Scribd
Upload
53 views34 pages

Tubulo-Interstitial Diseases

Uploaded by

gibreil
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
53 views34 pages

Tubulo-Interstitial Diseases

Uploaded by

gibreil
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
You are on page 1/ 34

TUBULO-INTERSTITIAL DISEASES

• The clinical presentation is with


a) tubular dysfunction and
b) electrolyte abnormalities,
c) moderate proteinuria and
d) varying degrees of renal impairment.
Acute interstitial nephritis
• Acute interstitial nephritis (AIN) may be
caused by
1) allergic reactions (e.g. penicillins, NSAIDs),
2) autoimmune nephritis, infections (e.g.
pyelonephritis, TB) or
3) toxins (e.g. mushrooms or myeloma light
chains).
Clinical presentation
1) non­oliguric renal impairment or
2) an eosinophilic reaction with fever and rash.
Investigations
1) Urinalysis shows leucocytes and
eosinophils in 70% of patients.
2) Renal biopsies show intense inflammation
surrounding tubules and blood vessels and
invading tubules, with occasional eosinophils
(especially in drug­induced disease).
Continue
• Only ~30% of patients with drug­induced
AIN have a generalised drug
hypersensitivity reaction (e.g. fever, rash,
eosinophilia).
Management
a) Remove/treat the cause.
b) Steroids may accelerate recovery and
prevent long­term scarring.
c) Short­term dialysis is sometimes required.
Chronic interstitial nephritis
• It is often diagnosed late and has no apparent
aetiology.
Clinical features
• Most patients present in adult life with
a) moderate CKD (stage 3),
b) hypertension and
c) small kidneys
d) electrolyte abnormalities (e.g.
hyperkalaemia, acidosis) may be severe.
• Urinalysis is non­specific.
Causes of chronic interstitial nephritis

1) Acute interstitial nephritis


2) Glomerulonephritis
3) Immune/inflammatory (sarcoid, Sjögren’s,
systemic lupus erythematosus)
4) Toxic (mushrooms, Balkan nephropathy,
lead)
Continue
5) Drugs (ciclosporin, tacrolimus, tenofovir,
lithium, analgesics)
6) Infection (severe pyelonephritis)
7) Congenital/developmental (reflux, sicklecell
nephropathy)
8) Metabolic and systemic diseases
(hypokalaemia, hyperoxaluria)
Management
• supportive, with correction of electrolyte
abnormalities and RRT if required
Reflux nephropathy (chronic pyelonephritis)

• This chronic interstitial nephritis is associated


with vesico­ureteric reflux (VUR) in early life,
and with the appearance of scars in the
kidney.
Clinical features
• Usually the renal scarring and renal/ureteric
dilatation are asymptomatic.
• Presentation may be at any age, with
a) hypertension,
b) proteinuria or
c) features of CKD.
Continue
• Frequency, dysuria and lumbar back pain
may be present; however, there may be no
history of UTIs.
• There is an increased prevalence of urinary
calculi.
Investigations
1) USS: will exclude significant obstruction
but is not helpful in identifying renal
scarring.
Continue
2) Longitudinal CT/MRI: may be useful to assess
progression.
3) Radionuclide scans: sensitive but seldom
required, as surgery for VUR is uncommon.
4) Urinalysis shows leucocytes and proteinuria
(usually < 1 g/24 hrs).
Management and prognosis
• Treat infection.
• If recurrent, use prophylactic therapy.
• Nephrectomy is indicated if infection recurs in
an abnormal kidney with minimal function.
• Hypertension is occasionally cured by the
removal of a diseased kidney when disease
is unilateral.
• Otherwise, surgery is rarely indicated as
most childhood reflux disappears
spontaneously
CYSTIC KIDNEY DISEASES
Polycystic kidney disease
• Adult polycystic disease (PKD) is a common
condition (prevalence ~1 : 1000) that is
inherited as an autosomal dominant trait.
Small cysts lined by tubular epithelium
develop from infancy or childhood and
enlarge slowly and irregularly.
• Autosomal dominant polycystic kidney disease
(ADPKD) is the most common genetic cause of
chronic kidney disease
• Renal failure occurs from recurrent episodes
of pyelonephritis and nephrolithiasis
Continue
• Renal failure is associated with grossly
enlarged kidneys.
• PKD is not a pre­malignant condition.
Continue
• Mutations in PKD1 account for 85% of cases
and PKD2 for ~15%.
• ESRD occurs in ~50% of patients with PKD1
with a mean age of onset of 52 yrs, but in a
minority of patients with PKD2 with a mean
age of onset of 69 yrs.
• Between 5 and 10% of patients on RRT have
adult PKD.
 Common clinical features
a) Asymptomatic until later life
b) Vague discomfort in loin or abdomen due to
increasing mass of renal tissue
c) One or both kidneys palpable, with
nodular surface
d) Acute loin pain or renal colic due to
haemorrhage into a cyst
Continue
e) Hypertension gradually develops over age
20 yrs
f) Haematuria (with little or no proteinuria)
g) Urinary tract or cyst infections
h) Gradual-onset renal failure
Associated features
1) Hepatic cysts (30%)
2) Berry aneurysms of the cerebral vasculature
3) Mitral and aortic regurgitation (common
but rarely severe)
4) Colonic diverticula
5) Abdominal wall hernias
Management
• Good BP control is important because of
cardiovascular morbidity and mortality, but
there is no evidence that this retards the
development of renal failure in PKD.
Continue
• There is some evidence that the vasopressin
V2 receptor antagonist tolvaptan may slow
cyst formation but this is not yet
established treatment.
Continue
• Patients with PKD are usually good
candidates for dialysis and transplantation.
• Sometimes kidneys are so large that one or
both have to be removed to make space for a
renal transplant.
Other cystic diseases
Medullary sponge kidney
• Characterised by cysts confined to papillary
collecting ducts, this disease is not inherited
and its cause is unknown.
Continue
• Patients usually present as adults with renal
stones.
• These are often recurrent, but the prognosis
is generally good.
• The diagnosis is made by USS or
intravenous urography (IVU).
• Contrast medium is seen to fill dilated or cystic
tubules, which are sometimes calcified
Acquired cystic disease
• Patients with a very long history of renal
failure (and usually on long­term dialysis) often
develop multiple renal cysts in their shrunken
kidneys
• Kidneys are enlarged but not to the size
seen in PKD.
Continue
• Acquired cystic disease is associated with
1) increased erythropoietin production and
2) an increased risk of renal cell carcinoma.

You might also like