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R J Shepherd

Publications and source records attributed to R J Shepherd.

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Age-related variations in presentation and outcome in Wegener's granulomatosis.

Wegener's granulomatosis (WG) is increasingly being diagnosed in older people. The object of this study was to see whether age influences the clinical presentation and prognosis. In a retrospective open case-note review of 51 patients with a diagnosis of WG based on internationally accepted criteria, 29 patients (56.9%) below 60 years and 22 (43.1%) aged 60 or above were compared. The incidence of limited (10% vs 9.1%) and classical (89.6% vs 90.9%) disease was similar in the two groups, but some clinical features were commoner in the younger cohort at presentation: ear, nose and throat (100% vs 68.1%; p < 0.01), ophthalmic (48.3% vs 18.2%; p = 0.05) and dermatological (51.7% vs 18.2%; p = 0.05). There were no significant differences in the incidence of renal, pulmonary, rheumatological or neurological involvement or in the presence of antineutrophil cytoplasmic antibody. Outcome was significantly worse for the over 60 group despite a similar treatment regimen (prednisolone, cyclophosphamide, and dialysis if required) (six months' survival: 96.5% vs 59.1%; p < 0.01). Renal function at presentation was a significant determinant of prognosis: mean serum creatinine at presentation was 525 mumol/l vs 291 mumol/l respectively in those who died within six months and in those who survived (p = 0.03). Uncontrolled pulmonary vasculitis was the commonest cause of death. In conclusion, the classical presentations of WG are similar in older patients. Disease in the latter is more often restricted to the lungs and the kidneys, and this may cause diagnostic uncertainty. The outcome is worse in older patients, with uncontrolled pulmonary vasculitis the commonest cause of death despite immuno-suppressive treatment. Early diagnosis and treatment may improve outcome.

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