Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Final Year Medicine Notes Nephrology
Letuda's Nephrology Note
Version 1.0
Created and compiled by
Prithwiraj Maiti
R.G.Kar Medical College
Batch 2015
Email ID: prithwiraj2009@yahoo.in
Table of contents
Content
Page no.
Chronic kidney disease (ckd)
2
Anemia in ckd
11
Acute kidney injury (aki)
12
Acute tubular necrosis (atn)
13
Nephrotic syndrome
19
glomerulonephritis
22
Post-infectious gn
26
IgA NEPHROPATHY
27
HENOCH ?SCHONLEIN PURPURA
27
MINIMAL CHANGE DISEASE
28
MEMBRANOUS NEPHROPATHY
29
UTI
30
VASCULITIS
33
WEGENER'S GRANULOMATOSIS
37
GOODPASTURE SYNDROME
37
CHURG-STRAUSS SYNDROME
38
LUPUS NEPHRITIS
39
Beh?et's disease
40
2
Chronic kidney disease (CKD)
Definition:
Presence of any of the following for at least 3 months:
1. Evidence of kidney damage with/ without reduced GFR
OR
2. GFR 60 ml/min/1.73 sq.mt body surface area.
Evidence of kidney damage:
1. Biochemical abnormalities: Urea Creatinine
2. Urinary abnormalities: Proteinuria/ sediment/ cast
3. Radiological evidence: Bilateral small kidneys
4. Histopathological abnormalities.
Stages of CKD:
Stage
GFR (Normal: 90)
Description
1
Evidence of kidney damage +Ve when
Minor kidney damage
GFR 90
2
60-89
Mild
3
30-59
Moderate
4
15-29
Severe
5
<15
Kidney failure/ end stage renal
disease*
*It is a condition where without renal replacement therapy (dialysis/ renal
transplantation), patient will not survive.
Azotemia and uremia
Azotemia: Accumulation of nitrogenous substance in the blood due to defective renal
clearance.
Uremia: Clinical manifestations due to azotemic condition.
3
Causes of CKD:
CKD
Tubulointerstitial
Glomerular disease
Blood vessels
Congenital causes
disease
Primary glomerular
Secondary
Analgesic
Polycystic kidney
Hypertension
diseases
glomerular diseases
nephropathy
disease
Membrano-
Heavy metal
Diabetes
Renal artery stenosis
Alport's syndrome
proliferative GN
poisoning
Connective tissue
igA nephropathy
disease (SLE)
Vasculitis
4
Pathophysiological effects of CKD:
Pathophysiological effects of CKD
A
B
C
Azotemia (toxic N2
BP
Blood
substances)
Bone
Circulatory changes
Accelerated
Effect on bone
Constitutional symptoms
atherosclerosis
metabolism
Encephalopathy
Na+/ water retension
Renal osteodystrophy
Pericarditis
Peripheral neuropathy
Effects of CKD on bone:
Effect on bone mineralization
1. De-mineralization
2. Osteitis fibrosa cystica.
5
Renal osteodystrophy
CKD
PO4- clearance
1,25 (OH)2 cholecalciferol
Serum PO4- level
Calcium absorption
Serum Ca++ (Hypocalcemia)
PTH secretion
Secondary hyperparathyroidism
Effect of CKD on blood picture:
1. Erythropoietin synthesis -- Anemia
2. Toxins-- Platelet dysfunction.
Circulatory effect:
Water retention
Volume overload
Congestion
Systemic venous
Pulmonary
congestion
congestion
6
Clinical features
1. Constitutional symptoms:
Anorexia
Nausea
Refractory hiccup
Metallic taste in mouth
Wasting of the muscles.
2. CNS (symptoms of uremic encephalopathy):
Altered sensorium
Behavioral disturbances
Confusion; in severe cases: convulsion and coma may occur
Delirium
Flapping tremor
GCS.
3. Circulatory/ CVS:
Symptoms and signs are mainly due to volume overload and may resemble heart
failure.
Symptoms:
Progressive swelling
Pulmonary congestion: Shortness of breath, orthopnea, PND
Signs:
Signs of systemic venous congestion: Edema, JVP
Signs of pulmonary congestion: Bilateral crepitations.
4. Kidney:
Features due to kidney dysfunction: Urine output
Note that urine output often remains normal till significant renal dysfunction
develops.
5. Disease:
Features of the underlying disease, which must be looked for.
6. Features due to disturbed bone metabolism:
Bone pain: Spontaneous/ after trivial trauma
Proximal myopathy (due to vitamin D deficiency).
Investigation
Principles:
1. To look for different biochemical and systemic effects of CKD
2. To detect the underlying cause.
7
Preliminary investigations
A. Blood biochemistry:
Urea creatinine:
Na+: Normal/ (due to dilutional hyponatremia)
K+: Normal/
Ca++: Normal/
PO4-: Normal/
ABG: pH (due to HCO3-)
Vitamin D level: Normal/
PTH level: Normal/
Uric acid: Normal/
Hb level: Normal/
Special note: Blood glucose in diabetic patients:
Often glucose control becomes better/ patient may develop recurrent
hypoglycemia due to delayed excretion of insulin which in turn prolongs half-
life of insulin. Therefore in diabetic patients, once CKD develops, dose of
medications often needs to be decreased.
