Renal Pathology Pt. 3 Flashcards
what are the hereditary nephritises?
Alport syndrome
Thin basement membrane lesion/benign familial hematuria
what is alport syndrome?
X-linked
mutated gene encoding collagen IV
what is the clinical presentation of alport syndrome?
hematuria
CKD
nerve deafness
eye disorders
what are the characteristics of alport syndrome under an electron microscope?
moth-eaten/frayed basement membrane
what is thin basement membrane disease?
AR
mutated gene encoding a3 or a4 chains of collagen IV
what is the clinical presentation for thin basement membrane disease?
asymptomatic hematuria
usually uncovered during routine UA
which systemic diseases cause secondary nephrotic syndrome?
DM
SLE
Hep C
HIV
which systemic diseases cause secondary nephritic syndrome?
SLE
bacterial endocarditis
goodpasture syndrome
Henoch Schonlein purpura
what is the pathogenesis underlying diabetic nephropathy?
metabolic defect linked to hyperglycemia produces thick GBM
hemodynamic effects associated with glomerular hypertrophy contribute to glomerulosclerosis
what can be seen histologically in diabetic glomerulosclerosis?
diffuse capillary basement membrane thickening
diffuse mesangial sclerosis
nodular glomerulosclerosis (PAS positive)
what can be seen histologically in advanced renal hyaline arteriolosclerosis?
marked thickened, tortuous afferent arteriole
what are the 3 prototypical renal lesions associated with diabetes?
nodular glomerular lesions
vascular lesions (arteriolosclerosis)
necrotizing papillitis
what are the 6 classifications of glomerular disease caused by SLE?
I. Minimal mesangial lupus nephritis II. Mesangial proliferative lupus nephritis III. focal lupus nephritis IV. diffuse lupus nephritis V. membranous lupus nephritis VI. advanced sclerosing lupus nephritis
which class of glomerular disease are most commonly seen in SLE?
IV. diffuse lupus nephritis
how does Henoch Schonlein purpura affect the kidneys?
IgA is deposited in the glomerular mesangium
what is fibrillary glomerulonephritis?
morphological variant of glomerulonephritis associated with fibrillar deposits in the mesangium and capillary walls
what is the clinical presentation of fibrillary glomerulonephritis?
nephrotic syndrome
hematuria
progressive renal failure
which three systemic disorders manifest in the kidneys with foci of glomerular necrosis and crescent formation?
goodpasture syndrome
microscopic polyangiitis
Wegener granulomatosis
what infection is associated with essential mixed cryoglobulinemia?
Hep C
what is the pathogenesis of essential mixed cryoglobulinemia?
IgG-IgM complexes that induce cutaneous vasculitis, synovitis, and proliferative glomerulonephritis
what is acute tubular injury?
acute renal failure and morphologic evidence of tubular necrosis
what are the causes of ATI?
ischemia
direct toxic injury by endogenous or exogenous agents
what is the most common cause of acute renal failure?
ATI
what are some uncommon causes of ATI?
hypersensitivity to drugs
urinary obstruction
what is the necrosis pattern associated with ischemic necrosis ATI?
patchy in PCT
patchy in PST
patchy in LoH
what is the necrosis pattern associated with toxic necrosis ATI?
continuous in PCT
continuous in PST
patchy in LoH
what is clinically evident in the initiation stage of AKI?
oliguria
what is clinically evident in the maintenance stage of AKI?
oliguric crisis with some uremic features
hyperkalemia
what is clinically evident in the recovery stage of AKI?
large urine volumes
large loss of water, Na, K
hypokalemia
susceptibility to infection
what is tubulointerstitial nephritis?
a group of renal diseases involving inflammatory injuries to tubules and interstitium that are insidious in onset
what are the clinical manifestations of tubulointerstitial nephritis?
azotemia
inability to concentrate urine (polyuria)
what are the primary etiologies of tubulointerstitial nephritis?
infections toxins metabolic diseases chronic obstruction neoplasms immunologic reactions vascular diseases
what is the pathway of infection to the kidneys?
UTI –> cystitis –> pyelonephritis
what is chronic pyelonephritis?
recurrent or continuous infection of the kidney with resultant damage to the pelvis/calyces/parenchyma resulting in anatomic distortion
what is the most common UTI causing bacteria?
E. coli
what other bacteria are known to cause UTI?
