[Development and prognosis of diffuse endocapillary and focal extracapillary proliferative glomerulonephritis]

Early renal biopsies performed in children with primary nephritic syndrome of acute onset have identified various glomerular pathological patterns with distinct evolution. Complete recovery has been observed in those cases with diffuse endocapillary proliferation. However, progressive disease has resulted from most of the other forms. A histological type characterized by diffuse glomerular endocapillary proliferation with a variable number of glomeruli affected by extracapillary proliferation with crescents, has been observed. A diffuse form of this entity, that is, crescents in 80 to 100 per cent of the glomeruli, has been described in the so-called "rapidly progressive glomerulonephritis". This lesion usually progresses to renal insufficiency in less than six months. Information concerning the focal form, that is, the presence of crescents in less than 80 per cent of the glomeruli, is limited and the long-term prognosis of this morphological finding has not been conclusively established. This communication deals with: 1) history and initial clinical features in 48 patients with focal endo and extracapillary glomerulonephritis, and 2) the correlations between streptococcal etiology, initial features, percentage of glomeruli with crescents and the final outcome, in 27 cases followed for more than two years. Age of patients at the onset of the disease was between 11 months and 14 years; 63 percent of the patients were male. Streptococcal infection was documented in 20 out of the 48 cases. Nephritic syndrome at onset was found in all the 48 cases. Twenty-nine patients had significant proteinuria and in 16 of these, the characteristics of the nephrotic syndrome were also present; 10 patients presented severe arterial hypertension and in other 6, acute renal failure was an initial feature.