B. Calculating creatinine clearance:
=(140- )? ( )
( /)?72 =
For females, =?0.85
C. Urine (microscopic examination):
To look for biochemical abnormalities:
Proteinuria
Any cast (particularly RBC case, tubular cast): May give an idea about the
underlying disorder.
24 hour urine for albumin: creatinine/ microalbumin: creatinine ratio.
D. Kidney-ureter-bladder (KUB) USG:
8
Bilateral small kidneys (seen in CKD)
Unilateral small kidney (seen in renal artery stenosis).
E. Chest X-Ray, ECG, Echo:
To look for any cardiac pathology.
F. Renal biopsy:
In selected cases only: confirms the underlying type of kidney disease: which in
most cases are: unexplained glomerulonephritis and nephrotic syndrome.
G. Relevant investigation(s) to detect the cause.
Treatment
General treatment
R:
1. Regular monitoring of urine output: particularly in significant CKD
2. Regular monitoring of volume status, i.e. regular monitoring of body weight
3. Regular monitoring of blood biochemistry.
E:
Treatment of the underlying etiology.
N:
Maintenance of nutrition:
Dietary modification
Fluid and salt restriction
Dietary protein restriction
In presence of associated DM: Restriction of carbohydrate
Restriction of K+:
o Parboiling of rice (to discard water after boiling)
o Avoid juicy food.
Avoid beverages (as they are rich in phosphates).
9
A:
1. Avoid all nephrotoxic drugs
2. Adjust the dose of drugs according to creatinine clearance.
L:
Look for common complications:
Features of dehydrations (due to diuretics + fluid restriction)
Features of dyselectrolytemia
Features of infection.
Specific treatment
F:
Fluid balance
There may be 3 sources of volume overload:
Volume
overload
Salt and water
Use of loop
Dialysis
restriction
diuretics
Often these patients will come with dehydration due to overdiuresis which should be
treated with IV fluid cautiously.
A:
1. Acidosis:
Mild to moderate: NaHCO3 if required
Severe: Dialysis.
2. Anemia:
Blood transfusion if required
Correct any underlying iron deficiency
Long term erythropoietin therapy.
10
I:
Infection: Treat with appropriately adjusted dose of antibiotics.
L:
Loss of blood due to associated coagulopathy: Treat with blood transfusion.
U:
Treat uremic encephalopathy
Treat uremic pericarditis
- By dialysis.
R:
Treatment of renal osteodystrophy, if present:
The underlying biochemical abnormalities are: PO4-, Vit-D, Ca, PTH.
PO4-:
1. PO4- restriction
2. PO4- binding agents:
o Calcium agents (Ca-carbonate/ Ca-acetate)
o Non-calcium agents (Sevelamer).
Vit-D:
Cholecalciferol/ Calcitriol.
Ca:
Ca-salts
PTH:
Calcimimetics: Cinacalcet.
E:
1. Treat any electrolyte imbalance:
Electrolyte imbalance
Treatment
Na+
Fluid restriction
11
Diuretics
K+
Restriction of dietary K+ intake
Regular monitoring of serum level
IV Ca-gluconate
Counteract cardiac toxicity
Severe K+
IV Dextrose + Insulin
Shifts extracellular K+ to
OR
intracellular compartment
Nebulized salbutamol
Dialysis
2. Treatment of end stage renal disease:
a. Dialysis:
o Hemodialysis
o Peritoneal dialysis.
b. Kidney transplantation.
Anemia in CKD
Pathogenesis/ mechanism:
1. Erythropoietin (EPO) synthesis
2. Marrow suppression by azotemic toxins
3. RBC life span
4. Coexistent iron deficiency
5. Loss of folic acid (particularly in patients on dialysis)
6. Blood loss due to coagulopathy.
Clinical features:
A. Asymptomatic
B. If symptomatic:
A. Anemic look
B. Breathlessness
C. Cardiac palpitation
D. Dizziness
E. Exercise intolerance
F. Fatigue.
12
Investigation:
1. Full blood count:
o Hb:
o Normochromic anemia.
2. Serum iron/ ferritin/ B12/ folic acid should be checked to rule out any coexisting
deficiency.
Treatment:
Symptomatic treatment:
1. Packed cell transfusion
2. Correct any underlying iron deficiency: Usually IV iron supplementation is given
3. Long term s.c. EPO injection
4. Look for any other causes of anemia.
Acute kidney injury (AKI)
Definition:
Any 1 of the following:
1. Serum creatinine increases by 0.3 mg/dL in 48 hours
2. Serum creatinine increases by 1.5 times of baseline in last 7 days
3. Urine output 0.5 mg/kg/hr for 6 consecutive hours.
Stages:
Stage
Increase in serum creatinine
Urine output
1
1.5 times of baseline
0.5 ml/kg/hr for 6-12 hr
2
1.5-2.9 times of baseline
-Do- for 12 hr
3
3 times of baseline
-Do- for 24 hr
Oliguria:
Urine output 100-500 ml in 24 hours
Urine output 0.5 ml/kg/hr for at least 6 hours.
