Pathologic Findings Light Microscopy

By light microscopy, the acute glomerular lesion is characterized by segmental to global fibrinoid necrosis with crescent formation in over 90 of Table 9.1. Frequency of immunopathologic categories of crescentic glomerulonephritis in over 3000 consecutive native kidney biopsies evaluated by immunofluorescence microscopy in the University of North Carolina Nephropathology Laboratory a 70 of 77 patients tested for ANCA were positive (91 ) (44 P-ANCA and 26 C-ANCA). b 3 of 19 patients tested for...

Clinicopathologic Correlations

Classification of lupus nephritis is considered useful to describe the patient's clinical status and for grouping patients with similar clinical profiles. Moreover, the classification is related to prognosis with respect to renal function and patient survival (40). It has been generally accepted that the use of the lupus nephritis classification facilitates the ease and reliability with which nephrologists and nephropathologists communicate information, and that it has greatly improved...

Recurrent Renal Disease

The frequency and clinical significance of recurrence varies with the disease (14). The diseases that recur in > 25 are dense deposit disease (90 ), immunotactoid fibrillary glomerulonephritis (GN) (67 ), diabetes (> 50 ), IgA nephropathy (45 ), Henoch-Schonlein purpura (33 ), focal glomerular sclerosis (30 ), idiopathic hemolytic uremic syndrome (27 ), and type I membranoproliferative GN (27 ). Lupus nephritis and anti-GBM disease recur infrequently (< 5 ). The diagnosis of recurrence...

Light Microscopy

Microscopically, there is tubular atrophy with associated interstitial fibro-sis, with areas of tubular dropout in more severe cases. Foci of thinned dilated tubules containing cast material may be seen (thyroidization), particularly in the outer cortex. Tubules focally are ruptured, and Tamm-Horsfall protein with other intraluminal contents is in extratubular locations. Mononuclear inflammatory cells including lymphocytes, histio-cytes, and plasma cells are throughout the interstitium in large...

Introduction Clinical Setting

Immunoglobulin A (IgA) nephropathy was first described by the pathologist Jean Berger (1,2) and thus is sometimes called Berger's disease. Immunoglobulin A nephropathy is defined by the presence of IgA-dominant or co-dominant mesangial immunoglobulin deposits (Fig. 6.l) (3). Lupus glomerulonephritis, which may have IgA dominant or co-dominant deposits, is excluded from this diagnostic category. Immunoglobulin A nephropathy occurs as a primary (idiopathic) disease, as a component of Henoch-Sch...

Immunofluorescence Microscopy

The sine qua non for a diagnosis of IgA nephropathy is immunohistologic detection of dominant or co-dominant staining for IgA in the glomerular mesangium (Fig. 6.1). A caveat to this is that the staining for IgA should at least be 1+ on a scale of 0 to 4 + or 0 to 3+. Trace amounts of IgA are not definitive evidence for IgA nephropathy. The IgA is predominantly IgA1 rather than IgA2. Capillary wall staining is observed in about a third of patients, and is more common in Henoch-Sch nlein purpura...

Etiology Pathogenesis

Numerous studies indicate that autosomal recessive Alport's syndrome and benign familial hematuria thin basement membrane disease may represent a spectrum of severe to mild or carrier forms, respectively, of varying molecular defects in the same genes. Linkage of hematuria to mutations in either a4 type IV or a3 type IV has been documented in about 40 of kindreds with apparent thin basement membrane nephropathy clinically (17,21-23). In remaining kindreds without apparent linkage, de novo...

Thin Basement Membranes Introduction Clinical Setting

This basement membrane abnormality has also been described as benign familial hematuria, and shows autosomal dominant or recessive inheritance (12-14). The clinical manifestation is that of chronic hematuria, either macroscopic or microscopic, intermittent or continuous. This lesion is common, and is present in 20 to 25 of patients biopsied for persistent isolated hematuria in some series, and may occur in more than 1 of the general population (17). The lesion may also coexist with other...

