f Kuliah-Nephritic Syndrome-FK UiSU
description
Transcript of f Kuliah-Nephritic Syndrome-FK UiSU
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Location and Structure
Details of a Glomerulus - The Membrane for Filtration
Detail of the Capillary Wall
Capillary Wall- Filter-
ProximalTubule
AfferentArteriole
EfferentArteriole
CapillaryLoops
Bowman’sCapsule
Inside ofthe Capillary - Blood Side -
Outside ofthe Capillary- Urine Side -
Healthy Kidney Renal ReplacementPhysical BasisDiseased Kidney
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A picture of a normal human glomerulusagainst which to compare the various diseases.
Tufts of capillaries
Afferent arteriole
Thinbasement membranes
Bowman’s Space
Tufts of capillaries
Nuclei ofglomerular cells
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Pathological features of glomerulonephritis
Descriptive Terms
Patterns of Damage - 1
All glomeruli normal
= No glomerulonephritis
All glomeruli abnormal
= diffuse glomerulonephritis
Some glomeruli abnormal
= focal glomerulonephritis
(often secondary to
systemic disease)
One Glomerulus compared with another
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Glomerular InjuryDiffuse vs. Focal
X
X X
X
X
XX
X
X
X
X
X
Diffuse Disease Focal Disease
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Pathological features of glomerulonephritis
Descriptive Terms
Patterns of Damage - 2
Normal
= No glomerulonephritis
Damage to part
of glomerulus only
= segmentalglomerulonephritis
Damage to all parts
of glomerulus only
= globalglomerulonephritis
Within a single glomerulus
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Glomerular InjuryGlobal vs. Segmental
XX
XX
XXXX
XX
XX
XXGlobal Disease
Segmental Disease
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Nephritic Nephritic SyndromeSyndrome
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Definisi:It is a clinical pattern characterized by sudden onset of :
1. Edema (sodium and fluid retention)2. Hypertension3. Urinary changes :
a. Mild proteinuriab. Variable degree of haematuria (macroscopic or
microscopic, RBCs cast)c. Olygouriad. Mild azotemia
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Etiology :Etiology :1. Immune complex disease :
– primary• Idiopathic membranoproliferative MPGN• Mesangioproliferative GN• Berger’s disease (Ig A nephropathy)• Membranoproliferative GN
– Secondary• Infection : Post streptococcal-Infective endocarditis• Collagenic : SLE• Allergic : Henoch Schonlein Syndrome• Drug : Sulpha
2. Anti glomerular basement membrane Abs : Good pasture syndrome
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3. Pauci Immune : PAN-TTP-DIC
If the inflammatory process is severe, GN may lead to a greater than 50% loss of nephron function over the course of weeks to months such a process is called Rapidly Progressive GN and maybe associated with crescent in > 50% of the glomeruli. This of GN may occur in post infectious, IgA nephropathy, SLE and others
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Clinical Picture of Nephritic Syndrome :
Symptoms :1. anorexia, nausea & vomiting dt mucosal edema2. Red urine dt haematuria3. Head ache, epistaxis dt hypertension
Sign :1. Edema : dt salt & H2O retentionacute onset, appear in the eye lid in the morning & disappear in the afternoon to appear in the ankle in ambulated patient
Firm, pitting dt hypoproteinemiaRarely generalized & not associated with serious effusion2. Hypertension : salt & water retention3. Urine changes : oligouriahaematuria (cola colour, smoky or reddish brown urine), micro and macroscopicProteinuria < 2 g/day
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Clinical Picture of Complication:
• CP of Complication1. Circulatory congestion :Congested pulsating neck veinPulmonary edema (dyspnoe or orthopnea) dt hypervolemiaNormal circulation time or heart failure (gallop & circulation time)
2. Hypertensive encephalopathy :dt loss of autoregulation of cerebral blood flow leads to brain edemaC/P : Head ache + vomiting + blurring of vision + focal sign
3. Non Resolution :Rapid progressive GN → ARFChronic GN → CRFAsymptomatic proteinuria → which may reach the nephrotic range (≥ 3.5 g/day →nephrotic phase of nephritic syndrome
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Clinical Picture of the Etiology
• CP of the Etiology1. Clinical manifestation of Post-Streptococcal GN (PSGN) :a. Patients presenting with Acute Nephritic Syndrome, often have evidence of a recent infection of the pharynx or skin with group A B-haemolytic Streptococci especially type 12b. Age : Children and adolescencec. This may occur sporadically or in epidemicsd. It commonly appears after pharyngitis or impetigo within 1-3 weeks after infection (1-2 weeks in pharyngitis & 2-4 weeks in impetigo)
2. Clinical manifestation of IgA Nephropathya. IgA Neph (Berger’s disease) is a primary renal diseases of IgA deposition in the glomerular mesangium → proliferative GNb. It is a common seen in children and young adultsc. Recurrent macroscopic haematuria is the most frequent clinical presentation
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Clinical Picture :•
d. It is frequently associated with : - URTI (follow by haematuria 1-2 days after onset
or URTI) - Gastrointestinal symptoms or flu like illnesse. Rarely, it presents with microscopic haematuria,
proteinuria & progressive RFf. Rapidly Progressive RF is unusual. It may results
from ATN as consequence of macroscopic haematuria or superimposed crescentic nephritis
g. Recurrent IgA deposisition is common after transplantation
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IgA Nephropathy
IgA
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Clinical manifestation of Henoch Schonlein Purpura (HSP)
a. Haematuriab. Arthritisc. Abdominal painc. Purpura
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Henoch Schonlein Purpura
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Often a multi-systemic Illness• Wegeners Granulomatosis
Sinusitis Lung haemorrhageSkin rashArthritisTesticular involvementmononeuritisAny organ
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Wegener’s Granulomatosis