Anuria:
Urine output <100 ml in 24 hours.
13
Causes of AKI:
1. Pre-renal causes:
Hemorrhagic shock (massive bleeding)
Hypovolemic shock (severe dehydration)
Cardiogenic shock (MI, acute LVF, acute RVF)
Fluid sequestration (septicemic shock, acute pancreatitis)
Septicemic shock (abnormal peripheral vasodilatation)
Severe burn ( Insensible loss).
2. Intrinsic renal causes:
I.
Acute glomerular diseases:
RPGN
MPGN
IgA nephropathy.
II.
Acute tubular necrosis:
Ischemic (pre-renal causes)
Toxins (endogenous and exogenous).
III.
Acute interstitial nephritis:
Iatrogenic (-lactam antibiotics)
Idiopathic
Infection (CMV).
IV.
Renal vasculitis:
Microangiopathic hemolytic anemia
DIC.
3. Post-renal causes:
An obstructive uropathy can cause AKI, particularly if there is single functioning
kidney:
Calculus
Carcinoma
Bladder outlet obstruction
Structure.
Acute tubular necrosis (ATN)
Acute tubular damage leading to acute malfunctioning of kidney.
14
Causes:
ATN
Ischemic causes
Toxins
Endogenous
Exogenous
Pre-renal causes
toxins
toxins
Hb: Mismatched
Complicated
Drugs
Snake bite
blood transfusion
malaria
Myoglobin:
Aminoglycosides
Crush injury/
rhabdomyolysis
Radiocontrast dye
Urate crystals:
Hyperuricemia
Pathophysiology:
AKI
Azotemia
BP
Blood chemistry
Circulatory
BP due to
Hypovolemia
Hypervolemia
Uremic
Urea creatinine
(due to
(due to
underlying pre-renal
underlying pre-
impaired fluid
causes
renal causes)
excretion)
Constitutional
Na+: N//
symptoms
Pulmonary
congestion
Encephalopathy
K+:
Systemic congestion
Pericarditis
Metabolic acidosis
15
Clinical features:
Cause
Clinical features
Azotemia
1. Constitutional symptoms:
Anorexia
Nausea
Refractory hiccup
Metallic taste in mouth
Wasting of the muscles.
2. Features of encephalopathy:
Altered sensorium
Behavioral changes
Confusion/ convulsion/ coma
Delirium
Flapping tremor
Low GCS.
3. Features of pericarditis:
Pericardial rub.
Kidney dysfunction
1. Low urine output:
Oliguria/ anuria/ dark coloured urine
Note: Some patients of AKI never develops oliguria/
anuria. They are called non-oliguric AKI.
Seen in: acute interstitial disease and in some cases,
acute tubular disease.
2. Volume status:
Hypovolemia:
H/O blood loss/ volume loss/ condition causing fluid
sequestration
Dry mucous membrane
Skin turgor
Hypotension
Tachycardia
Capil ary refil time (2 sec).
Hypervolemia:
Symptoms and signs of pulmonary and
systemic venous congestion.
16
Stages of progression of AKI:
Stage
Description
Stage of progression
Due to sudden assault on kidney, GFR abruptly decreases
which in turn leads to urinary output and accumulation
of excessive salt, water and toxins
Stage of maintenance
Falling GFR reaches its lowest limit leading to a full blown
picture of AKI
Stage of recovery
With treatment, kidney starts to regain its function. Often
in this stage, recovery of tubular function (salt and water
retention) lags behind recovery of glomerular function.
Patient goes into polyuric phase temporarily.
Investigations of AKI:
1. Full blood count
2. Urea creatinine:
=
2.8
<20
Is strongly suggestive of ATN.
>20
May be found in both pre-renal/ post-renal causes of AKI.
3. Electrolyte:
o Na+: Variable (depends upon the degree of fluid and solute loss)
o K+: Normal/ (hypokalemia may occur in diarrhea/ overdiuresis).
4. Blood gas analysis:
Peripheral
Metabolic Acidosis
BP
Cardiac arrhythmia
vasodilation
17
5. Urine routine examination/ microscopic examination:
Often gives vital clue of underlying causes.
Ex.:
Tubular cast is suggestive of ATN
RBC cast ? Dysmorphic RBCs is suggestive of GN.
6. KUB-USG:
To look for any obstructive uropathy.
7. Chest X Ray:
To look for any pulmonary edema.
8. ECG:
To rule out any cardiac pathology due to hyperkalemia.
9. Echocardiogram:
IVC collapsibility/ fullness indicates volume overload and volume overloaded
state respectively.
