Kidney Disease Management: A Concise Reference

Kidney Disease Management

ANCA Small Vessels

Amyloidosis

Dx: Clinical. Tx: No therapy, just advice (Low complement disease)

Lupus Nephritis

– Lupus: Malar Rash/ Discoid Rash, Photo(S), Arthralgia

– Lupus Nephritis: Peripheral Edema, Proteinuria, HBP, K+++

Dx:

– BUN/Cr/UA/24 H PRT/BIOPSY

– SLE: dsDNA/C3/C4/AMA/CRP

^^Tx:

I- General Tx (or Class I)

Hydroxychloroquine 6-6.5mg/kg day

II- Class II (mesangial proliferative)

– if Proteinuria < 1g/day ——> Sx of SLE

– if PRT > 3g/day ——> CSD or CNI

III- Class III (Focal lupus) + Class IV (Diffuse lupus)

Initial therapy:

– CSD + Cyclophosphamide/Mycophenolate Mofetil!

(Worsening Lupus, do biopsy)

Maintenance therapy:

– Azathioprine (1.5-2.5mg/kg/day)

– Mycophenolate mofetil (1-2g/day)

– OP CSD Low dose (< 10mg/d)

(For 1 year after remission)

VI- Class V (membranous Lupus):

– Normal Kidney function / no proteinuria —-> Antiproteinuric + anti HBP

– Nephrotic proteinuria —-> CSD + immunosuppressant

Non-responder —> rituximab/ IV immunoglobulins!!

***AL Chain:

Sudden RF/N Kidney Size/Fanconi Sx/CARB

– Dx: An.Cr++/GFR(-)/CA++/BIOPSY of BM

– Px:

MM —> Abnormal immunoglobulin —> PCT —> injury —> cytokine release —> Light chain overflow DCT damage

^^Tx:

– Hydration

– Tx Ca+++ (biphosphates)

– Tx Ur+++ (Allopurinol)

– Stop NSAID

– 40 mg Dexamethasone to reduce Light chains

– Chemo: melphalan/thalidomide

^^Prognosis

– OP melphalan + prednisolone 4 Day pulse/4-6W = 50%

– Myelablative Tx > 50% 3 Year survival

IgA Nephropathy

Dx:

1- UA: Gross hematuria following an URT infection (24-48 hours)

2- Must Get Renal Biopsy to confirm Dx (C)

^^Signs of bad prognosis:

I- Clinical:

– level of proteinuria;

– arterial hypertension;

– hyperuricemia;

– renal failure at the time of diagnosis

II- Morphological:

– tubular atrophy

– interstitial fibrosis

– vascular wall thickening

Minimal Change Disease

^^Symptoms:

– Rapid nephrotic development

(selective proteinuria loss of albumin not immunoglobulin)

– URT infection (2/3)

– HBP

– Relapse when discontinue steroid

^^Dx:

I- Children:

Start Tx with Renal CSD biopsy in 4-6 Weeks if no improvement

Adult: Renal Biopsy

*BIOPSY IF DEFINITIVE*

^^Tx:

Children:

– Steroids (Prednisone), 1mg/Kg Prednisone!

– Reduction in proteinuria in 2 W

– For Children Same Tx For Relapse

Adult:

– Steroids (Prednisone), 1mg/Kg (max 80mg)

– Slower recovery

– Relapse = Cyclophosphamide (2mg/kg)

Rapidly Progressive Glomerulonephritis (RPGN)

Dx:

1- Exclusion

2- Renal Biopsy (Crescent)

3- ANCA (-)

Tx: Steroids + Cyclophosphamide

I- Induction Therapy:

Methylprednisolone 7mg/Kg

Cyclophosphamide 2-3mg/Kg OP

II- Plasma exchange for severe cases

Post-Streptococcal Glomerulonephritis

^^Findings:

– hematuria (coca-cola urine)

– oliguria

– HBP

– Periorbital Edema

– Usually in children but may occur in adults

^^Pathophysiology:

Antibody + immune complex! Deposit on (Mesangium, Glomerular Capillary Wall) attracting immune system there

^^Dx:

– anti-streptolysin (ASO) Titer (must be +ve)

– Complement Levels Low C3

^^Tx:

– Supportive for symptoms

– Anti HBP (CCB)

– Antibacterial (Penicillin/erythromycin) 10-14 Days

Membranoproliferative Glomerulonephritis (MPGN)

^^Pathophysiology:

Thickening of GBM due to deposit of immune complex. Mesangial cells will proliferate in ”Tram-track” appearance!

Types (depends on Immune deposition)

Type I:

Immune complex deposit under endothelium (subendothelium)

– associated with Hepatitis B + V

Type II:

– Deposition of immune complex within BM inside BM (not under or above)

– Ab in their Blood C3 Nephritic Factor (blocks C3 conversion which will cause inflammation)

^^Dx:

Renal Biopsy

Tx:

– Secondary MPGN – treatment of primary disease

– ? MPGN – for children – prednisolone was effective

Focal Segmental Glomerulosclerosis (FSGS)

Pathophysiology:

– Non-immune immunoglobulin -ve

– Fusion of Foot Processes (same as minimal change)

^^Findings:

– Nephrotic (rapidly progressing)

– Microhematuria + Arterial HBP

– ESRF 50%

^^Tx:

I- Symptomatic

– ACE(-)/ARB ——–> proteinuria

– Diuretics ——–> For edema

II- Pathophysiology:

– Prednisolone 1 mg/kg p/o.

If it doesn’t work – cyclophosphamide 2 mg/kg per day 2-3 months + prednisolone 1 mg/kg per day

^^Prognosis

– Ineffective Tx Bad Prognosis!!

Transplant 30% reoccurrence

Wegener’s Granulomatosis

– Systemic vascular disease characterized by prominent respiratory + skin involvement (skin lesion)

– Starts with Azotemia ——-> Renal Failure

^^Dx:

– Symptoms may vary or not exist

– Lab: Normocytic anemia, Elevated ESR, UA microhematuria

– Best initial Dx c-ANCA (must be +ve)

– To Confirm Dx We need RENAL Biopsy that shows

^^Tx:

Cyclophosphamide + Steroids

Renal Artery Stenosis (RAS)

^^Etiology:

Atherosclerosis

Fibromuscular dysplasia

^^Pathophysiology:

– Blockage of lumen > 50%

– 75% of Area

– Systolic decrease 10-15 mmHg

^^Dx (RAS)

– Bruit Sound (RAS)

– Catheter Direct Renal Angiography (#1)

– Captopril renography

– US

^^Tx:

– Correct BP < 140/80

– Preserve Kidney function

(Take care of cholesterol emboli + non-toxic contrast)

**Fibromuscular dysplasia:

– Women

^^Dx:

Catheter Renal Angiography

^^Tx:

Renal Revascularization!

**Ischemic Nephropathy:

^^Etiology:

– RF due to occlusion (RAS)

– Atherosclerosis/thrombosis

^^Clinical Findings:

– Sudden occlusion

– Rapid renal insufficiency

^^Dx:

US

Captopril

MRI (#1)

Renal Angiography (invasive)

^^Tx:

– Prevent CV complications

– Avoid ESRD

BP NOT controlled with ACE/ARB

– CCB

– Tx dyslipidemia

– Tx atherosclerosis Risk Factors

– PTA

Surgical revascularization