Antineutrophil cytoplasm antibody (ANCA) associated vasculitis.

In 1982 we first reported the presence of antineutrophil cytoplasm antibodies (ANCA) in 8 patients with systemic vasculitis and segmental necrotizing glomerulonephritis. The results of long-term follow-up are described. Screening of 7,500 serum samples revealed positive ANCA in 9 additional patients with vasculitis. Eighty-eight other patients with vasculitis were ANCA negative, including 7 with microscopic polyarteritis nodosa (MPAN) and 8 with Wegener's granulomatosis (WG). Conversely, ANCA were never detected in the absence of vasculitis. Fourteen patients presenting with glomerulonephritis and ANCA were followed for a median of 6.3 years. Eleven patients had MPAN and 3 WG. Remissions were obtained with immunosuppressive therapy in all patients. Clinical relapse was associated with the reappearance of ANCA. Five-year survival was 89% and 5-year dialysis free survival was 77%. ANCA are specific markers for a sub-group of patients with vasculitis and are sensitive markers of disease activity. Glomerulonephritis associated with ANCA positive vasculitis has a favorable outcome with immunosuppressive therapy.

Glomerular lipid deposition and proteinuria in a patient with familial dysbetalipoproteinaemia.

This report describes a male patient who was found to have proteinuria at age 31. Renal biopsy showed glomerular hypercellularity with enlarged, lipid-filled endocapillary cells. On subsequent lipid analysis there was elevation of cholesterol and triglyceride, with apolipoprotein E genotype E2/E2. The clinical course was complicated by pancreatitis and onset of diabetes. After treatment with gemfibrozil and some improvement of the lipid profile, a second renal biopsy showed marked reduction of the glomerular foam cells, despite an increased level of proteinuria. This case emphasizes the potential role that lipid abnormalities may play in renal dysfunction.

The Renal Handling of Dextran 40 by Surgical Patients.

A study has been made of the renal handling of low molecular weight dextran (dextran 40) by surgical patients. In spite of the usual fall in glomerular filtration rate during surgery, high urine dextran concentrations may occur, with the formatin of highly viscous urine. In view of the possible association between high dextron concentrations and oliguric renal failure, precautions in its administration are suggested with particular emphasis on the maintenance of urine flow rates.

Non-dilated urinary tract obstruction.

OBJECTIVE: To describe the occurrence of obstructive uropathy in the absence of dilatation of the urinary tract. CLINICAL FEATURES: Five cases of non-dilated obstructive nephropathy are described. All patients were uraemic on presentation. Obstruction was caused by retroperitoneal malignancy in two patients and uric acid lithiasis in the remaining three. All patients had at least one ultrasound examination. Isotope renography and computed tomography were performed in three and four patients respectively. None of these imaging techniques suggested obstruction in any of the cases. Radionuclide scans were characterised by unusually poor perfusion and parenchymal phase images. INTERVENTION AND OUTCOME: An immediate diuresis and a rapid return of normal renal function occurred after relief of the obstruction in all cases. CONCLUSION: The absence of dilatation in obstructive nephropathy is uncommon but may be responsible for delayed diagnosis and management of a readily treatable cause of acute renal failure.

Wegener's granulomatosis: clinical features and prognosis in 37 patients.

Thirty-seven patients (21 female, 16 male) with Wegener's granulomatosis (WG) were reviewed. Patients were followed for a mean six years after diagnosis; 14 were followed for more than seven years. The clinical features were similar to those in previous studies. In this series, only 13 patients (35%) had renal disease at presentation and the cumulative incidence of renal involvement was 51%. Thirty-one patients received treatment which included cyclophosphamide (CP). The case fatality rate of the six patients not treated with CP was 83% (five deaths). By contrast, all CP treated patients improved and 21 (68%) had complete remissions. Nine (29%) were in complete remission for a mean 4.9 years after discontinuing all treatment. Two were disease free for over ten years. The actuarial probability of survival for these patients was 97% at one year and 71% at ten years. Only three CP treated patients (10%) progressed to end-stage renal disease. The case fatality rate was 26% (eight patients) and sepsis was the cause of death in five. Fourteen patients (45%) treated with CP had at least one relapse of vasculitis and seven (23%) had multiple (two or more) relapses. These data indicate that CP is effective in inducing remissions and prolonging survival in patients with WG; however, relapses are frequent.

Membranous glomerulonephritis associated with sarcoidosis.

Two patients with sarcoidosis involving pulmonary hilar lymph nodes developed the nephrotic syndrome. Renal biopsy in both cases showed membranous glomerulonephritis. In one patient, there was an associated renal vein thrombosis.