10. Investigation(s) to assess the underlying disease.
If cause is not obvious, patients are often investigated for:
Underlying glomerulonephritis: Autoantibody markers
Endogenous toxins: Serum uric acid, Serum + urinary protein electrophoresis
Renal biopsy.
Treatment
General treatment:
1. Absolute bed rest till patient is stable
2. Regular monitoring of volume status:
a. Urine output [intake-output chart]
b. Central venous pressure measurement
c. Echocardiogram: Status of IVC.
C. Treatment of the etiology
D. Nutrition:
Protein and K+ restriction.
E. Avoid all nephrotoxic drugs
F. Adjust dosage of dugs according to creatinine clearance
18
G. Look for complications:
Hypo/hyper-volemia
Dyselectrolytemia
Infection.
Specific treatment:
1. Treatment of fluid imbalance:
Hypovolemia
IV fluid resuscitation
If required: Packed cell transfusion
Vasopressor: Noradrenaline/ Adrenaline
Forced diuresis: If urine output does not improve in spite of adequate fluid
resuscitation; desperate attempt can be tried by giving IV Furosemide high
dose + Osmotic diuretics (Mannitol).
Hypervolemia
Salt and water restriction
Loop diuretics:
o IV: Furosemide
o Oral: Furosemide/ Torsemide/ Metolazone.
2. Treatment of acidosis:
Mild to moderate: IV NaHCO3
Severe: Emergency diuretics.
3. Infection:
Treat with antibiotics.
4. Treatment of uremic complications (if present)
5. Treatment of electrolyte imbalance:
Na+ imbalance: Treatment depends upon the underlying cause
Hyperkalemia:
o K+ restriction
o If it becomes dangerous, any of the following may be attempted:
IV Calcium gluconate
IV Dextrose + Insulin
Nebulized salbutamol
Dialysis
Indications of dialysis:
i.
Uremic manifestations
ii.
Refractory volume overload
19
iii.
Significant acidosis
iv.
Severe hyperkalemia.
Nephrotic syndrome
Introduction:
It is a condition characterized by:
1. Heavy proteinuria (>3 gm/24 hr)
2. Hypoalbuminemia
3. Hyperlipidemia.
Causes:
Glomerular causes
Primary
Secondary
Minimal change
Diabetes
Amyloidosis
Drugs
HIV nephropathy
disease
MPGN
Gold
FSGS
Penicillamine
MPGN
Physiological effects:
1. Hypoalbuminemia:
Due to increased tubular permeability causing heavy proteinuria.
20
2. Hyperlipidemia:
I.
Accelerated lipid synthesis
II.
Loss of lipid metabolizing protein.
3. Hypercoagulability:
Due to loss of circulating anticoagulant antithrombin.
4. Hypervolemia:
Colloidal osmotic
Hypoalbuminemia
pressure
Fluid leakage
Intravascular
Renal
Activity of RAS
volume depletion
hypoperfusion
system
Secondary
Na+ and water
Hypervolemia
hyperaldosteronism
retention
5. Hormonal disturbance:
Due to loss of hormone binding proteins, causing:
Hypothyroidism
Hypovitaminosis-D.
6. Hypo-gamma-globulin-emia:
It makes the patient prone to infection.
Clinical features:
Symptoms:
Progressive swelling of body
Recurrent infection particularly in children
Patient may develop unprovoked DVT; particularly renal vascular thrombosis,
causing acute loin pain ? hematuria.
Signs:
Edema: ++
21
Puffy/ swollen face with Periorbital edema.
Ascites: ++
Scrotal swelling
Signs of pleural effusion: Bilaterally seen.
- Signs and symptoms of underlying disease may be present.
Investigation:
1. Blood:
Full blood count
Urea-creatinine: Derangement in renal function is often absent
Na+: Normal/ (due to dilutional hyponatremia)
Liver function test:
o Albumin:
o Globulin:
o Liver enzymes: Normal
Fasting lipid profile: May be deranged as a long term complication.
2. Urine (routine and microscopic examination):
Protein: ++
24 hour urinary albumin: >3 gm.
3. ECG and Echocardiogram:
To assess cardiac function and status.
4. Investigations to diagnose underlying cause:
In primary glomerular diseases, renal biopsy confirms the diagnosis.
However, in clinical life, often diagnosis is presumed to be MCD (minimal change
disease) and MPGN (Membranoproliferative glomerulonephritis) in pediatrics
and adult age groups respectively.
Treatment:
Supportive treatment:
1. Dietary salt and fluid restriction
2. Protein restriction is not needed unless kidney function is deranged
3. Loop diuretics
4. Prophylactic anticoagulation is often instituted. The agent of choice is Warfarin.
5. If dyslipidemia is found: then statins are prescribed.
22
Definitive treatment:
Depends upon the cause. However, in many glomerular diseases, the following drugs
may be prescribed:
Corticosteroid: Often patient needs long term steroid treatment
Steroid sparing agents:
o Mycophenolate mofetil
o Levamisole
o Rituximab.