Pathologic Findings Gross Findings

As high-grade reflux is such an important and representative form of chronic interstitial nephritis, it alone will be the focus of this discussion. When examined grossly, the kidney in patients with reflux and chronic interstitial nephritis (also called reflux nephropathy) has scarring primarily at the poles with dilated calyces and overlying thinned pale parenchyma. These areas have irregular, broad, deep scars with contraction. The other areas of kidney may not be affected, or may have a...

Gross Findings Light Microscopy

Grossly, petechial hemorrhages or even renal infarcts may be present in patients with scleroderma renal crisis, similar to hemolytic uremic syndrome or malignant hypertension. Microscopically, there is fibrinoid necrosis of afferent arterioles. Interlobular arteries show intimal thickening, proliferation of endothelial cells, and edema. Red blood cell fragments are often present within the injured vessel wall, and there may be vessel wall necrosis and or fibrin thrombi within vessels. Glomeruli...

Cholesterol Emboli Introduction Clinical Setting

Patients with significant atherosclerosis are also at risk for cholesterol embolization due to dislodgment of atheromatous plaque material. These emboli shower organs downstream from the site of origin in the aorta, and Figure 10.3. Cholesterol emboli in artery with surrounding mononuclear and early fibrotic reaction (PAS). Figure 10.3. Cholesterol emboli in artery with surrounding mononuclear and early fibrotic reaction (PAS). thus often involve the kidney, skin, gastrointestinal (GI) tract,...

Scleroderma Progressive Systemic Sclerosis Introduction Clinical Setting

Progressive systemic sclerosis (PSS) is a multisystem disease that affects the skin, the GI tract, the lung, the heart, and the kidney. In the cutaneous limited form (CREST calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia), visceral involvement typically takes much longer to become manifest. Kidney involvement is rare in CREST patients, occurring in 1 (19,20). In PSS, kidney involvement occurs in approximately 60 to 70 . Scleroderma renal crisis,...

Electron Microscopy

The typical ultrastructural finding is immune complex-type electron-dense deposits in the mesangium (Figs. 6.5 and 6.6). Dense deposits most often are found immediately beneath the paramesangial glomerular basement membrane. The amount of deposits varies substantially, with occasional specimens having massive replacement of the matrix by the dense material (Fig. 6.6). Rare specimens that have well-defined IgA deposits by immunofluorescence microscopy do not have detectable mesangial dense...

Table of Contents

Section I Renal Anatomy and Basic Concepts and Methods in 1. Renal Anatomy and Basic Concepts and Methods in Renal Pathology Section II Glomerular Diseases with Nephrotic Syndrome 19 3. Membranoproliferative Glomerulonephritis 4. Minimal Change Disease and Focal SegmentalGlomerulosclerosis Section III Glomerular Disease with Nephritic 5. Postinfectious Glomerulonephritis 6. Immunoglobulin A Nephropathy and Henoch-Sch nlein Purpura 7. Thin Basement Membranes and Alport's Syndrome Section IV...

Arterionephro sclero sis Introduction Clinical Setting

Approximately 60 million people in the United States have hypertension. Many are undiagnosed or untreated. Different populations have different risks and different consequences of hypertension. Increased hypertension is seen with aging, positive family history, African-American race, and exogenous factors such as smoking. Although African Americans make up only 12 of the U.S. population, they are fivefold overrepresented among patients with end-stage renal disease (ESRD) presumed due to...

Light Microscopy Immunofluorescence and Electron Microscopy

Classes I and II lupus nephritis refer to pure mesangial glomerulopathy. These patients present clinically with mild hematuria, or proteinuria, or Figure 8.1. Lupus nephritis ISN RPS class II (mesangial) with granular mesangial immunoglobulin G (IgG) (immunofluorescence). Figure 8.1. Lupus nephritis ISN RPS class II (mesangial) with granular mesangial immunoglobulin G (IgG) (immunofluorescence). both. In general they have a good prognosis with respect to their renal function, and the histologic...

