Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
Klinger 1931; Wegener 1936
- Granulomatosis with polyangiitis, granulomatosis with polyangiitis and eosinophilia, Wegener-Klinger-Churg syndrome
- Necrotising, granulomatous multisystem vasculitis of the small and medium-sized arteries.
- Incidence: 2-5/ 100 000 per year.
- Most common between 55 and 65 years of age
It is probably a reaction to an infectious antigen. Granulomatous inflammatory reactions occur, causing necrotising vasculitis of the small and medium-sized arteries as well as the veins. The respiratory tract and kidneys can also be affected.
- Stage I (Localised stage): Rhinitis, sinusitis, AZ deterioration (usually only mild).
- Stage II (Early systemic stage): Cough (partly bloody), mucosal ulcers, pulmonary infiltrates, pleurisy. Overall no vital threat.
- Stage III (Generalisation stage): Involvement of the kidney (focal segmental glomerulonephritis), mostly severe systemic signs. Vital threat possible.
- Lung: >90%, pulmonary infiltrates, caverns
- Superior respiratory tract: 90%, gingivitis, laryngitis, saddle nose after tissue destruction, nasal septal perforation
- Kidney involvement (90%); joints (70%); ear (60%) eye (60%
- Skin changes: 50%, e.g. positive pathergia.e.g. positive pathergy phenomenon, leukocytoclastic vasculitis-like changes, haemorragic bullae, PG-like lesions)
- Nervous system: 25%, e.g. mononeuritis multiplex
- Heart: 10%, vasculitis of coronary arteries
- Clinical picture
- X-ray chest
- Urine status
- cANCA (antineutrophil cytoplasmic antibody against proteinase-3)
Vasculitic changes of the small and medium-sized vessels, palisade granulomas.
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