Glomerulonephritis
Introduction:
Inflammation of the glomerulus which may affect podocytes/ glomerular basement
membrane/ endothelium.
23
Types:
Glomerulonephritis
Nephritic pattern (Hematuria + Hypertension)
Nephrotic pattern
Immune complex
Pauci-immune
Minimal change
mediated
disease
disease
Vasculitis (Small and
Post infectious GN
Membranous GN
medium vessel disease)
MPGN
ANCA +Ve
ANCE -Ve
MPGN
Wegener's
Goodpasture
IgA nephropathy
FSGS
granulomatosis
syndrome
Microscopic
HS purpura
Amyloidosis
polyangitis
Churg?Strauss
Lupus nephritis
HIV nephropathy
syndrome
Hepatitis B & C
Clinical features:
Depends upon the predominant pattern:
In case of nephritic pattern
1. Hematuria (may be microscopic)
2. Progressively increasing BP
3. Constitutional symptoms: Fever, malaise, weakness. Weight loss
24
4. Often anemia is seen
5. AKI/ CKD can occur in some of the underlying types of GN.
In case of nephrotic pattern
1. Progressive swelling
2. Edema +ve , Ascites +ve
3. AKI/ CKD may occur depending upon the underlying lesion.
In all patients of suspected GN, particularly those coming with nephritic pattern, an
underlying connective tissue disorder/ vasculitis must to be looked for.
Look for the following symptoms and signs:
1. CNS manifestations: Headache/ focal neurodeficit (suggestive of vasculitis)
2. Eye symptoms: Red/ gritty/ painful eyes (suggestive of connective tissue
disorders*)
3. ENT manifestations: Wegener's granulomatosis
4. Lungs: Look for pleurisy/ pneumonitis/ acute shortness of breath (suggestive of
Goodpasture syndrome); H/O asthma (suggestive of Churg?Strauss syndrome)
5. Intestinal symptoms: GI bleed, Post-prandial pain (suggestive of ischemia)
6. Vasculitic rash (suggestive of vasculitis)
7. Arthralgic manifestations (suggestive of rheumatoid arthritis).
[* Particularly, Sjogren's syndrome]
Investigations:
A. Blood:
1. Full blood count:
Hb: (due to hematuria/ chronic inflammation)
WBC: (due to chronic inflammation)
Platelet: Normal
ESR/ CRP: (due to chronic inflammation).
2. Renal function:
Urea creatinine Na+ K+ (to rule out renal derangement)
3. Serum albumin: (in nephrotic spectrum of diseases)
4. Autoimmune markers:
ANCA: +Ve in:
i.
Wegener's granulomatosis
ii.
Microscopic polyangitis
iii.
Churg-Strauss syndrome.
25
Anti-GBM-antibody: +Ve in: Goodpasture syndrome
Antinuclear antibody (ANA): +Ve in: SLE; to confirm: Anti-ds-DNA
ASO titre: in PSGN.
5. Viral serology:
Anti-HCV
Anti-HIV.
B. Urine (routine and microscopic examination):
1. RBC: +Ve
2. RBC cast
3. Dysmorphic RBC
4. Proteinuria: Nephrotic range/ sub-nephrotic range.
C. 24 hour urinary albumin estimation
D. USG kidney: To assess kidney architecture
E. ECG and Echocardiogram: To assess cardiac function
F. Special tests:
Nature of which depends upon preliminary diagnosis.
If the cause of GN is obscure, patient often undergo renal biopsy to find out the
exact type of disease.
Treatment:
A. Supportive treatment:
BP control: Antihypertensives
If edema present: Diuretics.
B. Definitive treatment:
Depends on exact type of underlying disease. Many patients are treated with
corticosteroid/ cytotoxic/ immunosuppressive drugs.
Ex.:
Mycophenolate mofetil
Rituximab
Azathioprine.
C. Appropriate treatment of AKI/ CKD (if develops).
26
Post-infectious GN
Glomerulonephritis triggered by an infectious agent.
Causes:
1. -hemolytic streptococcal infection (LRTI)
2. Staph.aureus (Infective endocarditis/ Lung abscess)
3. Rarely, post-viral/ post-fungal.
Clinical features:
Preceding/ ongoing features of bacterial infection often present
In case of PSGN, there is often a recent H/O severe LRTI: sore throat, cough etc.
GN typically starts with hematuria along with progression hypertension. In PSGN,
there is almost always an asymptomatic period between LRTI and onset of
hematuria.
Kidney dysfunction very rare.
Investigation:
1. Full blood count
2. Urea creatinine Na+ K+
3. ASO titre
4. ESR/ CRP
5. Urine: Routine and microscopic examination
6. USG KUB
7. Renal biopsy.
Treatment:
Supportive:
1. Salt restriction
2. Control of hypertension
3. Antibiotic to clear any residual infection (although its role is doubtful).
27
IgA nephropathy/ Berger's disease
Immune complex mediated GN which commonly occurs in children.
