life has improved as a result of adequate therapies and the disease no longer leads so frequently to a shortened life expectancy.

  • Men and women are equally affected.

Typical symptoms and course of the disease

The ANCA-associated vasculitides, in particular the GPA and EGPA, are characterized by a stage-like course.

Initial stage

During the initial phase, symptoms and disease-related changes are often localised and can be very similar for the different vasculitis types. While asthmatic complaints may indicate EGPA, other symptoms such as fever, night sweats, weight loss, joint pain, cough, nasal or sinusitis are rather unspecific and require further investigation. MPA does not have a classical initial stage.

In the further course

The further course of the disease is very variable. About 5% of patients seem to remain in the localized stage.

Depending on the organ system affected, among other things, this can occur::

  • Visual disturbances, double vision, eye inflammations (red eyes)
  • Borks and crust formation in the nose
  • Salivary gland swellings
  • Shortness of breath
  • Cough, bloody cough
  • Heart stumble
  • Sensory disturbances and tingling ants
  • Joint pain


How does the physician make the diagnosis?

It is important to make a diagnosis at an early stage in order to initiate an adequate therapy in good time and thus curb the progression of the disease. A detailed anamnesis, physical examination, special blood tests and imaging procedures can be groundbreaking here. Very often systemic signs of inflammation can be detected in the blood. Regular check-ups of the individual organs are indispensable in the course of the disease.

How to treat the disease?

The treatment of ANCA-associated vasculitis should be done in specialized centers.

Depending on their severity, vasculitic complications are treated with various immunomodulating drugs. In mild forms, methotrexate and leflunomide may be used in addition to glucocorticoid preparations ("cortisone"). In severe cases, glucocortioid preparations and cyclophosphamide and/or rituximab are used, which can then be converted to the weaker preparations methotrexate, azathioprine and leflunomide along with others to maintain disease control. Cotrimoxazole, an antibiotic, plays a special role. It has a weak but demonstrable effect in maintaining disease control. Due to its efficacy, a possible induction of the disease by pathogens is also repeatedly discussed.

With EGPA, asthmatic complaints are treated virtually independently of vasculitic complaints. Supportive measures and psychosocial support round off the treatment.

Practical tips for everyday life

  • If ANCA-associated vasculitis is suspected, an emergency appointment with a rheumatologist is necessary.  
  • Long-term care of patients with ANCA-associated vasculitis should be carried out at larger centres, as the co-care of other specialist disciplines may often be necessary.