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LUPUS NEPHRITIS

Mrs G.G, 27 yr old female


referred from Northdale Hosp & admitted on 29/1/02 to
Greys Hosp via renal unit
Presenting complaints :
1.Diarrhoea, vomiting
2.haematuria
3.swelling of feet and abdomen
Past Medical History - seen at Derm Clinic(Greys) for
eczematous rash since March 2001.
Skin biopsy: feats of acute inflammatory ulcer involving
the full thickness of the epidermis. Stains for fungi and
AFB were negative. Pt treated for eczema.
GYNAE HX : Hyperemesis gravidarum in May 2000
Had NVDs in 1996 and 2000. No complications.
SURG HX : Nil
FAMILY HX : Nil of note
SOCIAL HX : Works with computrs. Married with 2 children.
No smoking or alcohol intake.
DRUG HX : Emolient cream, Phenergan 10mg bd (Derm)
EXAMINATION
Patient was comfortable and not in distress.
Generalised mild swelling of body assoc with facial
swelling. BP = PR =
Pallour present No lymphadenopathy
Chest : No resp distress. RR = 20/min. Mild bilatral
crepitations auscultated at bases.
CVS : JVP increased to 5cm. Heart sounds normal. No
murmurs auscultated. Apex beat undisplaced.
Abd : Mild ascites. Not tense. No organomegaly. Kidneys
not ballotable
CNS : GCS = 15/15. No focal signs.
SUMMARY
27 yr old female presenting with diarr, vomiting, and
haematuria x 1 day with elevated urea and creatinine
levels of
Examination revealed generalised swelling of the face
and body with ascites and crepitations of the chest - in
keeping with hypervolaemia.
Hypertension also present with findings of blood +++ in
urine dipstix and protein?
INVESTIGATIONS
FBC: Hb = 8,4 Normochromic normocytic anaemia
WCC = 4,2 Plt = 99
U and E : 136/ 4,5/ 104/ 17/ 32/ 730
LFT : ALP = 120 Tot bili = 14 Tot prot = Albumin=
ALT = Gamma GT =
Cal = 2,10 Phos = 1,7 Mg = 0.8
Specimens for complement screen, ANF, ANCA were taken
and results were pending.
24 hr urine protein = 2g/l Red cell casts present.
Renal biopsy - results pending
RADILOGICAL INVESTIGATIONS
CXR : Globular shaped heart with bilateral pleural
effusions and features of pulmoedema
ULTRASOUND KIDNEYS : Dense kidneys bilaterally
ECHO : Pericardial effusion present of about 9mm with no
tamponade effect present.
DIFFERENTIAL DIAGNOSIS
ACUTE RENAL FAILURE
? Rapidly progessive GN (RPGN) or Acute Nephritic Syn
1. Secondary glomerulopathy - Collagen vasc dx eg. SLE,
Cryoglobulinaemia
2. Post Streptococcal GN
3. Ig A Nephropathy
INITIAL MANAGEMENT
IV LASIX 40MG BD, HAEMATINICS
.
RESULTS
1. ANF - POSITIVE AT 1:3200
. ANTI HEP 2 CELLS POSITIVE
. ANTI DNA AB NEG
2. COMPLEMENT C3 - 0.22 (L)
. COMPLEMENT C4 - 0.05 (L)
. COMPLEMENT FACTOR B - 0.27
3. RENAL BIOPSY - NON-REPRESENTATIVE SAMPLE.
FINAL DIAGNOSIS : SLE NEPHRITIS IN ARF
FOLLOW UP MX
1. SOLUMEDROL 1G IVI DAILY X 3/7
2. CYCLOPHOSPHAMIDE 750mg MONTHLY IVI
3. CONTINUED ON PREDNISONE 60mg DAILY
4. INH PROPHYLAXIS, TITRALAC, PREGAMEL, VIT C, LASIX IV
01/03/02 - DIALYSIS COMMENCED - STARTED RECEIVING
HAEMODIALYSIS ABOUT 2-3 x / WEEK
LUPUS NEPHRITIS
HISTORY
- The term lupus is latin for wolf - used in the 18th
century to describe a variety of skin conditions.
- In 1872, Kaposi described the systemic nature of lupus
erythematosis.
- Descriptions of lupus nephropathy were initially repoted
bt Keith and Rowntree in 1922.
DEFINITION - SLE is a disease of unknown aetiology in
which tissues and cells are damaged by pathogenic
autoantibodies and immune complexes.
EPIDEMIOLOGY
- 90% of cases are in women - usually child-bearing age.
- Children, men and elderly can be affected
- Prevalence in the U.S varies from 15 to 50 per 100000 and
is more common amongst blacks than whites. Asian and
Hispanic populations are also susceptible.
CRITERIA FOR CLASSIFICATION OF
SLE
IF 4 OF THESE CRITERIA ARE PRESENT AT ANY TIME DURING THE
COURSE OF THE DX, A DIAG OF SLE CAN BE MADE WITH 98%
SPECIFICITY AND 97% SENSITIVITY
1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis
7. Renal disorder
8. Neurological disorder
9. Haematological disorder
10. Immunological disorder
11. Anti-nuclear Abs
CLINICAL MANIFESTATIONS OF RENAL DX
- Only one half have clinical nephritis defined by
proteinuria.
- Early in disease, most are asymptomatic, although some
develop the oedema of nephrotic syndrome.
- Urinanalysis shows haematuria, cylinduria and
proteinuria
- The revised criteria for the classification of SLE
recognizes proteinuria of > 0.5g per 24hrs (or >3+ if
quantitative evaluation is not done) or the presence of
casts as evidence of renal disease.
- In addition, the presence of haematuria (>5RBC/ high
power field) and/or pyuria in the absence of infection, and
the detection of an elevated serum creatinine has been
recognized as evidence for clinical renal disease.
PATHOGENESIS
- The pathogenesis of lupus nephritis is thought to be
similar to that of chronic immune complex
glomerulonephritis.
- The current paradigm for the pathogenesis of tissue
injury in SLE including lupus nephropathy involves two
linked components.
- The first component involved production of
autoantibodies and the deposition of pre-formed antibody-
antigen complexes in situ. These complexes acivate the
complement cascade and result in neutrophil ifiltration
and activation.
- The second component is characterized by cell
proliferation, necrosis and thrombus formation secondary
to factors released by infiltrating cells and platelets.
THE ROLE OF RENAL BIOPSY
Clinical value is controversial
Helps to direct management
- in a subset of pts with circulating anti-coagulants, the
renal pathology may be dominated by microvascular
thrombosis. Corticosteroids may have adverse effects on
these pts.
- Value of renal biopsy is greater if obtained early in dx.
CLASSIFICATION OF LUPUS NEPHRITIS
1. CLASS I
- Renal biopsy reveals an essentially normal kidney by
light, electron and immunofluorescence microscopy .
- Patients usually do not have clinical disease.
.
2. CLASS II
- Class IIa - have minimal or no significant changes by
light microscopy although there may be
immunoflourescent evidence of immune deposits
confined to the mesangium and/or dense deposits by EM.
- Class IIb - shows definite glomerular mesangial
hypercellularity by light microscopy, which is confined to
the centrilobular regions away from the vascular pole.
Clinically, these patients have mild to moderate evidence
of renal involvement and in general have a good
prognosis.
3. CLASS III
- Focal segmental proliferative lupus nephritis with
necrosis or sclerosis affecting fewer than 50% of
glomeruli.
- Up to one third of patients have nephrotic syndrome and
glomerular filtration is impaired in 15-25%.
.
4. CLASS IV
- Characterized by diffuse proliferative GN.
- Most glomeruli show cell proliferation often with
crescent formation and sometimes nuclear debris
represented by haematoxylin bodies.
- By EM, the characteristic findings are subendothelial
deposits and mesangial deposits. Sometimes, there may
be sub-epithelial and intramembranous deposits.
- Patients have a relatively severe clinical picture with
nephrotic-range proteinuria and an active sediment. A
high percentage progress to renal failure