Clinical features:
Recent history of LRTI: Fever, sore throat, cough often present
GN: Mostly present with hematuria and progressive hypertension. Usually, no
latent period between LRTI and onset of hematuria is present.
Renal dysfunction not common.
Investigation:
1. Full blood count
2. Urea creatinine
3. ASO titre (not raised)
4. Urine: R/E and M/E
5. USG KUB
6. Renal biopsy: Shows mesangial IgA deposition.
Treatment:
Supportive: Control of hypertension.
Henoch-Schonlein Purpura (HSP)
It is a type of small vasculitis affecting children.
Clinical features:
H/O preceding episode(s) of URTI/LRTI
Hematuria due to GN
Skeletal change: Arthralgia
Small bowel ischemia: Leading to abdominal pain and melena
Purpura: Typically small purpuric spots appear extensively over the buttocks and
posterior compartment of lower limb; usually palpable purpura.
Investigation:
A. Blood:
I.
Full blood count
II.
Urea creatinine: Permanent renal dysfunction is rare
III.
ASO titre: Not raised
28
IV.
ESR/ CRP:
B. Urine: R/E and M/E
C. USG
D. Renal biopsy: Typically shows deposition of IgA and C3
E. Skin lesion shows: Leukocytoclastic vasculitis.
Treatment:
Supportive:
I.
Salt restriction
II.
Control of hypertension
Definitive: In severe cases; Rituximab/ Plasma exchange may be offered.
Minimal change disease (MCD)
Commonest nephrotic pattern glomerulopathy in children; rarely may occur in adults.
Clinical features:
Typically presents with features of nephrotic syndrome:
Progressive swelling
Edema: +Ve
Ascites: +Ve
Signs of bilateral pleural effusion: +Ve
Hypertension may occur.
Investigations:
A. Blood:
I.
Full blood count
II.
Urea creatinine: Mostly normal
III.
Na+ : (dilutional hyponatremia)
IV.
K+: Normal
V.
Serum albumin: Typical y .
B. Urine (R/E and M/E):
24 hour urinary protein >3 gm
C. Renal biopsy: Typically shows loss of foot processes (effacement) of podocytes.
Rarely required, as mostly the disease is presumed.
29
Indications of renal biopsy:
I.
Steroid resistant cases (proteinuria doesn't significantly decreases even
after 4 weeks of corticosteroid)
II.
Steroid dependent cases.
Treatment:
A. Supportive: Salt and fluid restriction, Diuretics.
B. Definitive:
I.
Corticosteroids
II.
Steroid sparing agents (Mycophenolate mofetil).
Membranous nephropathy
Commonest nephropathy in adults.
Causes:
1. Mostly idiopathic: Immune complex deposition occurs in glomerular capillary wall
2. Secondary causes:
I.
Malignancy (Lung CA)
II.
Infection (HepB, Syphilis)
III.
Drug induced (Captopril).
Investigation:
Same as above.
Renal biopsy: Shows deposition of IgG + C3.
Treatment:
A. Supportive:
I.
Salt and fluid restriction
II.
Diuretics
III.
Control of hypertension (ACE-I/ ARB).
B. Definitive:
I.
Cyclophosphamide
II.
Cyclosporine
III.
Chlorambucil.
30
Urinary tract infection (UTI)
Infection anywhere in the urinary passage.
Site of infection:
1. Kidney: Pyelonephritis
2. Urinary bladder: Cystitis
3. Urethra: Urethritis.
Organisms:
E.coli
Klebsiella
Proteus
Enterococci
Pseudomonas.
Clinical features:
A. Constitutive symptoms:
Fever ? chill and rigor, malaise
Anorexia + nausea
Vomiting (Pyelonephritis -> Fever-> Loin pain + Vomiting)
Confusion, drowsiness (particularly in elderly patients).
B. Urinary symptoms:
Dysuria: Burning sensation during micturition
Mild hematuria
Suprapubic pain (cystitis)
Symptoms due to prostatitis: Urgency, frequency
Kidney: New onset loin pain (pyelonephritis)
Urinary incontinence (often occurs in elderly population).
Investigation:
1. Blood:
WBC count: (predominantly neutrophils)
ESR/ CRP: .
2. Urine analysis (R/E and M/E):
Pus cells: +Ve (it is called significant pyuria when cell count > 7-10/ HPF)
Protein: +Ve
RBC: +Ve
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Microorganism may be seen.
3. Urine culture and sensitivity:
Significant bacteruria is said to be present when no. of CFU > 105/ml.
Special note on drug sensitivity:
I.
Usually the organisms are sensitive to Cotrimoxazole/ Nitrofurantoin.
II.
ESBL (Extended spectrum -lactamase) producing organisms: These
organisms are usual y resistant to traditional -lactams (including
fluoroquinolones) and sensitive to Carbapenem.
III.
Carbapenemase producing organisms: These organisms are sensitive to
Aztreonam.
