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.