Page 2 Fundamentals of Renal Pathology Page 3 Fundamentals of Renal Pathology Agnes B ...

215 Pages·2007·5.36 MB·English

Fundamentals of Renal Pathology
Fundamentals of
Renal Pathology
Agnes B. Fogo, Arthur H. Cohen,
J. Charles Jennette, Jan A. Bruijn,
Robert B. Colvin
Agnes B. Fogo, M.D. Jan A. Bruijn, M.D., Ph.D.
Vanderbilt University Medical Center Leiden University Medical Center
Nashville, TN, USA Leiden, the Netherlands
Arthur H. Cohen, M.D. Robert B. Colvin, M.D.
Cedars-Sinai Medical Center Massachusetts General Hospital
UCLA School of Medicine Boston, MA, USA
Los Angeles, CA, USA
J. Charles Jennette, M.D.
University of North Carolina
Chapel Hill, NC, USA
Library of Congress Control Number: 2005939064
ISBN-10: 0-387-31126-2 e-ISBN 0-387-31127-0
ISBN-13: 978-0387-31126-5
Printed on acid-free paper.
© 2006 Springer Science+Business Media, LLC
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Table of Contents
Section I: Renal Anatomy and Basic Concepts and Methods in
Renal Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
1. Renal Anatomy and Basic Concepts and Methods in
Renal Pathology
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Section II: Glomerular Diseases with Nephrotic Syndrome
Presentations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
2. Membranous Glomerulopathy
Jan A. Bruijn . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3. Membranoproliferative Glomerulonephritis
Agnes B. Fogo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
4. Minimal Change Disease and Focal
SegmentalGlomerulosclerosis
Agnes B. Fogo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Section III: Glomerular Disease with Nephritic
Syndrome Presentations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
5. Postinfectious Glomerulonephritis
Jan A. Bruijn . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
6. Immunoglobulin A Nephropathy and
Henoch-Schönlein Purpura
J. Charles Jennette. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
7. Thin Basement Membranes and Alport’s Syndrome
Agnes B. Fogo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Section IV: Systemic Diseases Affecting the Kidney. . . . . . . . . . . . . 77
8. Lupus Glomerulonephritis
Jan A. Bruijn . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
9. Crescentic Glomerulonephritis and Vasculitis
J. Charles Jennette. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
v
vi Table of Contents
Section V: Vascular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115
10. Nephrosclerosis and Hypertension
Agnes B. Fogo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
11. Thrombotic Microangiopathies
Agnes B. Fogo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125
12. Diabetic Nephropathy
J. Charles Jennette. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132
Section VI: Tubulointerstitial Diseases . . . . . . . . . . . . . . . . . . . . . . . 143
13. Acute Interstitial Nephritis
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145
14. Chronic Interstitial Nephritis
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149
15. Acute Tubular Necrosis
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153
Section VII: Plasma Cell Dyscrasias and Associated Renal
Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159
16. Bence Jones Cast Nephropathy
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
17. Monoclonal Immunoglobulin Deposition Disease
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165
18. Amyloidosis
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170
19. Other Diseases with Organized Deposits
Arthur H. Cohen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 174
Section VIII: Renal Transplant Pathology . . . . . . . . . . . . . . . . . . . . . 179
20. Allograft Rejection
Robert B. Colvin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181
21. Calcineurin Inhibitor Toxicity, Polyoma Virus, and
Recurrent Disease
Robert B. Colvin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 209
Section I
Renal Anatomy and Basic Concepts
and Methods in Renal Pathology
1
Renal Anatomy and Basic Concepts
and Methods in Renal Pathology
Arthur H. Cohen
Normal Anatomy
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 corticome-
dullary junction and give rise to arcuate arteries, which arch between
cortex and medulla and course roughly perpendicular to interlobar arter-
ies. Interlobular arteries, branches of arcuate arteries, run perpendicular
to the arcuate arteries and extend through the cortex toward the capsule
(Fig. 1.1). Afferent arterioles branch from the interlobular arteries and give
rise to glomerular capillaries (Fig. 1.2). A glomerulus represents a spheri-
cal bag of capillary loops arranged in several lobules (Fig. 1.3); the capil-
laries merge to exit the glomerulus as efferent arterioles, which, in most
nephrons, branch to form another vascular bed, peritubular or interstitial
capillaries, which surround tubules. Efferent arterioles from juxtamedul-
lary glomeruli extend into the medulla as vasa recta, which supply the outer
and inner medulla. The vasa recta and peritubular capillaries collect,
forming intointerlobular veins; the veins follow the arteries in distribution,
size, and course, and leave the kidneys as renal veins, which empty into the
inferior vena cava.
The kidneys have three major components: the cortex, the medulla, and
the collecting system. On the cut surface, the cortex is the pale outer
region, approximately 1.5 cm in thickness, which has a granular appearance
because of the presence of glomeruli and convoluted tubules. The medulla,
a series of pyramidal structures with apical papillae, number normally
eight to 18, and have a striped or striated appearance because of the paral-
lel arrangement of the tubular structures. The bases of the pyramids are
at the corticomedullary junction and the apices extend into the collecting
3
4 A.H. Cohen
Figure1.1. Low magnification of cortex with portions of two glomeruli, tubules,
and interstitium and interlobular artery with arteriolar branch [periodic acid-
Schiff (PAS) stain].
A
B
Figure 1.2. A: Low magnification of cortex. An arcuate artery (AA), interlobular
artery (IA), and afferent arteriole (aa) are in continuity (Jones silver stain). B:
Interlobular artery (IA) with afferent arteriole (aa) extending into glomerulus
(Masson trichrome stain).
1. Renal Anatomy and Basic Concepts and Methods 5
Figure 1.3. Normal glomerulus with surrounding normal tubules and interstitium
(Jones silver stain).
system. Cortical parenchyma extends into spaces between adjacent pyra-
mids; this portion of the cortex is known as the columns of Bertin. A
medullary pyramid with surrounding cortical parenchyma, which includes
both columns of Bertin as well as the subcapsular cortex, constitutes a
renal lobe. The collecting system consists of the pelvis, which represents
the expanded upper portion of the ureter, and is more or less funnel
shaped. Each pelvis has two or three major branches known as the major
calyces. Each calyx divides further into three or four smaller branches
known as minor calyces, each usually receiving one medullary papilla.
Each kidney contains approximately 1 million nephrons, each composed
of a glomerulus and attached tubules. Glomeruli are spherical collections
of interconnected capillaries within a space (Bowman’s space) lined by
fl attened parietal epithelial cells (Fig. 1.3). Bowman’s space is continuous
with the tubules, with the orifice of the proximal tubule generally at the
pole opposite the glomerular hilus, where the afferent and efferent arteri-
oles enter and leave, respectively. The outer aspects of the glomerular
capillaries are covered by a layer of visceral epithelial cells or podocytes.
Each visceral epithelial cell has a large body containing the nucleus and
cytoplasmic extensions, which divide, forming small finger-like processes
that interdigitate with similar structures from adjacent cells and cover the
capillaries. These interdigitating processes, known as pedicles, are also
called foot processes because of their appearance on transmission electron
microscopy. The space between adjacent foot processes is known as the
fi ltration slit; adjacent foot processes are joined together by a thin mem-
brane known as the slit-pore diaphragm. Epithelial cells cover the glo-
merular capillary basement membrane, a three-layer structure with a
central thick layer slightly electron dense (lamina densa) and thinner elec-
tron lucent layers beneath epithelial and endothelial cells (lamina rara