Conrad L. Pirani

Long-term follow-up of patients with lupus nephritis: A study based on the classification of the World Health Organization

The long-term course of 56 patients with systemic lupus erythematosus who had precisely defined renal histology and carefully assessed clinical status at the time of their initial renal biopsy prior to 1976 was evaluated and analyzed by life-table analysis. The average length of follow-up has now been greater than 10 years since initial biopsy. Patients with mesangial lesions (World Health Organization [WHO] classes IIA and IIB) had a more favorable renal and patient survival at five and 10 years than did patients in the other WHO classes (III, IV, and V). Individual renal histologic features of activity and chronicity when combined into an activity index and a chronicity index did not significantly predict renal survival in this population, nor did the presence of hypertension or renal dysfunction at the time of the initial renal biopsy significantly influence renal or patient survival. Patients with the nephrotic syndrome at initial biopsy had a poorer renal survival than did patients without the nephrotic syndrome. However, patients who experienced a remission of the nephrotic syndrome fared better in terms of both renal and patient survival than did those patients without a remission. By life-table analysis, patient survival was significantly better for patients in whom biopsy was performed after 1973 than for those in whom biopsy was performed prior to that time despite similar clinical features and WHO histology in each group interval. Our data suggest that improved survival for patients in recent studies may relate to better supportive care and more selective use of immunosuppressive therapy in patients with milder forms of lupus nephritis.

Renal vein thrombosis and the nephrotic syndrome

Abstract 1.1. Two cases of renal vein thrombosis with the nephrotic syndrome have been observed. One patient died. The second patient had multiple pulmonary infarctions and thrombosis of both common iliac veins. The nephrotic syndrome developed after gradual or incomplete occlusion of the left renal vein. Two months later more rapid occlusion of the right renal vein occurred. The right kidney became functionless, secondarily infected and was later removed. Treatment with anticoagulant and antibiotic drugs reversed the previously relentless downhill course and the patient thereafter made an uninterrupted recovery. 2.2. The clinical features of the reported cases have been reviewed, and the clinical syndrome of renal vein thrombosis with the nephrotic syndrome has been delineated. In adults, renal vein thrombosis may present with acute symptoms referable to the affected kidney, as the nephrotic syndrome of obscure etiology or as a combination of the two. The presenting picture is related to the rapidity and completeness of occlusion of the vein. 3.3. In two cases histologic studies have been made on sections from serial percutaneous renal biopsy and autopsy material and in five other cases from autopsy material. The glomerular basement membrane was thickened and the tubules were atrophied. The interstitial tissue was edematous at first and later became the seat of an extensive fibrosis. The changes in the tubules and interstices were out of proportion to the affection of the glomeruli.

THE NEPHROTIC SYNDROME IN ADULTS: A COMMON DISORDER WITH MANY CAUSES

Excerpt The metabolic, nutritional and clinical consequences of continued massive albuminuria constitute the nephrotic syndrome. Florid cases are readily recognized from infancy1, 2to extreme old a...

Tubulointerstitial Disease in Lupus Nephritis: Relationship to Immune Deposits, Interstitial Inflammation, Glomerular Changes, Renal Function, and Prognosis

The interrelationships between tubulointerstitial immune deposits (TID), interstitial inflammation, glomerular changes, renal function, and prognosis were assessed in the renal biopsies from 93 patients with lupus nephritis. The prevalence of TID was 33% by immunofluorescence and 23% by electron microscopy. Although predominantly detected along and within tubular basement membranes, extraglomerular immune deposits were also present in the wall of renal interstitial capillaries and small arteries as well as in Bowmans capsule. The prevalence of TID correlated with the activity of glomerular lesions and, to a lesser extent, with the severity of proliferative lupus nephritis (WHO classes II-IV). TID were much less common in the membranous form (WHO class V). The severity of interstitial inflammation correlated with the degree of renal insufficiency and was an accurate prognostic indicator of progressive deterioration of renal function. However, there was no correlation between prevalence of TID and prevalence and severity of interstitial inflammation, suggesting that the latter is not necessarily secondary to the presence of immune complexes and that other pathogenetic mechanisms may be involved.

THE REPRODUCIBILITY OF SEMIQUANTITATIVE ANALYSES OF RENAL HISTOLOGY.

(1) Observations are reported on two independent semiquantitative histologic analyses of 57 renal biopsy and autopsy specimens from patients with SLE. The analyses were each made by two observers and

Natural History of Lipoid Nephrosis and of Membranous Glomerulonephritis

Abstract Twenty-one patient each with lipoid nephrosis (LN) and membranous glomerulonephritis (MGN) were observed for a long period of time. Six patients with LN and one with MGN were under 14 year...

Production of experimental toxemia in the pregnant rabbit

Experimental toxemia of pregnancy was induced in 51 of 122 rabbits by constriction of the aorta below the renal arteries. The approach was extraperitoneal and dependent on the accuracy in calibrating this stricture between 0.6 and 1.0 mm. Experimental toxemia in the rabbit was characterized by hypertension, proteinuria, weight gain, and reduced weight of the fetus. Blood pressure and blood flow studies distal to the aortic constriction demonstrated a marked diminution of blood supply below the constriction. The light microscopic changes in the kidneys and in the liver were similar to those of human toxemia. The electron microscopic changes consisted of endothelial swelling and subendothelial deposits. In a separate experiment, 22 pregnant rabbits near term had an aortic constriction varying between 0.6 and 1.0 mm. This constriction lasted 4 to 12 days. Glomerular deposits of fibrinogen were demonstrated by immunofluorescence in 20 of 22 animals. The intensity of the immunofluorescence was related to the severity and duration of the aortic stricture.

Rapidly Progressive (Nonstreptococcal) Glomerulonephritis

Abstract Eight patients with renal failure associated with a rapidly progressive glomerulonephritis were observed. The onset of illness was insidious and remarkable for the lack of a history sugges...

De Novo Membranous Glomerulonephropathy in Renal Allografts: A Report of Ten Cases and Review of the Literature

De novo posttransplantation membranous glomerulonephropathy (MGN) is the most common form of de novo glomerulopathy in renal allografts. The clinical and pathological features of ten patients with de novo MGN were studied and the related literature was reviewed to assess the clinical features, morphologic characteristics, and natural course of this disease. De novo MGN may occur in both living related and cadaveric allografts at any time after transplantation. It presents clinically either as asymptomatic proteinuria or the nephrotic syndrome, a feature of poor prognostic implication. Morphologically, de novo MGN in most instances has distinct differences from idiopathic MGN in native kidneys and is accompanied by varying features of rejection. About 50% of grafts which develop de novo MGN eventually fail. This rather poor outcome may not represent the natural history of de novo MGN per se but rather the consequences of associated chronic rejection. Evidence is presented that many of the cases of so-called de novo MGN may be a complication of transplant glomerulopathy rather than being caused by mechanisms totally independent from rejection.

ELECTRONMICROSCOPIC STUDIES OF REVERSIBLE GLOMERULAR LESIONS IN THE ADULT NEPHROTIC SYNDROME

Excerpt The nephrotic syndrome is associated with a number of disease processes, which produce a variety of pathologic lesions in the kidney. The histology of many of these disease processes, inclu...