Imbasciati E

Idiopathic IgA mesangial nephropathy. Clinical and histological study of 374 patients.

Histological features and data on the natural history after 1 to 45 years (mean 6.56 +/- 8.55) of total apparent duration and 1 to 13 years (mean 3.48 +/- 5.04) of post-biopsy follow-up, are reported in 374 patients (mean age, 33.9 +/- 11.9 yrs) with idiopathic mesangial IgA nephropathy, who presented with a history of macroscopic hematuria (56%), recurrent in two-thirds of the patients, or with persistent microscopic hematuria and no previous episodes of gross hematuria (44%). Mesangial cell proliferation ranged from minimal to diffuse. Associated varying degrees of extracapillary proliferation, segmental and global glomerular sclerosis, tubulo-interstitial damage and arteriolar hyalinosis usually correlated with each other and with the extent of mesangial proliferation (P less than 0.05). The actuarial curve of progression to renal death showed a 75% survival after 20 years from apparent onset. Progression to renal failure was more rapid in patients with: an older age at onset (P = 0.0582); male sex (P = 0.0730); no history of recurrent gross hematuria (P = 0.0406); high blood pressure (P = 0.0011); more marked global (P = 0.0007) and segmental (P = 0.0026) glomerular sclerosis; more severe interstitial sclerosis (P = 0.0147); more diffuse and global mesangial proliferation (P = 0.0820); mesangio-parietal pattern at immunofluorescence (P = 0.0778). However, all these parameters showed a poor predictive value if applied to any single patient.

Lupus nephropathy and pregnancy. A study of 26 pregnancies in patients with systemic lupus erythematosus and nephritis.

We describe 26 pregnancies in 19 patients with lupus nephritis. There were 4 spontaneous abortions, 2 therapeutic abortions, 4 stillbirths and 1 neonatal death. 10 deliveries were preterm and 2 fetuses were small for gestational age. 8 pregnancies were not accompanied by change of renal symptoms. Mild signs of renal involvement appeared during pregnancy in 4 patients. 6 patients showed an increase in proteinuria already present before pregnancy without renal function deterioration. A moderate worsening of renal function was observed in 3 patients. 4 patients, 3 of whom had an apparent onset of systemic lupus erythematosus during pregnancy, developed anuric acute renal failure after delivery or after late spontaneous abortion. 2 of them died from sepsis and disseminated intravascular coagulation while 2 had complete recovery of renal function. A high rate of complications was observed in patients not adequately treated during pregnancy. Renal biopsy before gestation was not predictive of the outcome of nephropathy during pregnancy, and change of histology in repeated biopsies was frequently observed.

Glomerular Disease and Pregnancy

The clinical course of 123 pregnancies in 86 patients with biopsy-proven glomerular diseases have been studied. In 35 women the onset of nephropathy occurred during pregnancy. No complications were ob

Controlled trial of methylprednisolone pulses and low dose oral prednisone for the minimal change nephrotic syndrome

In a multicentre, randomised, prospective trial 89 patients (67 children and 22 adults) with the minimal change nephrotic syndrome were treated with three intravenous pulses of methylprednisolone followed by low dose oral prednisone for six months (group given methylprednisolone) or with high dose oral prednisone for four weeks followed by low dose oral prednisone for five months (control group). Five patients in the group given methylprednisolone and one in the control group did not respond initially. The time to response was shorter in children treated with methylprednisolone. No significant differences between the two groups were observed in the number of patients who relapsed or number of relapses per patient per year. Patients given methylprednisolone tended to relapse earlier than patients in the control group. Side effects related to treatment were significantly fewer in the group given methylprednisolone than in the control group. These data suggest that a short course of methylprednisolone pulses followed by low dose oral prednisone is only marginally less effective than a regimen of high dose oral steroids but can improve the ratio of risk to benefit associated with treatment of the minimal change nephrotic syndrome.

Acute renal failure in idiopathic nephrotic syndrome.