Postinfectious Glomerulonephritis

Meningioma Cell Line Picture

Acute postinfectious glomerulonephritis is a kidney disease that follows after an infection. The most common and best understood form of acute postinfectious glomerulonephritis is poststreptococcal glomerulonephritis. Less is known about the other forms of postinfectious glomerulonephritis. In addition, there are glomerulonephritides that occur during persistent bacterial infections such as bacterial endocarditis, deep abscesses, and infected atrioventricular shunts in hydrocephalus (1). A...

Membranoproliferative Glomerulonephritis

Subendothelial Deposits

Membranoproliferative glomerulonephritis (MPGN) refers to a pattern of injury characterized by diffuse mesangial expansion due to endocapillary proliferation and increased mesangial matrix, and thickened capillary walls, often with a split tram-track appearance (1,2). The immune complexes may be undefined in terms of the inciting antigen (idiopathic) or secondary to chronic infections (3). Of note, basement membrane splitting may be seen in other non-immune complex injuries, such as the late...

Minimal Change Disease and Focal Segmental Glomerulosclerosis

Focal Segmental Glomerulosclerosis

Minimal change disease MCD and focal segmental glomerulosclerosis FSGS are both common causes of the nephrotic syndrome. Minimal change disease accounts for greater than 90 of cases of nephrotic syndrome in children, vs. 10 to 15 of adults with nephrotic syndrome 1 . Focal segmental glomerulosclerosis has been increasing in incidence in the United States in both African Americans and in Hispanics, in both adult and pediatric populations 2-4 . It is now the most common cause of nephrotic...

V7 CVv

Immunofluorescence showing diffuse fine granular distribution pattern of immunoglobulin G IgG along the GBM in membranous glomerulopathy. Figure 2.3. Electron microscopy showing subepithelial electron dense deposits, spike formation of GBM and obliteration of visceral epithelial cell pedicles in membranous glomerulopathy. Figure 2.3. Electron microscopy showing subepithelial electron dense deposits, spike formation of GBM and obliteration of visceral epithelial cell pedicles in...

References

Les depots intercapillaires d'IgA-IgG. J Urol 74 694695,1968. 2. Berger J. IgA glomerular deposits in renal disease. Transplant Proc 1 939-944, 1969. 3. Jennette JC. The immunohistology of IgA nephropathy. Am J Kidney Dis 12 348-352, 1988. 4. Galla JH. IgA nephropathy. Kidney Int 47 377-387, 1995. 5. Donadio JV, Grande JP. Immunoglobulin A nephropathy a clinical perspective. J Am Soc Nephrol 8 1324-1332, 1997. 6. Barratt J, Feehally J. IgA nephropathy. J Am Soc Nephrol...

Renal Anatomy and Basic Concepts and Methods in Renal Pathology

Each kidney weighs approximately 150 g in adults, with ranges of 125 to 175 g for men and 115 to 155 g for women both together represent 0.4 of the total body weight. Each kidney is supplied by a single renal artery originating from the abdominal aorta the main renal artery branches to form anterior and posterior divisions at the hilus and divides further, its branches penetrating the renal substance proper as interlobar arteries, which course between lobes. Interlobar arteries extend to the...

Membranous Glomerulopathy

Membranous Glomerulopathy

Membranous glomerulopathy is a major cause of the nephrotic syndrome in adults 1,2 . Only in the past decades has it been surpassed by focal and segmental glomerulosclerosis as the main cause of the nephrotic syndrome 3-5 . Membranous glomerulopathy develops mostly idiopathically, but can also be seen in relation with and possibly secondary to, among others, hepatitis B, Sjogren's syndrome, transplantation, lupus erythematosus, diabetes mellitus, sarcoidosis, syphilis, exposure to certain drugs...