The epidemiology and prognosis of glomerulonephritis in Denmark 1985–1997
James Heaf, Hans Løkkegaard, Svend Larsen
Nephrology, Dialysis and Transplantation
presence of IgA nephropathy and systemic lupus Background. The existence of a national renal biopsy erythematosus. register and a national terminal uraemia status register in Denmark provides an opportunity to study the prognosis of glomerulonephritis (GN ), and factors Key words: glomerulonephritis; immunofluorescence; influencing prognosis. uraemia; dialysis; lupus nephritis; Wegener's Methods. Multivariate analysis of 2380 renal biopsies granulomatosis with GN performed between 1985 and 1997
... was done to determine the influence of clinical and histological factors on prognosis. Results. The incidence of GN (39/mio/year) and individual diagnoses did not change during the period. After 10 years, 32% were dead, 13% terminally uraemic, Introduction 5% uraemic and 50% well. Older age increased mortality, but not the incidence of renal failure after the first Glomerulonephritis (GN ) is a rare disease, with a large year. Male sex increased both mortality and incidence number of sub-classifications, and most nephrology of renal failure (34 vs 24% at 10 years, P<0.001). The centres will only meet a small number of patients per diagnoses could be divided into three prognostic groups year with each type. Reliable prognostic information compared with the general population: a good protherefore requires excessively long follow-up periods gnostic group (minimal change GN and membranous or multicentre investigations. The existence of a GN ), with a relative mortality of three and a combined national renal biopsy register (DANYBIR) in renal and patient mortality of four; a poor prognostic Denmark provides an opportunity to study the progroup [crescentic GN, HUS/TTP, chronic GN ] with gnosis of GN and to determine the existence of prorelative mortalities of 8-19 and 13-33, respectively; gnostic factors. A recent questionnaire (personal and the remainder with mortalities of 4-7 and 6-12. communication) of 11 of the 15 referral centres, The presence of multiple glomerular pathology, chronic weighted for biopsy activity, showed that the first line GN, nephrosclerosis and chronic interstitial nephrotreatment of the following glomerular diseases is unipathy worsened the prognosis, while the presence of form in Denmark: endocapillary GN is treated symptoimmune deposits only worsened the prognosis of focal matically; minimal change GN (MCGN ) with steroids; segmental glomerulopathy. Mortality was related to crescentic GN (Cresc) with steroids, cyclophosphamide uraemia and co-morbidity at biopsy, and to the incidand often plasmapheresis (37%); haemolytic uraemic ence of renal failure. Renal failure was correlated to syndrome and thrombotic thrombocytopenic purpura uraemia and hypertension at biopsy but not to (HUS/TTP) with plasmapheresis; lupus nephritis nephrotic syndrome or atherosclerosis. All vascular ( WHO 3 and 4) with steroids and pulse cyclophoscomplications were increased and were positively phamide; and chronic GN symptomatically. The treatrelated to hypertension and negatively correlated to ment of other forms for GN is heterogenous; only a the incidence of uraemia. Crescentric glomerulominority [membranous GN (Mem) 20%, membranonephritis combined with anti-GBM disease had a worse proliferative GN (Mes-P) 36%, mesangioproliferative prognosis than Wegener's granulomatosis, with micro-GN (Mem-P) 36%, focal segmental glomerulopathy scopic polyangiitis and pauci-immune disease occu-( FSGN ) 43%)] treated primarily with immunosupprespying an intermediate position. The prognosis of sive therapy (most commonly steroids and cyclophosmesangioproliferative GN was unaffected by the phamide or azathioprine), the remainder reserving a trial for occasional patients with severe nephrosis or Correspondence and offprint requests to: James Heaf, Graevlingestien 9, 2880 Bagsvaerd, Denmark. progressive renal failure.