lupus nephritis - wits...
TRANSCRIPT
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LUPUS NEPHRITIS THE MANY FACES OF A RARE DISEASE
BY DR. LINDOKUHLE MAHLASE
23/06/2019
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CASE 1
• 13 YE AR OL D GIR L F R OM MOZAMB IQUE P R E S E NT E D TO BAS E HOS P ITAL WITH
S E IZUR E S AND HYP E R T E NS ION , S HE WAS T R E AT E D AS A ME NINGIT IS IN VIEW
OF T HE HIGH CR P AND F IT S .
• S HE WAS T HE N R E F E R R E D TO CMJAH FOR CT SCAN, WHE R E NE P HR OL OGY
WAS CONS ULT E D FOR HYP E R T E NS ION WOR K UP.
• E XAMINAT ION S HOWE D A PAT IE NT WITH VAS CUL IT IC L E S IONS , P E R IP HE R AL
OE DE MA AND HYP E R T E NS ION
UDIP S IT X : 3+ PR OT E INS , 3+ B L OOD
MICR OS COP E S HOWE D ACT IVE S E D IME NT WITH GR ANUL AR CAS T AND R B C
CAS T S
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CASE 1
• U&E : S HOWE D A PAT IE NT IN ACUT E R E NAL FAIL UR E ( HD FOR 6 WE E KS )
• L UP US WOR K UP WAS POS IT IVE
• B IOP S Y SHOWE D A CL AS S 3 L UP US NE P HR IT IS ( T R E AT E D AS SUCH )
• S HE DE FAULT E D F OL L OW UP AND DE MIS E D AT HOME
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CASE 2
• 11 YE AR OL D CAUCAS IAN GIR L WHO PR E S E NT E D WITH MIGR AINE S AND
DE P R E S S ION WITH E NXIE T Y DIS OR DE R . CL INICAL E XAMINAT ION R E VE AL E D
S IGNS IN KE E P ING WITH L UP US .
• S HE HAD NOR MAL R E NAL F UNCT ION B UT T HE UR INE DIP S T IX R E VE AL E D
NE P HR OT IC R ANGE PR OT E INUR IA
• IMMUNOL OGY WAS POS IT IVE FOR LUP US
• S HE WAS T R E AT E D AS A S E VE R E L UP US DUE TO MR I F INDING OF NE UR O
INVOLVE ME NT
• B IOP S Y SHOWE D CL AS S 1 L UP US NE P HR IT IS
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CASE 2
• DE FAULT E D FOL L OW UP WITH CMJAH AND S E L F R E F E R R E D TO P R IVAT E
NE P HR OL OGIS T WHE R E SHE IS DOING WE L L .
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INTRODUCTION
• SLE IS CHRONIC AUTOIMMUNE DISEASE AFFECTING MULTIPLE ORGAN SYSTEMS , INCLUDING
SKIN, CNS , HEART , BLOOD , KIDNEYS AND SEROUS MEMBRANES.
• RENAL INVOLVEMENT POSES THE GREATEST RISK OF MORTALITY AND MORBIDITY.
• RENAL MANIFESTATION ARE HIGHLY VARIABLE AND ARE PRESENT IN 30 — 60% OF THE
PATIENTS WITH SLE.
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PATHOPHYSIOLOGY
• NOT FULLY UNDERSTOOD BUT INVOLVES AUTOIMMUNITY AT THE CENTER OF THE DISEASE.
• THIS INVOLVES COMPLEX PROCESSES LIKE:
- LOSS OF SELF TOLERANCE
- DYSREGULATED APOPTOSIS AND INADEQUATE REMOVAL OF APOPTOTIC CELLS
- FORMATION OF ANTIBODIES THAT TARGET TISSUE-SPECIFIC ANTIGENS
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PATHOPHYSIOLOGY
• POOR CLEARANCE : PATIENTS WITH SLE HAVE POOR CLEARANCE MECHANISM FOR CELLULAR
DEBRIS , THIS INCLUDE DYSFUNCTIONAL COMPLEMENT SYSTEM.