4. Imaging studies:
Females with recurrent episodes of UTI/ males after 1st episode of UTI should
ideally be investigated for any underlying structural/ functional abnormality of
urinary passage. Therefore, they should undergo an USG-KUBP.
Treatment:
Antibiotics:
Empirical antibiotics:
Choice of drug:
Depends upon:
1. Severity of infection
2. Any previous urine culture-sensitivity report.
Any of the following drugs:
1. Fluoroquinolone: Ciprofloxacin/ Levofloxacin/ Ofloxacin
2. Cotrimoxazole
3. Nitrofurantoin.
In severe cases, if there is previously documented result of ESBL +Ve organisms:
1. Ertapenem
2. Meropenem + Imipenem
All empirical drugs administered should be modified according to urine culture-
sensitivity report.
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Duration of treatment:
Usually 5-7 days, however, in pyelonephritis, at least 10-14 days.
Preventive measures:
Personal hygiene
Plenty of fluid
Any structural/ functional defect should be treated:
Ex:
Urethral stricture
Bladder outlet obstruction
Vesico-ureteral reflux.
`
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Vasculitis
A group of disorders characterized by inflammation of wall of the vein, arteries and
capillaries.
Classification:
Vasculitis
Large vessel
Medium vessel
Small vessel
Giant cell arteritis/
Polyarteritis
Connective tissue
ANCA +Ve
ANCA -Ve
Temporal arteritis
nodosa (PAN)
disorders (CTD)
Wegener's
Anti-GBM
Takayasu disease
SLE
granulomatosis
antibody +Ve
Churg-Strauss
Goodpasture
Rheumatoid
disease
syndrome (GPS)
arthritis
Microscopic
Mixed connective
polyangitis
tissue disorders
HSP
A vasculitis can be a primary/ secondary vasculitis due to an underlying connective
tissue disorder/ malignancy/ infection/ drug induced.
Approach to a patient of vasculitis
Principle:
1. To suspect vasculitis even if there is no obvious manifestation of it
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2. To look for systemic manifestations
3. To look for organ specific manifestations
4. To look for underlying disease.
History:
1. Systemic manifestations:
Low grade fever
Loss of appetite
Loss of weight
Lassitude.
2. Organ damage:
Look for the following histories:
Organ/ system
Manifestations of damage
Inference
CNS
Headache/ seizure/ TIA/ stroke*
Stroke in a young patient:
always suspect vasculitis
Eye
Red eye/ gritty eye/ painful eye
Connective tissue disorders
ENT
Blocked nose/ epistaxis/ crusting/
Wegener's granulomatosis
ulceration/ ear infection
CVS
Angina (coronary vasculitis)
Takayasu's disease
Asthma
Churg-Strauss disease
Respiratory
Sudden breathlessness ? Hemoptysis
Goodpasture syndrome
Recurrent pneumonia
Connective tissue disease
GIT
Melena/ Post-prandial pain
Intestinal ischemia
Renal
Hematuria/ history suggestive of AKI/ CKD
-
Skin
Skin rash
Vasculitic rash
Joint
Joint pain ? Swelling
Rheumatoid arthritis
- Above questions are asked to screen for any organ involvement. Distribution
of organ involvement depends upon the underlying type of vasculitis.
3. Underlying cause:
Infection (infective endocarditis/ meningococcemia/ typhoid fever)
Connective tissue disease
Malignancy
- To be ruled out if possible.
Examination:
Organ/ system
Look for:
Eye
Any evidence of uveitis
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ENT
Crusting/ granulations/ septal perforation
Skin
Skin rash: Palpable purpura; Petechiae: suggestive of cutaneous
vasculitis. Typical rash for CTDs should be looked for.
CVS
Signs of pericarditis/ pericardial effusion (suggestive of CTD)
Respiratory
1. Pleurisy/ pleural effusion: CTD
2. Widespread crepitations: GPS
3. Focal signs of pneumonia: CTD
Joint
Small joint arthritis: CTD
Investigations:
BLOOD
A. Routine investigations:
1. Ful blood count: Hb (due to chronic inflammation/ ongoing hematuria/ GI
bleed/ alveolar hemorrhage)
2. WBC: (often in SLE)
3. Platelet: Normal
4. ESR/ CRP:
B. Blood culture:
To rule out any underlying bacterial infections.
C. Urea-creatinine: To assess any renal derangement
D. Serum auto antibodies:
ANCA (Anti-neutrophil cytoplasmic antibody):
It is of 2 types:
I.
c-ANCA (Cytoplasmic pattern):
Target antigen: Proteinase-3 (PR3)
+Ve in:
Wegener's granulomatosis.
II.
p-ANCA (Perinuclear pattern):
Target antigen: Myeloperoxidase (MPO)
+Ve in:
Microscopic polyangitis
Churg-Strauss syndrome.
Anti-GBM antibody:
+Ve in Goodpasture syndrome.
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Anti-nuclear antibody:
+Ve in SLE (Confirm with Anti-ds-DNA).
Anti-CCP antibody/ RA factor:
+Ve in Rheumatoid arthritis.
