f kuliah-nephritic syndrome-fk uisu
DESCRIPTION
kedokteranTRANSCRIPT
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
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
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
Glomerular InjuryDiffuse vs. Focal
X
X X
X
X
XX
X
X
X
X
X
Diffuse Disease Focal Disease
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
Glomerular InjuryGlobal vs. Segmental
XX
XX
XXXX
XX
XX
XXGlobal Disease
Segmental Disease
Nephritic Nephritic SyndromeSyndrome
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
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
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
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
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
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
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
IgA Nephropathy
IgA
Clinical manifestation of Henoch Schonlein Purpura (HSP)
a. Haematuriab. Arthritisc. Abdominal painc. Purpura
Henoch Schonlein Purpura
Often a multi-systemic Illness• Wegeners Granulomatosis
Sinusitis Lung haemorrhageSkin rashArthritisTesticular involvementmononeuritisAny organ
Wegener’s Granulomatosis
Cavitating Nodules
Wegener’s Granulomatosis
Haemorrage
Wegener’s Granulomatosis
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
ArthritisSkin rashHair lossAnaemia, thrombocytopenia, leucopeniaPleuritis, pericarditisEye inflammationMouth ulcers
Systemic Lupus Erythematosis
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
Lupus Rash
Lupus Nephritis
Diffuse proliferative GN (grade IV) Wire loops
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
Goodpastures
IgG and C3
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
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
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
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
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
• > 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
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)
• 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
• 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
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
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