• THIS RESULTS IN PROLONGED EXPOSURE OF SELF ANTIGEN TO THE AUTOIMMUNITY!
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PATHOPHYSIOLOGY
• IMMUNE SYSTEM VS THE KIDNEY:
1. ANTIGEN THAT ARE DIRACTED AGAINST NUCLEOSOMES CROSS REACT WITH THE
GLOMERULAR BASEMENT MEMBRANE
2. AUTOANTIBODIES HAVE HIGH AFFINITY FOR VASCULAR ORGANS LIKE GLOMERULI
3. CATIONIC AUTOANTIBODIES VS ANIONIC BASEMENT MEMBRANE
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ETIOLOGY
• GENETIS FACTORS
• ENVIRONMENTAL
• HORMONAL
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GENETIS
• GENETIC PREDISPOSITION PLAYS AN IMPORTANT ROLE IN THE DEVELOPMENT OF BOTH SLE
AND LUPUS NEPHRITIS . MULTIPLE GENES HAVE BEEN IMPLICATED WITHOUT CONCLUSIVE
EVIDENCE IN ANIMAL STUDIES .
• MONOZYGOUS TWINS 20-40 PERCENT OF DEVELOPING SLE IF ONE TWIN IS AFFECTED
• DIZYGOUS TWINS 2-5 % RISK
• HLA-DR2 AND DR3 ARE ASSOC WITH SLE
• HLADR4 APPEARS TO BE PROTECTIVE AGAINST SLE
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ENVIRONMENTAL FACTORS
• TOXINS:
- LARGACTIL
- ISONIAZID
- QUINIDINE
- HYDRALAZINE
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ENVIROMENTAL FACTORS
• INFECTIONS : EBV ( CAUSE VS ASSOCIATION )
CAUSE: ANTI RO ANRIBODIES CROSS REACT WITH EBNA-1
MICE IMMUNIZED WITH EBNA-1 ANTIGEN DEVELOP CLINICAL LUPUS SYMPTOMS
ASSOC : SLE IS AN IMMUNOSUPPRESSIVE CONDITION , COULD LEAD TO SEROCONVERSION OF
THE LATENT EBV INFECTION.
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ENVIRONMENTAL FACTORS
• UV LIGHT EXPOSURE
• SMOKING
• ALCOHOL
• SILICA DUST
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CLINICAL PRESENTATION
• ASYMPTOMATIC PROTEINURIA OR HAEMATURIA
- DIPSTIX IN ALL PATIENTS AT ALL CLINIC VISITS
- FULL WORK UP AND REFERRAL OF PATIENTS WITH ASYMPTOMATIC PROTEINURIA AND
HAEMATURIA
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CLINICAL PRESENTATION
• ACTIVE NEPHRITIS
- OEDEMA
- HEADACHE , DIZZINESS, SEIZURES
NEPHRITIS SYMPTOMS ARE USUALLY RELATED TO HYPERTENSION AND POOR RENAL
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CLINICAL PRESENTATION
• NEPHROTIC SYNDROME
- ALL PATIENTS WITH NEPHROTIC SYNDROME HAVE TO HAVE LUPUS WORK UP
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CLINICAL PRESENTATION
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INVESTIGATION
BASELINE LABORATORY:
URINE DIPSTIX
URINE MICROSCOPE
URINE PCR
FBC , DIFF , SMEAR
ACUTE PHASE REACTANTS ( CRP , ESR)
ALBUMIN
CHOLESTEROL
U&E , CMP
IMMUNOLOGY:
ANA, ANT-DS DNA, C3, C4
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RENAL BIOPSY
• INDICATIONS:
- PROTEINURIA > 500MG/DL WOTH OR WITHOUTH ACTIVE CLINICAL LUPUS DISEASE
- PERSISTENT DECLINE IN RENAL FUNCTION IN A LUPUS PATIENT
- PERSISTERNT MICROSCOPIC HAEMATURIA
- ACTIVE SEDIMENT ON MICROSCOPE ( WCC & RBC CASTS , GRANULAR )
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CLASSIFICATION OF LUPUS NEPHRITIS
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Weening J et al J Am Soc Nephrol 2004
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CLASS I – MINIMAL MESANGIAL LUPUS NEPHRITIS:
• NORMAL GLOMERULI BY LIGHT MICROSCOPY
• MESANGIAL IMMUNE DEPOSITS BY IMF/EM
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Jennette et al. Hepinstall's Pathology of the kidney, 6th Ed
Normal glomerulus Immunofluorescence microscopy shows
mesangial positivity for immunoglobulin
G
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CLASS II – MESANGIAL PROLIFERATIVE LUPUS NEPHRITIS:
• PURELY MESANGIAL HYPERCELLULARITY OF ANY DEGREE OR MESANGIAL MATRIX EXPANSION
BY LIGHT MICROSCOPY WITH MESANGIAL IMMUNE DEPOSITS
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Jennette et al. Hepinstall's Pathology of the kidney, 6th Ed
Class II: Mild, global mesangial
hypercellularity
Immunofluorescence microscopy shows
deposits of IgG confined to the
mesangium.
