9) Balkan Endemic Nephropathy. Interstitial nephrites. Flashcards Preview

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what is balkan endemic nephropathy

unique familial chronic renal disease with high prevalence in serbia , Bulgaria , romania , croatia and Herzegovina

prominent feature - long incubation period , familial clustering , unusual high incidence association with upper urothelilal cancer ( bladder cancer )

insidious onset at 5th decade of life


what are the clinical features of balkan endemic nephropathy ?

unspecific - and remain unrecognised for years

unsymptomatic period

, the patient becoming symptomatic only in advanced renal failure, when anemia is usually marked

then weakness (maybe due to anemia) , mild lumbar pain
pallor of skin and a copper brownish discolouration of the palms and soles

in the early stages of the disease. If hypertension is established, it is usually mild or moderate. In patients with advanced renal failure, blood pressure is frequently increased as a consequence of salt and water excess

Polyuria and nocturia are features of a decreased concentrating capacity of kidneys due to the marked tubulointerstitial damage and progressive renal impairment

Uric acid, urate, and some other crystal formation is frequent

urinary tract infection


what is the diagnosis of balkan endemic nephropathy ?

there are no pathogenic diagnostic features - set of epidemiological , clinical , biochemical data with the pattern of pathological injury in the absences of any other renal disease = highly suggestive

anemia is associated with significant loss of renal function and indication for CKD
Normo- or hypochromic normocytic anemia

intermittent tubular proteinurea maybe found early It seems that the major site of injury in BEN lies in the nephron, predominantly affecting the proximal tubule
it later becomes permanent in advanced renal failure being around 1 g/L or less

Low molecular proteinuria is a hallmark of BEN and is usually accompanied by enzymuria

early stages - sparse urinary sediment

urine concentration capacity problems preceded the decline of GFR
measuring the maximal urinary specific gravity and creatinine clearance in BEN patients has shown that the concentration capability of the kidney and the glomerular filtration rate decrease slowly and progressively over the years

Macroscopic hematuria usually reflects the presence of a urinary tract tumor.
= a variable decrease in kidney size
extreme kidney atrophy - criterion for endemic nephropathy

in BEN patients it is found that the dimension of the kidney dependent on the parental status and age of the offspring = in offspring of mother with BEN = kidney cortex thickness = hotter
with increased excretion of albumin and total protein


shares histological similarity with tubulointerstitial kidney disease

consistent renal histological findings characterizing BEN are :

extensive hypercelllar interstitial fibrosis
associated with tubular atrophy involving medullary rays , where it decreases typical from outer medullary to inner cortex

periglomerular fibrosis
focal segmental glomeruli sclerosis

vascular - arteriolar hyalinosis
intimal fibrous hyperplasia

multifocal thickening and splitting of peritubular capillary basement membrane detected by EM


what is the cause of balkan endemic nephropathy ?

unclear but strong correlation to chronic poisoning with Aristolochic acids

endogenous = genetic predisposition


wat is the treatment for balkan endemic nephropthy ?

Dialysis or kidney transplantation may be needed


what are the characteristics of acute interstitial nephritis / tubulointerstitial disease ?

70 percent are drug induced

transient renal function disorder

inflammation of the renal interstitial

edema in the renal interstitial


what is the etiology of acute interstitial nephritis ?

allergic interstitial nephritis to drugs
analgesics - Saids


crystal induced interstitial nephritis
E.g, acyclovir, indinavir, ciprofloxacin, methotrexate

infectious - bacteria , virus or fungi

autoimmune disease such as SLE , sjoren

neoplasia - leukemia , lymphoma

acute allograft rejection


what are the pathophysiology of acute interstitial nephritis ?

Allergic interstitial nephritis

Drugs, particularly antibiotics and NSAIDs, act as haptens, inducing a hypersensitivity reaction.

Crystal-induced acute kidney injury
Drugs with low urine solubility precipitate within the renal tubules→ tubular obstruction & toxicity to tubules


clinical features of acute interstitial nephritis ?

Allergic interstitial nephritis
Painless hematuria
Flank pain
Faint, diffuse maculopapular rash
fever arthralgia

analgesia involved
- headache
- posterior back ache of kidneys
- vomitting


Crystal-induced acute kidney injury

Patients are usually asymptomatic
Renal colic


what is the diagnosis of acute analgesic interstitial nephritis ?

Allergic interstitial nephritis

after 2/3 days taking drugs

proteinurea less 1g/day


high relative weight of urine

eosinophilia , eosinophilurea

sterile pyuria


urea in blood increased

creatinine in blood increased


Histology: diffuse interstitial inflammatory infiltrate (T lymphocytes, monocytes)

Crystals on brightfield microscopy


what is the treatmnet for acute interstitial nephritis ?

drug induced
- stop taking the drugs immediately
- calcium gluconium ampule
-vit c ampute
- corticosteroids


what is the etiology of chronic tubulointerstitial nephritis ?

Analgesic nephropathy
NSAIDs and acetaminophen
Formerly associated with phenacetin

Myeloma kidney (myeloma cast nephropathy)


what is pathophysiology of chronic analgesic nephropathy ?

arises from the result of daily use of analgesics

Inhibition of prostacyclin synthesis → the decrease synthesis of prostaglandins in kidneys lead to vasoconstriction
of the medullary blood vessels → papillary ischemia and papillary necrosis


what are the risk factors in developing chronic analgesic nephropathy ?

older than 60
heart failure


what is the diagnosis of chronic analgesic nephropathy ?

↑ BUN and creatinine
Tubular proteinuria (usually < 2 g/24 h)
Hematuria: tubulointerstitial diseases show no RBC casts or acanthocytes (in contrast to glomerulonephritis)
Sterile pyuria
WBC casts

histology - kidney biopsy is the only conclusive means of diagnosing tubulointerstitial nephritis although a biopsy is rarely done except when any other causes cannot be found

tubular atrophy and interstitial fibrosis

atrophic tubules may contain pas stain positive hyaline casts
other tubules may be very small containing no lumen representing a endocrine gland acini - endocrine type atrophy

patchy non specific interstitial inflammation


sometimes papillary necrosis

Intravenous urography: test of choice for renal papillary necrosis; findings include:
Irregular papillae
Ring shadows - Contrast material extending into the space around a desquamated papilla
Filling defects in the renal pelvis
Small contrast-containing cavities in the papillary regions


what are the clinical features of chronic tubulointerstitial nephritis ?

the first symptoms to appear :

decreased appetites
difficult breathing
blood pressure is normal or slightly above normal nearly stages

gradually progresses = renal papillary necrosis

painless hematurea
sterile pyuria

colicky pain due to papillary necrosis


what is the dd for chronic tubulointerstitial nephritis ?

chronic pyelonephritis
balkan endemic nephropathy


what is the treatmnet for chronic tubulointerstitial nephritis ?

stopping the causative drug or treating the underlying disorder

controlling blood pressure is often used

Irreversible severe kidney damage, whatever the cause, results in the need for dialysis or kidney transplantation.


what is the prognosis for interstitial nephritis ?

acute has good prognosis

Chronic tubulointerstitial nephritis usually progresses to end-stage renal disease. It is associated with an increased risk of urothelial carcinoma.