Proteus
Klebsiella
Enterobacter sp.
what anatomic defects predispose a patient to pyelonephritis?
vesicoureteral reflux
intrarenal reflux
what medical conditions predispose a patient to pyelonephritis?
diabetes
pregnancy
BPH
being female
what are possible complications of pyelonephritis?
papillary necrosis
pyonephrosis
perinephric abscess
what is the most common cause of pyelonephritis?
ascending cystitis combined with a predisposing anatomical defect
what is the most common cause of vesicoureteral reflux?
congenital partial or complete lack of oblique angle of the intravesical portion of the ureter
what is the most common cause of AKI?
pyelonephritis
what is the second most common cause of AKI?
tubulointerstitial nephritis induced by drugs or toxins
what drugs cause tubulointerstitial nephritis?
analgesics synthetic penicillins (methicillin, ampicillin) rifampin diuretics NSAIDs allopurinol cimetidine
what is the clinical presentation of tubulointerstitial nephritis induced by drugs/toxins?
fever
eosinophilia
interstitial renal parenchymal infiltrates
what is a potential complication of analgesic nephropathy?
urothelial carcinoma
what is urate nephropathy?
precipitation of uric acid crystals in the renal tubules leading to obstruction of the nephrons and acute renal failure
what is the primary cause of acute uric acid nephropathy?
chemotherapy causing tumor lysis syndrome
what is the primary cause of chronic urate nephropathy?
hyperuremic syndrome –> gout
what is a potential complication of hyperuricemia?
renal stones/nephrolithiasis
what are potential complications of hypercalcemia?
formation of calcium stones
deposition of calcium in the kidney
what is acute phosphate nephropathy?
accumulations of calcium phosphate crystals in the tubules that can occur in patients after consuming high doses of oral phosphate solutions in preparation for colonoscopy
what is myeloma kidney?
renal insufficiency as a result of multiple myeloma
what factors contribute to renal damage in myeloma kidney?
Bence-Jones proteinuria cast nephropathy amyloidosis light-chain deposition disease hypercalcemia hyperuricemia
what is bile cast nephropathy?
hepatorenal syndrome in which renal dysfunction manifests in patients with advanced liver failure due to bile cast formation in the distal nephron
what are the renal vascular diseases?
nephrosclerosis renal artery stenosis thrombotic microangiopathies atheroembolic renal disease sickle-cell nephropathy
what is benign nephrosclerosis?
morphologic changes associated with hyaline sclerosis of the renal arterioles and small arteries
what is the effect of benign nephrosclerosis?
multi-focal ischemia of the renal parenchyma supplied by the sclerotic vessels
what conditions contribute to the formation of benign nephrosclerosis?
age
HTN
DM
what is malignant arteriosclerosis?
a medical emergency
renal vascular disorder exhibiting injury associated with accelerated HTN
what are the pathologic effects of malignant arteriosclerosis?
ischemic kidney injury
elevated renin
a self-perpetuating cycle of damage and HTN
what is the pathogenic pathway of malignant HTN?
renal vascular damage
increased permeability of small vessels
endothelial irreversible injury
focal vascular cell death/hemorrhage
platelet deposition/thrombosis
fibrinoid necrosis of arterioles and small vessels
hyperplastic arteriolitis (renal failure)
what are the renal morphological changes associated with malignant htn?
“flea-bitten” appearance of renal hemorrhages
what is the clinical presentation of malignant htn?
BP >180/120 papilledema retinal hemorrhages encephalopathy CV abnormalities renal failure
what is renal artery stenosis?
potentially curable form of HTN secondary to atherosclerosis or fibromuscular dysplasia
what are the thrombotic microangiopathies that result in renal damage?
hemolytic uremic syndrome
thrombotic thrombocytopenic purpura
what is HUS?
endothelial cell injury and activation due to Shiga-like toxin (E. coli O157:H7)
what is TTP?
acquired deficiency of ADAMTS13 resulting in abnormal function of vWF
what is the clinical presentation of TTP?
fever neurological symptoms microangiopathic hemolytic anemia thrombocytopenia renal failure
what is the most common cause of renal atheroembolism?
mural thrombosis from left side of the heart
what are the clinical signs of renal atheroembolism?
flank pain hematuria HTN arrhythmia nausea/vomiting oliguria/anuria
what is sickle cell nephropathy?
papillar necrosis due to accelerated sickling in the renal medulla
what are the clinical features of sickle cell nephropathy?
hematuria
hyposthenuria (diminished concentrating ability)
proteinuria