Nephrology DivisionKing Khalid Universitytutorial to course441

Tutorial Case

14 year old Saudi young female student

C/0 fever- Headache for 10 days

General malaise

Dark urine x 3 days

HPI

Fever intermittentFever intermittent

Muscular joint painMuscular joint pain

no skin rashno skin rash

urine characterurine character

edemaedema

PH & Med PH & Med

antibiotic & NSAIDantibiotic & NSAID

Social HxSocial Hx

S. reviewS. review

FHFH

ExaminationExamination

Bp : 160/90 &Pulse 120/MIN Bp : 160/90 &Pulse 120/MIN Temp 39 C Resp 25/MIN .paleTemp 39 C Resp 25/MIN .pale

Look SickLook Sick

Puffiness in facePuffiness in face

Head& neckHead& neck

JVP highJVP high

Chest: Bronchial breathing

decrease Breath Rt sideIncrease TVFIncrease Dull percussionS1 increaseS2 NS3 Positivepan systolic murmurradiation to axillagrade 3

Abd tend epigastictend lionsBS + ve

CNS Drawsy

M.S tender joint &muscle

Initial DiagnosisFever? Infection RT lung consolidationM R HEAMATURIA: Renal stone (ARF –AKI- ATN) Acute glomerulonephritis (post infection)GN RPGN IgAHemolytic anemiahemolytic uremic syndromeNSAID papillary necrosis

Investigation

WBC 15000 cells per microliter Hb 100 g/L Plat 150 g/L

PT normal PPT normal Sec Bl film Sec

U & E

Scr 210 μmml

urea 20 mmol/L

K6 mmol/L Na 125 mmol/L

Ca 1.9 mmol/L album 28 g/L

Urine Analysis

few RBC

No cast

Protein 1.2g/24h

U/S : kid size ENLARGE 12.2CMU/S : kid size ENLARGE 12.2CM

Treatment

Patient receive ceftrixone IV and IV fluid TREAT HYPERKALEMIA

Follow up:

S cr 300 μmml/L

JVP

Oliguria

Edema

Management:

IV lasixRepeat urine analysis RBC CAST ANCA(P) positive Kidney Biopsy RPGN

Final diagnosis microscopic polyangitis

Glomerular Disease – Glomerular Disease – Acute GlomerulonephritisAcute Glomerulonephritis

Post infectious glomerulonephritis

Group A Strep Infection Infective endcarditis

Membranoproliferative glomerulonephritis:

Systemic lupus erythematosusHepatitis C virus

Rapidly progressive glomerulonephritis Vasculitis (cryoglobulinemia

Type I RPGN (direct antibody) Good Posture syndrome

Type II RPGN (immune complex) Post infectious Systematic lupus erythematosus Henoch – Schonlein pupura (IgA) Others

Type III RPGN (pauci-immune)

o Wegener granulomatosiso Microscopic polyarteritis

Investigations for Investigations for GlomerulonephritisGlomerulonephritis

Regular Follow-up and U&ERegular Follow-up and U&EAntistreptolysin O (ASO)Antistreptolysin O (ASO) ANA, Anti DNAANA, Anti DNA C3-C4C3-C4 ANCA (p, c)ANCA (p, c) HCV Antibody HBsAgHCV Antibody HBsAgHIVHIVRFRFCryoglobulinCryoglobulinAnti-basement Membrane Antibody (With lung Anti-basement Membrane Antibody (With lung hemorrhage)hemorrhage)

Acute postinfection Acute postinfection GlomerulonephritisGlomerulonephritis

Often associated with group A Often associated with group A

B-hemolytic streptococcal type 12 infectionB-hemolytic streptococcal type 12 infection

Abrupt onset 10-12 days after strept throat Abrupt onset 10-12 days after strept throat or 14-21 skin infection (impetigo)or 14-21 skin infection (impetigo)

Also staphylococcus or virusesAlso staphylococcus or viruses

Acute GlomerulonephritisAcute Glomerulonephritis

Symptoms occur 10-21 days after infection

HematuriaProteinuriaDecreased GFR, oliguriaHypertensionEdema around eyes, feet and anklesAscites or pleural effusion

Low C3, normal C4, Antistreptolysin O (ASO) – Kidney biopsy immune complexes and proliferation,

Proliferative GN- poststretococcal

This glomerulus is hypercellular and capillary loops are poorly defined.

This is a type of proliferative glomerulonephritis known as post- streptococcal glomerulonephritis.