In 4 adults with idiopathic nephrotic syndrome an acute renal failure with prolonged anuria has been observed. At renal histology 1 patient showed minimal glomerular changes and 3 showed focal glomerulosclerosis. No vascular abnormalities were seen. In all cases severe tubular lesions with casts and cell debris in the lumina were observed. In 2 patients renal function improved after 3 and 6 months, respectively; the remaining 2 died after several months from the onset of acute renal failure being still anuric. Although the pathogenesis of this complication is unknown, tubular obstruction by proteinaceous casts and vasoconstriction may play an important role. The prognosis of this complication is severe, but the possibility of spontaneous recovery even after protracted anuria cannot be ruled out.

Prognostic Value of Renal Biopsy in Acute Rejection of Kidney Transplantation

Graft biopsy was performed in 49 cases of acute rejection in which plasma creatinine levels had not decreased after a first course of high-dose intravenous methylprednisolone. The severity of the hist

Acute renal failure in systemic lupus erythematosus

Acute anuric renal failure complicating systemic lupus erythematosus does not usually respond to treatment with corticosteroids and immunosuppressive agents. We describe four cases treated by dialysis, corticosteroids, and heparin in anticoagulant doses in which there was remarkable improvement in renal function after prolonged anuria. One patient died later from a gastric haemorrhage. The other three were alive and well 55, 54, and 30 months from the onset of anuria. In two cases a second renal biopsy showed a striking improvement in the lesions. Large doses of corticosteroid and heparin may be the best treatment in acute anuric lupus nephritis.

De novo focal glomerular sclerosis in an identical twin renal transplant recipient

A patient with end-stage renal failure caused by bilateral kidney stones received a kidney transplant from his identical twin. No immunosuppressive therapy was given. After a few days he developed a urinary fistula from a polar artery section, which spontaneously healed. Renal function remained subnormal, blood pressure and urinalysis were normal. After one year proteinuria appeared, and after about four years it entered a nephrotic range. Renal biopsy showed focal glomerular sclerosis (FGS). In the following years progressive renal insufficiency and arterial hypertension developed, and the patient had to be submitted to regular dialysis about 9 years after transplantation. As far as we know this is the first case of late renal failure in an isograft related to the development of de novo FGS. It is suggested that de novo FGS in this isotransplant was related to the partial loss of renal mass caused by polar necrosis, which caused glomeralar hyperfiltration. Another possible contributing factor may be kidney denervation, which removes an important mechanism for adjustment of renal arterial flow.

Post-Partum Renal Failure with Microangiopathic Haemolytic Anaemia

Five cases of acute post-partum renal failure with microangiopathic haemolytic anaemia are reported. In 4 patients the pregnancy had been complicated by preeclampsia or frank eclampsia, and in 1 patie

Glomerular disease and pregnancy. A study of 123 pregnancies in patients with primary and secondary glomerular diseases

The clinical course of 123 pregnancies in 86 patients with biopsy-proven glomerular diseases have been studied. In 35 women the onset of nephropathy occurred during pregnancy. No complications were observed in more than half of the pregnancies. In the others, one third of the complications were obstetrical or fetal accidents, one third were renal manifestations (hypertension or deterioration of renal function) and one third were both causes. The lowest incidence of complications was observed in patients with membranous nephropathy and the highest in membranoproliferative glomerulonephritis patients. There were 6 spontaneous late abortion, 6 stillbirths and 5 neonatal deaths. 17 deliveries were preterm and 7 fetuses were small for gestational age. Hypertension appeared in 24 pregnancies, in 13 of which it was reversible and related to superimposed preeclampsia and in 11 it persisted after delivery (5 of these 11 pregnancies were in patients with IgA nephropathy). Renal function deteriorated in 10 cases during pregnancy. The deterioration was reversible in 6 and progressive in 4 (2 of whom had membranoproliferative glomerulonephritis). It is suggested that in most patients pregnancy does not change the natural history of glomerular disease.