Wegener's granulomatosis

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 4A44.A1

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.

Organ involvement:

  • 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

  • Histories
  • Clinical picture
  • X-ray chest
  • Urine status
  • Laboratory:
    • cANCA (antineutrophil cytoplasmic antibody against proteinase-3)

Vasculitic changes of the small and medium-sized vessels, palisade granulomas.

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