Poststreptococcal GN

Post-streptococcal glomerulonephritis is immunologically mediated, and the immune deposits are distributed in the capillary loops in a

granular, bumpy pattern because of the focal nature of the deposition process.

Post-Streptococcal GlomerulonephritisPost-Streptococcal Glomerulonephritis

CONSERATIVE TREATMENT (acute kidney CONSERATIVE TREATMENT (acute kidney injury)injury)

Improves 1-4 weeks, C3 normalizes in 1-3 Improves 1-4 weeks, C3 normalizes in 1-3 months, hypertension improves 1-3 months, months, hypertension improves 1-3 months, intermittent hematuria x 3 yearsintermittent hematuria x 3 years

99% complete recovery in children and 85% 99% complete recovery in children and 85% in adultin adult

IgA Nephropathy (Berger's Disease)IgA Nephropathy (Berger's Disease)

Most common acute glomerulonephritis in US ;South East Asia Most common acute glomerulonephritis in US ;South East Asia Associated with H.S. PurpuraAssociated with H.S. Purpura Upper respiratory(50%) IN 1—2 Day(Synpharyngitic hematuria)Upper respiratory(50%) IN 1—2 Day(Synpharyngitic hematuria) Primary versus secondary (IBD, Liver Disease, SLE, vasculitis)Primary versus secondary (IBD, Liver Disease, SLE, vasculitis) 50% risk of CRF50% risk of CRF Proteinuria, hypertension, renal insufficiency predict worse prognosisProteinuria, hypertension, renal insufficiency predict worse prognosis 50% increased IgA, normal compliments 50% increased IgA, normal compliments

TREATEMENT COSERVATIVE; ACEI TREATEMENT COSERVATIVE; ACEI

HIGH RISK: patient prednisone & alkylating agentHIGH RISK: patient prednisone & alkylating agent(cyclophosphamide-azothroprim) &ASA&ACEI&tosilectomy(cyclophosphamide-azothroprim) &ASA&ACEI&tosilectomy

Rapidly Progressive GN

Develops over a period of days or weeksDevelops over a period of days or weeks

Primarily adults in 50’s and 60’sPrimarily adults in 50’s and 60’s

Progresses to renal failure in a few weeks or Progresses to renal failure in a few weeks or monthsmonths

Hematuria is common, may see proteinuria, Hematuria is common, may see proteinuria, edema or hypertensionedema or hypertension

Rapidly Progressive (Crescentic) Glomerulonephritis

Morphology crescent formation

crescents are formed by proliferation of parietal cells

infiltrates of WBC’s & fibrin deposition in Bowman’s space

EM reveals focal ruptures in the GBM

MICROSCOPIC POLYANGITIS

Necrotizing vasculitis of small- and medium- sized vessels in both the arterial and venous circulations Frequently involves the lung and the kidneys

with typical complications of hemorrhage and glomerulonephritis Associated with ANCA in three-fourths of all

cases, usually anti-myeloperoxidase antibodies (MPO-ANCA) that cause a P-ANCA pattern on immuno-flourescence testing. ANCA directedf againsts proteinae-3 (PR3-ANCA) can also be observed in microscopic polyangiitis

Treatment

Initial TherapyInitial TherapyCombination cyclophosphamide-Combination cyclophosphamide-corticosteroid therapy corticosteroid therapy A slow steroid taper, with the goal of A slow steroid taper, with the goal of reaching 20 mg of prednisone per day reaching 20 mg of prednisone per day by the end of two months and an overall by the end of two months and an overall glucocorticoid course of between 6 and glucocorticoid course of between 6 and 9 months. 9 months. Either daily oral or monthly intravenous Either daily oral or monthly intravenous cyclophosphamide.cyclophosphamide.

Treatment

Plasmapheresis Plasmapheresis Severe manifestations of pulmonary Severe manifestations of pulmonary hemorrhage on presentation. hemorrhage on presentation.

Dialysis-dependent renal failure upon Dialysis-dependent renal failure upon presentation.presentation.

Concurrent anti-GBM antibodies. Concurrent anti-GBM antibodies.

Goodpasture Syndrome

Antibody formation against pulmonary and glomerular capillary basement membranes

Damage glomerular basement membrane Men 20 - 30 years of age Pulmonary hemorrhage and renal failure

TREATMENT:Early treatment is essential Pulse Steroid (10 mg/ kg/day for 3-5 days)CyclophosphamidePlasmaphorisis

Goodpasture’s syndrome

This immunofluorescence micrograph shows positivity with antibody to IgG has a smooth, diffuse, linear pattern that is characteristic for glomerular basement membrane antibody with Goodpasture's syndrome.