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CLASS III – FOCAL LUPUS NEPHRITIS:
• ACTIVE OR INACTIVE FOCAL, SEGMENTAL, AND/OR GLOBAL ENDOCAPILLARY AND/OR
EXTRACAPILLARY GN INVOLVING < 50% OF ALL GLOMERULI, TYPICALLY WITH FOCAL
SUBENDOTHELIAL IMMUNE DEPOSITS, WITH OR WITHOUT MESANGIAL ALTERATIONS
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Lupus nephritis class III;
Segmental crescent with mild
mesangial hypercellularity
Seshan & Jennette Arch Pathol Lab Med February 2009
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Jennette et al. Hepinstall's Pathology of the kidney, 6th Ed
Segmental capillary necrosis with sparing of
the remainder of the capillary tuft
Segmental rupture of the GBM with
mesangial hypercellularity
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CLASS IV – DIFFUSE LUPUS NEPHRITIS:
• ACTIVE OR INACTIVE DIFFUSE, SEGMENTAL OR GLOBAL ENDO- OR EXTRACAPILLARY
GLOMERULONEPHRITIS INVOLVING 50% OF ALL GLOMERULI, TYPICALLY WITH DIFFUSE
SUBENDOTHELIAL IMMUNE DEPOSITS, WITH OR WITHOUT MESANGIAL ALTERATIONS.
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Lupus nephritis class IV:
Endocapillary hypercellularity,
subendothelial deposits and
karyorrhectic debris
Circumferential crescent
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CLASS V – MEMBRANOUS LUPUS NEPHRITIS:
• GLOBAL OR SEGMENTAL SUBEPITHELIAL IMMUNE DEPOSITS OR THEIR MORPHOLOGIC
SEQUELAE BY LIGHT MICROSCOPY AND BY IMMUNOFLUORESCENCE OR ELECTRON
MICROSCOPY, WITH OR WITHOUT MESANGIAL ALTERATIONS
• CLASS V LUPUS NEPHRITIS MAY OCCUR IN COMBINATION WITH CLASS III OR IV
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Lupus nephritis class V. There is regular
thickening and rigidity of the
glomerular capillary walls
accompanied by global mesangial
hypercellularity
Subepithelial deposits with formation
of spikes
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CLASS VI - ADVANCED SCLEROSING LUPUS NEPHRITIS:
• 90% OF GLOMERULI GLOBALLY SCLEROSED WITHOUT RESIDUAL ACTIVITY
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Lupus nephritis class VI. Extensive glomerular sclerosis
with fibrous crescents. The global sclerosis affects more
than 90% of glomeruliJennette et al. Hepinstall's Pathology of the kidney, 6th Ed
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WHY CLASSIFY ?
