Wegener's granulomatosis

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.

• Cyclophosphamide 2 mg / kg bw daily until remission and then for at least 1 year. Tapering off with a reduction of 25 mg every 2 months, provided there is no new exacerbation.
  There should always be bladder protection (with e.g. Mesna) for prophylaxis of bladder carcinoma.
• Methylprednisolone 1 mg/kg bw tgl, slowly taper off in the course.
• Ciclosporin A 7-10 mg/kg bw daily.
• Methotrexate 15-25 mg s.c. 1x weekly
• Azathioprine 100-150 mg daily p.o.
• Plasmapheresis
• Intravenous immunoglobulins (IVIG) i.v. 30 g over 5 days.

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