Wegener's granulomatosis in Finland in 1981-2000: clinical presentation and diagnostic delay

Objective: To determine the features of clinical presentation and the diagnostic delay of Wegener's granulomatosis (WG) in Finland in 1981-2000. Methods: A retrospective cohort study using hospital discharge registers with review of hospital reports. We determined the spectrum of WG symptoms in relation to individual organ groups and ELK (Ear, nose, throat, Lung, Kidney) score within the first 6 months from disease onset. Diagnostic delay and contributing factors were analysed. Results: Of 513 WG patients treated at Finnish specialized medical care hospital, 489 patients had sufficient information in the first 6 months of their disease. The ENT (Ear, Nose, and Throat) system was involved in 63%; the pulmonary system in 59%, and 62% had general symptoms. Over time, the general symptoms became more common at presentation. The diagnostic delay hhad decreased from 17 to 4 months. Conclusions: The initial symptoms of WG remained essentially unchanged. The proportion of general symptoms increased. A considerable shortening of diagnostic delay occurred, mostly between the 5-year periods of 1981-1985 and 1986-1990, when anti-neutrophil cytoplasmic antibody (ANCA) tests were brought into routine use. ELK scores of 2 and 3 and the presence of pulmonary and general symptoms predicated a shorter diagnostic delay.