Kuliah Nephritic Syndrome 29jan 14
description
Transcript of Kuliah Nephritic Syndrome 29jan 14
Dr. SARTONO Sp PD
Essential Diagnosis * Hematuria * RBC cast * Variable Proteinuria * Renal Insufficiency * Salt retension ( hypertensive
nephropathy/ HTN & edema )
GENERAL CONSIDERATIONS * Glomerulonephritis intraglomerular inflama tory process & renal dysfunction acute, subacute & chronic ESRD * May be due to : - intrinsic renal disease - part of systemic disease * Classic causes : - Post infectious glomerulonephritis - IgA Nephropathy worldwide - Lupus Nephritis * Subset of acute glomerulonephritis RPGN -> Goodpasture’s disease, polyarteritis
nodosum& Wagener’s granulomatosis
PATHOGENESIS * Inflamatory process determine the severity of renal dysfunction & clin. manifestation. * Two basic mechanisms underlie glomerulopathy: - 1st , antibodies binds to structural comp. or other material implanted in the glomeruli ( circulating ab. form & directed GBM , e.g. Goodpasture’s disease ) - 2nd , circulating ag-ab complex escapes RES-> deposited in the glomeruli ( e.g. DNA- nucleosum complex -> SLE & cryoglob. -> hepatitis C related MPGN
- Other post streptococcal glomerulonephritis -> due to antigen deposition in glom. activation of complement direct tissue injury or inflama- tory reaction intrinsic glomerular cell prolifera tion ( mesangial, endothelial & epithelial cells ).
PREVENTION * Not yet known to guard intrinsic renal or systemic diseases nephritic syndrome ( Ig A nephropathy or SLE ) * Focus -> early recognition with prompt Dx & Tx
to prevent irreversible loss of renal function.
CLINICAL FINDINGS A. Symptoms & signs * can present with edema, oliguria, uremic symptoms * many with Ht , even malignant * other PE , d.o. Underlying disorders ( malar rash & oral ulcers SLE palpable purpura Henoch-Schonlen
purpura & cryoglobulinemia )
B. Laboratory Findings 1. Urinalysis * Px may present with mac. hematuria/tea or cola- colored urine * Mic. Exams, RBCs dysmorphic or mishapen ( as a result of osmotic / chemical stress as they pass through nephron ) * Urinary RBCs with membrane blebs or “buble-like” projections ( acanthocytes ) strong evidence of glomerular cause of hematuria. * RBC casts , may also be found
* Urinary protein excretion varies , but less than 3 g of protein / day * Proteinuria can be quantified by 24-hr urine collection or based on single urine specimen
2. Serum Chemistry * Chemistry profile should be obtained to estimate the GFR ( to determine the degree of renal dysfunction ) * CBC demonstrate anemia, thrombocyto penia, or leukopenia ( e.g. SLE )
* Additional test d.o. : Px’s history or PE - fever & heart murmur : blood culture - sore throats : streptozyme & ASO titer - history of IVDA / hepatomegaly : hepatitis panel &
cryoglobulins
* Other tests – - complement levels - anti neutrophilic cytoplasmic antibodies (ANCA) - anti-GBM - immune complex disease markers ( ANA &
anti DNA )
* 10% of Px with heavy proteinuria may have
negative serologic findings ( loss of Ab / tissue
deposition )
c. Imaging Studies * Chest x-ray pulmonary edema or findings
suggestive of Wagener’s granulomatosis or Goodpasture’s disease * Echocardiogram identify a pericardial
effusion or endocarditis * Renal USG renal size when decreased GFR
+ Renal size < 9 cm extensive renal scarring
low irreversibility
D. Special Tests Renal biopsy * definitive diagnosis * distinguishing – primary & sec. causes of
renal disease & subtypes in
SLE * rapid dx for RPGN ( prompt dx & tx
essen- tial in perserving renal
function. * yield info level of inflamation, extent of fibrosis & overall
prognosis.
Differential Diagnosis * Require to distinguish primary renal disease & one as result
of systemic disease. * Useful clin. Approach can be based on : - serum complement levels - serologies - immunofluorescence findings on
renal biopsy.
Complications * Fluid retension edema & hypertension * Renal Insufficiency need replacement
Tx * Anemia a result of resistance to
erythropoetin or decreased production
Treatment A. Underlying Renal or Systemic Disease 1. Immunosuppresive agents * For primary renal disease e.g. Ig A nephropathy often involves corticosteroid * Fish oil in doses of 6-12 g / day to slow
the rate of loss of renal function. 2. Cytotoxic agents * Diseases such as Goodpasture’s disease, Wagener’s granulomatosis & SLE require cyclophospamide, mycophenolate mofetil, azathioprine
3. Plasmapheresis * To remove circulating pathogenic autoantibodies , e.g. : - Goodpasture’s disease - Pauci-immune crescentic
glomerulonephritis - Cryoglobulin-related MPGN 4. Others * Tx of infections , e.g. - Hepatitis B with lamivudine - Hepatiris C with IF α and ribavirin - HIV with HAART all wil lead to substansial improvement of renal dysfunction.
- Tx of post streptococcal glomerulonephritis
is only supportive, since the use of antimicro
bial doesn’t prevent/attenuate its course.
- But , it is given to prevent spread of organism
to other susceptible hosts.
B. Complication of Nephritic Syndrome 1. Edema salt & water restriction with
diuretics 2. Hypertension – tx achieved usually with
ACEI & ARB ( have added antiprotein effect
by reducing intraglomerular cappilary
hydrostatic pressure ) 3. Renal Insufficiency if severe
progressive renal replacement tx.
Prognosis * Depends on the underlying etiology of disease * Poststreptococcal glomerulonephritis
excellent with spontaneous recovery of renal function ( 70 – 85 % of adults ). * In MPGN unfavorable spontaneous remissions are infrequent ( 50% of Px with proteinuria > 10 g / day ESRD within 7 years * Other diseases : Wegener’s granulomatosis & Goodpasture’s disease may be fatal if left untreated.