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:
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::
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.
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.
