Lessons learnt in the management of Wegener's Granulomatosis: long-term follow-up of 60 patients.

OBJECTIVE: To assess all patients with Wegener's Granulomatosis treated in Nottingham, with particular focus on relapse rate and the useful predictors of relapse. We evaluated how well the findings of nasal examination correlated with disease relapse compared to other parameters such as c-ANCA, ESR and CRP. Presenting features, diagnosis, adverse effects of treatment and mortality rate, were also studied.
DESIGN: Retrospective examination of 60 patient notes, diagnosed and treated for Wegener's granulomatosis at Queen's Medical Centre, Nottingham. The mean follow up period was 8.7 years. Relapse was defined as per the European Vasculitis Study criteria.
RESULTS: cANCA is a useful test at presentation for diagnosis but a negative result does not rule out the disease. Those presenting with ENT symptoms alone may have less raised inflammatory markers but similar cANCA titres as patients with multi-system disease. However, at relapse, patients with ENT disease alone have similar levels of inflammatory markers as those with multi-system relapse. Nasal examination was useful at monitoring the presence of disease activity where the nasal lining is affected.
CONCLUSIONS: Signs of intranasal disease in the form of granular tissue, erythema and bleeding to light touch and crusting over granulation tissue are good predictors of disease activity. A raised cANCA, ESR or CRP provide supporting information about disease activity but if they are negative this does not exclude active disease. cANCA levels were as elevated at relapse in patients who had isolated nasal symptoms and signs as in those with evidence of systemic disease. Low relapse rates were found possibly due to prompt and rigorous initial immunosuppression even in limited disease. This seemed to lead to less progression of patients to multi-system disease and hence a low mortality rate of 5%.