ANSWER: TREATMENT
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TREATMENT
• GOAL :
- PREVENT ESRD
- ACHIEVE COMPLETE REMISSION OR MINIMAL RELAPSE EPISODES
- MINIMIZE SIDE EFFECTS
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TREATMENT
• INDUCTION
• ADJUVANT THERAPIES
• MAINTNANCE
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TREATMENT
• CLASS 1& 2
TREATMENT IS DICTATED BY THE SEVERITY OF THE EXTRARENAL LUPUS
NO LONG-TERM ASSOCIATION WITH ERSD
MAY REQUIRE MANAGEMENT OF PROTEINURIA AND SHORTER COURSE OF CORTICOSTEROIDS
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TREATMENT
• CLASS 3&4
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TREATMENT
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TREATEMENT
• CLASS V:
GENERALLY TREATED WITH PREDNISONE FOR 1-3 , THEN TAPERING FOR 1-2 YEARS DEPENDING
ON THE RESPONSE
OTHER IMMUNOSUPPRESSANTS ARE ADDED IF THEY ARE ASSOCIATED WITH ANOTHER
PROLIFERATIVE CLASS
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CLASS VI
• TREAT WITH CORTICOSTEROIDS AND OTHER IMMUNOSUPPRESSIVE AGENTS AS DICTATED BY
THE EXTRARENAL SYMPTOMS
• DIALYSIS
• TRANSPLANT ONCE THE DISEASE IS BURNT OUT
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TAKE HOME POINTS
• KEEPS HIGH INDEX OF SUSPICION
• URINE DIPSTIX IS IMPORTANT SCREENING TOOL
• CONSULT NEPHROLOGY TIMEOUSLY
• GOD LOVES YOU
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REFERENCES:
• SESHAN S ET AL, RENAL DISEASE IN SYSTEMIC LUPUS ERYTHEMATOSUS WITH EMPHASIS ON CLASSIFICATION OF LUPUS GLOMERULONEPHRITIS: ADVANCES AND IMPLICATIONS. AR CH PAT HOL LAB ME D
2009 ;33: 233-248
• CIMBALUK D, PATHOLOGY, CLASSIFICATION AND PATHOGENESIS OF LUPUS GLOMERULONEPHRITIS. DIAGN HIS T OP AT H . 2013;19: 151-157
• BANFI G ET AL, RENAL VASCULAR LESIONS AS A MARKER OF POOR PROGNOSIS IN PATIENTS WITH LUPUS NEPHRITIS. AM J KIDNE Y DIS 1991;18:240-248
• WU L ET AL, INCLUSION OF RENAL VASCULAR LESIONS IN THE 2003 ISN/RPS SYSTEM FOR CLASSIFYING LUPUS NEPHRITIS IMPROVES RENAL OUTCOME PREDICTIONS. KIDNE Y INT 2013;83:715-723
• HAAS M ET AL, HIV-ASSOCIATED IMMUNE COMPLEX GLOMERULONEPHRITIS WITH “LUPUS-LIKE” FEATURES: A CLINICOPATHOLOGIC STUDY OF 14 CASES. KIDNE Y INT E R NAT IONAL 2005;67:1381–1390
• PARK MH ET AL, TUBULOINTERSTITIAL DISEASE IN LUPUS NEPHRITIS: RELATIONSHIP TO IMMUNE DEPOSITS, INTERSTITIAL INFLAMMATION, GLOMERULAR CHANGES, RENAL FUNCTION AND PROGNOSIS.
NE P HR ON 1986;44:309-319
• PETRI M ET AL, DERIVATION AND VALIDATION OF SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS. AT HR IT IS R HE UM
2012;64:2677-2686
• OKPECHI I ET AL, LUPUS NEPHRITIS: AN APPROACH TO DIAGNOSIS AND TREATMENT IN SOUTH AFRICA. SAMJ 2015
• D’AGATI V ET AL. (2005). AFIP ATLAS OF NON-TUMOUR PATHOLOGY: NON-NEOPLASTIC KIDNEY DISEASE.
• JENNETTE C ET AL. (2007). HEPINSTALL'S PATHOLOGY OF THE KIDNEY, 6TH EDITION