5. CLASS V
- represented by diffuse membranous GN.
- On Light microscopy, thickening of the GBM is evident.
- EM reveals predominantly subepithelial deposits.
- Most (90%) of pts present with a nephrotic syndrome but
significant impairment of the GFR is unusual.
.
6. CLASS VI
- represents the end stages of proliferative lupus
nephritis and is characterized by diffuse glomerulo-
sclerosisand advanced tubulo-interstitial disease.
- Patients are often hypertensive, may have nephrotic
syndrome, and usually have impaired GFR.
SILENT LUPUS NEPHRITIS
- Although the renal lesion is mild in the majority of
patients with clinically silent lupus nephritis, several
reports have described severe proliferative GN in the
absence of proteinuria or abnorm of urinary sediment.
- Clinical Mx of these pts is uncertain.
CLINICAL COURSE OF SLE IN ESRD AND AFTER
TRANSPLANTATION
- Several reports indicate that even though pts may have
severe systemic dx and serological abnormalities at the
time renal dx progresses to RF, within a few months
clinical activity of non-renal SLE markedly decreases.
- A reduction in systemic manifestations and lack of
evidence of recurrent lupus nephritis have been
characteristic features of lupus pts treadted with renal
transplantation.
MANAGEMENT
Principle goals of therapy is to improve or prevent loss of
renal fxn and treatment must do the pt as little harm poss
1. Renal biopsy - if no contra-indications
2. Diet
3. Loop diuretics - to decrease oedema
4. Evaluate renal activity : urine sediment appearance,
serum creatinine, creat clearance, BP, serum alb, 24hr
urine prot, c3 complement, anti-DNA, urine dipstix.
5. Monitoring toxicity of steroids, diuretics and cytotoxic
agents : FBC, K+, gluc, chol, LFTs.
6. Aggressive Rx of Hypertension
7. Discourage pregnancy - increased risk of renal failure
8. Antimalarials if have active skin dx.
TREATMENT
1. GLUCOCORTICOIDS
2. PULSE STEROID THERAPY
3. CYTOTOXIC DRUGS
4. PLASMAPHARESIS
5. ANCROD
6. TOTAL LYMPHOID IRRADIATION
7. IMMUNOTHERAPY

PROGNOSIS - poor indicators


-Nephrotic synd - Class IV lxes
- High chronicity indices - HPT
- Childhood onset of nephritis
FOLLOW UP OF PT
Initially improved dramatically on Rx and haemodialysis
Subsequently developed Nosocomial Pseudomonas
Pneum sensitive to Ciprobay
Developed Pulm TB - proven on sputum AFB in April
Patient demised on 26/5/02
REFERENCES
1. Nephropathy of SLE - Hayslett, Kashgarian
2. Dubois Lupus Erythematosis
3. Harrisons Principles of Int Medicine

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