Wegener Granulomatosis (WG):
• predominantly affects small arteries
• granulomatosis arteritis of upper and lower respiratory tracts associated with glomerulonephritis in kidneys and a necrotising vasculitis
• prevalence of around 3-8.5/1 000 000. Most white in 4th or 5th decade. M=F (or maybe M slightly > F).
• strongly associated with autoantibodies against proteinase 3 (a constituent of neutrophil azurophilic granules)
• fever, weight loss, malaise, respiratory symptoms (sinusitis, haemoptysis, chest pain, nasal discharges; nasal deformity), nondestructive painful arthritis, peripheral neuropathy (mononeuritis multiplex and symmetric distal polyneuritis), ulcerative skin lesions, ocular problems
• American College of Rheumatology criteria for diagnosis is when 2 of 4 criteria is present (nasal or oral inflammation; abnormal chest x-ray; red cells in urine; granulomatous lesions on biopsy)
• ROUGH – Chest Radiograph, Oral ulcers, Urinary sediment, Granulomas, Haemoptysis
• Arthritis – in up to 75%; various patterns of joint involvement have been described; usually symmetrical polyarthritis, non-deforming and non-erosive.
• over half of those with WG have been reported to have peripheral neuropathy signs present before the WG was diagnosed (de Groot et al, 2001)
• prognosis is poor due to renal and respiratory problems, but early treatment improves prognosis
• treatment – corticosteroids, cyclophosphamide and immunosuppressives
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