Cavitating Nodules
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Wegener’s Granulomatosis
Haemorrage
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Wegener’s Granulomatosis
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Wegener’s Granulomatosis • c ANCA associated disease• Microscopic polyarteritis p ANCA assoc
disease• Untreated 80% mortality• Cyclophosphamide and Pred +/- plasma
exchange• Treated 80% cured, 10% resistant disease,
10% die from side effects of the drugs
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ArthritisSkin rashHair lossAnaemia, thrombocytopenia, leucopeniaPleuritis, pericarditisEye inflammationMouth ulcers
Systemic Lupus Erythematosis
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Lupus Nephritis• Auto-immune disease• 7:1 female to male • Classical treatment
Cyclophosphamide and prednisolone. • Good recent evidence for
Mycophenolate mofetil• Hardly ever cured usually just get it
under control
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Lupus Rash
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Lupus Nephritis
Diffuse proliferative GN (grade IV) Wire loops
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Lupus Nephritis• Grade I Normal• Grade II mesangial proliferative GN• Grade III focal proliferative GN• Grade IV Diffuse Proliferative GN• Grade V Membranous GN• Grade VI diffuse scarring
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Goodpastures
IgG and C3
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Goodpastures
• Autoantibody directed against the basement membrane of the Glomerulus and Alveolus
• Pulmonary Haemorrhage and Crescenteric acute renal failure
• Plasma exchange, steroids and cyclophosphamide
• Rare to recover renal function. Can usually save their lives
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Investigations :A. Urine analysis :• Physical
• Microscopic or gross haematuria (smoky)• Turbid (proteinuria)• Oligouria• specific gravity
• Biochemical : proteinuria < 3 g/day• Microscopic :
• Dismorphic (deformed) RBCs (Glomerular origin)• Hyaline & tubular epithelial casts• RBCs cast (Diagnostic)• Granular casts
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Investigations :B. Blood Chemistry :• Anaemia• Creatinine is usually normal• BUN maybe increased disproportionately• Serum albumin is often (dilutional + urinary
loss) Na →dilutional due to volume overload• Hypercholesterolemic acidosis with mild K
C. Renal Imaging : US : Mild swelling of kidneys or Normal
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Investigations : (con’t)D. Renal Biopsy :• Indicated if there is atypical presentation or there is
severe azotemia or anuria or persistenly low C3 or persistent proteinuria > 6 months
• Light microscopy :– Show diffuse proliferative GN (mesangial cell proliferation,
infiltration with PMN, monocyte in capillary lumina, mesangium). Severe disease may show crescent in urinary space.
• Electron microscopy :– Show large dense sub epithelial deposits or humps
• Immunofluorescence (IF) :– Show IgG and C3 in a granular pattern in the mesangium and
along the capillary basement membrane
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Investigations : (con’t)D. Serological test :
• In PSGN1. serum complement (CH50, C3). C3 returns to normal level
within 6-8 weeks of the disease. Persistently low C3 suggest MPGN, lupus or endocarditis
2. Anti Streptolysin O titers (ASOT) unless antibiotic treatment was
taken. It starts to rise 10 – 14 days after pharyngeal infection,
peaks at 3-4 weeks then declines maybe till 6 months3. Throat or skin culture for Group A Stretococcus
• In Ig A nephropathy :1. Serum Ig A level is increased up to 50% of patients2. Serum complement level are usually normal
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• > 3g proteinuria• Oedema• Low serum
Albumin• Hypercholesterol• bacterial infections• increased clotting
• Blood and proteinuria• Red cell casts• Hypertension• Raised creatinine• Often systemic illness
Nephrotic Nephritic
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Treatment :Treatment :
• Treatment of the disease :General treatment
• Complete bed rest, till edema, haematuria & hypertension subside
• Diet : High CHO dietSalt & H2O restriction (in
marked oliguria and edema)Protein restriction (in case of
renal failure)K restriction (in case of
Hyperkalemia)
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• Treatment of the disease :
Symptomatic treatment
• Diuretic (Furosemide) for edema• Nifedipine, furosemide or ACE-I for hypertension• In the vast majority of patient < 1 g/day, no specific
treatment is provided• In patient with significant proteinuria > 1 g/day, ACE-
I or ARB should be used to reduce proteinuria. The target BP is < 130/80 mmHg
• Patients with progressive disease with deteriorating kidney functions → aggressive treatment including steroid, plasmaparesis or cytotoxic drugs
• Renal transplantation is an option for patients with ESRD
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• Treatment of the disease :
Treatment of the cause :A. Post Streptococcal GNPenicillin 1 million Unit/ 6 hours IV for 1 week
B. Good Pasture Syndrome :- Plasmapharesis- Pulse Corticosteroid therapy1 g methyl prednisolonemaintenance : 2 mg/kg/ every other day- Mini-pulse corticosteroid therapy : 250 mg prednisolone/day for 3
days (less toxic)
C. SLE- Pulse corticosteroid therapy- Cyclophosphamide pulse therapy- Plasmapharesis
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Treatment of the Treatment of the complication:complication:
• Heart failure :• Salt restriction• Diuretic• Small dose digitalis• Peritoneal dialysis
• Renal Failure :• Dialysis
• Hypertensive encephalopathy :• Mannitol for brain edema• Diazepam for convultion
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Prognosis:Prognosis:
• Prognosis in children is very favourable• Adult are more prone to crescentic
formation and chronic Renal Insufficiency• Less than 5% of adults will develop a
RPGN and smaller percentage will progress to ESRD
• Patients with proteinuria < 1 g/24 h usually have good prognosis
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