A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets

A Danish multicentre study was undertaken of the manifestations, infections , thrombotic events, survival and predictive factors of survival in 513 Dan ish patients with systemic lupus erythematosus (SLE) according to the 1982 classification criteria of the American College of Rheumatology. The mean d uration of follow-up was 8.2 years from diagnosis and 12.8 years from first symptom. This paper describes the most common clinical and laboratory mani festations and their relationship to sex and age at the time of onset and d iagnosis. Cluster analysis revealed three clinically defined clusters at th e time of disease onset. Cluster 1 (57% of patients) consisted of relativel y elderly patients without nephropathy or malar rash, but with a high preva lence of discoid lesions. Cluster 2 (18%) consisted of patients with nephro pathy, a third of whom also developed serositis and lymphopenia. The patien ts of the third cluster (25%) all had malar rash and half were photosensiti ve. Follow-up showed that the patients of cluster 2 developed azotaemia, la rge proteinuria, arterial hypertension and myositis significantly more ofte n than did the rest of the patients, but the mortality was not increased. T he risk of developing renal end-stage disease was highest in men with early -onset disease.