URINE
Routine and microscopic examination of urine: To look for:
RBC
RBC cast
Dysmorphic RBC.
BIOPSY
To confirm diagnosis.
Common site of taking biopsy: Kidney, skin.
IMAGING
Chest X-Ray: To look for nodules/ cavity (suggestive of Wegener's)
If positive: Do a CE-CT chest.
Echocardiogram
To look for infective endocarditis.
USG-KUB
To look for any structural / functional abnormality of urinary passage.
Treatment:
Specific treatment depends upon the type of vasculitis; however, the mainstay of
treatment are:
1. Corticosteroid
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2. Immunosuppressants:
Cyclophosphamide
Azathioprine.
Wegener's granulomatosis
Vasculitis characterized by necrotizing granulomatous inflammation of vessels.
Clinical features:
System
Manifestations
ENT
Recurrent episodes of epistaxis/ nasal crusting/ nasal ulceration
Recurrent otitis media
Recurrent septal perforation.
Eye
Uveitis
Lung
Multiple cavitations/ nodules/ cavitary pneumonia
May be asymptomatic/ may cause breathlessness/ productive cough.
Kidney
Glomerulonephritis Hematuria ? Hypertension
Limbs
Recurrent DVTs
Investigations:
1. Blood:
Hb:
WBC:
ESR/ CRP:
2. Urea creatinine: To rule out renal dysfunction
3. c-ANCA: Characteristically +Ve and a high titre is often a predictor of severe
disease.
4. Urine R/E and M/E: Look for RBC cast and dysmorphic RBCs.
5. CXR ? CECT chest
6. Biopsy:
Site: Lung, kidney.
Goodpasture syndrome
Vasculitis characterized by acute reno-pulmonary involvement.
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Clinical features:
1. Pulmonary: Typically abrupt onset severe intra-alveolar hemorrhage; sudden
shortness of breath, productive cough; often blood mixed/ frank hemoptysis.
2. Renal: RPGN: Hematuria, hypertension.
3. Often patient becomes significantly anemic.
Investigation:
1. Full blood count:
Hb:
WBC:
ESR: .
2. Anti-GBM antibody: +Ve
3. CXR: Diffuse bilateral alveolar opacities (resembling cardiogenic/ non-cardiogenic
pulmonary edema)
4. CECT chest: If possible
5. Urine (R/E and M/E): Look for RBC, RBC cast, Dysmorphic RBC.
Treatment:
1. Supportive: Ventilatory supports
2. Definitive: Corticosteroid, Cyclophosphamide.
Churg-Strauss syndrome
ANCA +Ve small vessel vasculitis.
Clinical features:
1. Background H/O asthma often present which is often difficult to control
(however, cases may also develop in non-asthmatics)
2. Mononeuritis/ Mononeuritis multiplex: Rapid damage of a motor neurone;
causing sudden foot drop/ ocular cranial nerve paralysis
3. Eye: Scleritis/ Episcleritis/ Uveitis
4. Skin: Rash/ vasculitic rash
5. Kidney: Usually spared.
Investigation:
1. Full blood count: Eosinophilia
2. Autoantibody: p-ANCA +Ve.
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Treatment:
Corticosteroid ? Cyclophosphamide.
Lupus nephritis (LN)
Renal complication of SLE.
Clinical features:
1. Usually presents with nephritic pattern of disease: hematuria, hypertension
2. Renal dysfunction: Gradual/ rapidly progressive in nature.
Types:
Grade
Name
Grade 1
Minimal mesangial
Grade 2
Mesangio-proliferative
Grade 3
Focal
Grade 4
Diffuse
Grade 5
Membranous
Grade 6
Advanced
Each stage can be further divided into:
Acute (A)
Chronic (C)
Acute on chronic (A/C).
Investigation:
1. Urea creatinine
2. Urine (R/E and M/E):
Look for RBC, RBC cast, Proteinuria, Dysmorphic RBC.
3. Serum autoantibodies:
ANA
Anti-ds-DNA.
4. Renal biopsy.
Treatment:
1. Supportive: For renal dysfunction
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2. Definitive:
Corticosteroid
Immunosuppressants:
Cyclophosphamide
Mycophenolate
Azathioprine
Tacrolimus.
Beh?et's disease
Small and medium vessel vasculitis which has no diagnostic test.
Clinical features:
1. Recurrent painful oral aphthous ulcer
2. Recurrent painful genital ulcer
3. Skin:
Erythema nodosum like lesion
Follicular skin rash
Pathergy: Spontaneous blistering of venipuncture site.
4. Eye: Posterior uveitis
5. CNS:
Seizure
Cranial nerve palsy
Meningitis.
6. Recurrent DVT (hypercoagulable state).
Investigation:
No diagnostic test available.
Treatment:
1. Corticosteroid
2. Colchicine
3. Thalidomide
4. Immunosuppressants:
Rituximab
Cyclophosphamide.
This post was last modified on 01 September 2021