renal diseases

advertisement

Random Kidney Diseases:

Disease

Tubulointerstitial

Nephritis

Renovascular

Disease

ADPKD – Autosomal

Dominant Polycystic

Kidney Disease

Definition

Inflammation of renal interstitum, may be acute or chronic

Stenosis of renal artier or one of its branches

Characterised by multiple fluid filled cysts on both kidneys.

Causes

Acute: immune reaction to medications

(NSAIDS, antibiotics etc.), Infections

(Staph, Strept etc), immune disorders

(SLE, GN)

Chronic (CIN): results from many disorders such as pyelonephritis, reflux nephropathy, sickle-cell disease, lead intoxication

80% due to atherosclerosis. IHD, stroke, peripheral vascular disease, antiphospholipid syndrome, post renal transplant.

Prevalence 1:1000

Genes on chromosome 16 and 4

Urate Nephropathy

Thrombotic

Thrombocytopenic

Purpura (TTP)

Oliguric/anuric renal failure due to uric acid precipitation within tubules

Deficiency of protease that normally cleaves vWf , leading to platelet aggregration in small vesselsand microthrombi.

Overproduction of uric acid in pts with lymphoma, leukemia or myeloproliferative diesese, particularly after chemo.

If chronic could be due to gout.

Genetic or Acquired. Often unknown cause; drugs, pregnancy, HIV, SLE.

Signs and Symptoms

Renal impairment,

BP or ARF with fever, rash, arthralgia, ↑

IgE.

Investigations

Renal Biopsy shows T lymph and macrophage infiltration of interstitum.

Extensive fibrosis and loss of renal tubules

Management

Stop cause of ARF. Prednisolone usually will have full recovery.

BP, worsening renal function after ACE In, pulm oedema, abdom or carotid bruits, weak leg pulse

Renal Enlargement with cysts, ab pain, possible haematuria if haemorrhage into cyst, infection,

BP, liver cysts

U/S: affected side smaller kidney, disturbed renal flow on

Doppler. Angiography god standard but invasive.

Monitor U+E

U/S for cysts and sixe of kidneys

Drug Tx, stent placement or revascular surgery.

Target BP with ACEIn, treat infections , laproscopic cyst removal or neprectomy. ↑ water intake and ↓ na and caffeine

Hydratiion, allopurinol, urinary alkalisation with NaHCO3 to make uric acid more soluble.

Oliguric., tophi and findings of gout.

Fever, CNS signs of seizures,

 vision,

 consciousness,

Mucosal bleeds, renal failure, haematuria, proteinuria.

U/S: renal parenchyma appears bright .

Plasma Urate raised with birefringent crystals on microscopy.

Blood film: fragmented RBC

(schistocytes).

 platelets,

Hb,

Normal clotting tests.

Haematuria, Proteinuria on urinalysis.

*Haematological Emergency: need urgent plasma exchange, steroids, IV vincristine and immune globulin.

Renal Manifestations of Systemic disease:

Disease Definition Causes

Amyloidosis

Diabetic nephropathy

Myeloma

Disorder characterised by extracellular deposits of abnormal protein resistant to degradation.

Feature of

Alzheimers, T2DM.

Diabetes mellitus is disease where cells become insulinresistant or body cannot produce insulin, leading to hyperglycaemia.

Nephropathy is a progressive kidney disease caused by angiopathy of glomerular capillaries leading to sclerosis.

Malignant Excess production of monoclonal antibody

+- light chains.

Usually IgG or IgA.

1° (AL): clonal proliferation of plasma cells producing amyloidogenic immunoglobulins.

2°(AA): From chronic inflame of RS, IBD, TB,

OM etc

Familial: autosomal dominant

Accounts for 18%

ESRF.

Diabetes, usually atleast 15 years after disease begins but earlier if poor glyceamic control and high cholesterol.

Malignancy. Age >70

Diffuse Systemic

Sclerosis

Connective tissue disease that involves skin and organ fibrosis

Autoimmune

Signs and Symptoms

Glomerular lesions: proteinuria

/nephritic. Arrythmias, angina, rest cardiomyopathy, Carpal tunnel, malabsorption, hepatomegaly, periorbital purpura

Oedema, malaise, fatigue, anorexia, headaches, pruritis.

These symptoms don’t prt=esent until later stages.

Earliest sign microalbuminuria, followed by proteinuria.

Pathophysiology

Unknown. Possibly similar to myeloma??

Hyperglyceamia causes renal hyperperfusion increasing GFR = kidney hypertrophy. Leads to mesangial hypertrophy and focal glomerulosclerosis due to ↑ glomerular pressure, causes microalbuminuria progressing to proteinuria.

Backache, pathological fractures, hypercalaceamia, anaemia, neurtopenia and thrombocytopenia, recurrent infn, renal impairment

Renal Crisis if ARF + accelerated

HT. Also involves cardiac and lung disease due to fibrosis.

Presents with scleroderma (skin fibrosis) of skin.

Renal: Cast nephropathy causes tubular occlusion, light chain deposition, hypercalaemia due to tubular toxin and volume depletion, immunoglobulin induced glomerular changes, plasma call infiltration.

Investigations

Biposy affected tissue and positive congo red stainig with red-green biregfringence under light microscopy.

Management

If secondary disease treat primary cause and may improve. Primary may respond to therapy as for myeloma. Otherwise 1-2 year survival rate.

Earliest sign is microalbuminuria which should be tested every

6 months with EMU

(early morning urines) to test albumin:creatinine ratio.

Good glyceamic control delays onset and progression. Target BP with ACE In. If ESRF reached combined renal and pancreatic transplant should be considered.

Smoking cessation.

FBC: norm/norm anaemia, ↑ ESR, ↑urea and creatinine, increase

Ca. X rays show lytic punced out lesions and osteoporosis.

Bone pain analgesia and bisphosphonate.

EPO/transfusion for anemia. Fluid replacement and Dialysis if RF severe.

Tx infections.

No cure. Control BP and annual echocardiogram and spirometry.

Immunosuppression including IV cyclophosphamide.

Vasculitis (ANCA +):

1. Wegeners

2. Microscopic

Polyangiitis

3. Goodpastures

SLE

Inflammatory disorder of blood vessel walls. Small

ANCA + associated with GN

Inflammatory disorder of blood vessel walls. Small

ANCA + associated with GN

Also called Antiglomerular basement membrane disease

(GBM)

Multisystem disease in which autoantibodies are made against a variety of autoantigens (ANA).

Unknown

Autoimmune

Autoimmune, smoking may contribute

Autoimmune.

Typically woman of child-bearing age. Also

Afro-carribeans and

Asian ethnicity. May be triggered by EBV.

Can be drug induced.

URT disease, sinusitis, RPGN, heamoptysis, purpure, nodules, ocular involvment

Purpura, livedo, RPGN signs, pulm haemorrhage

Haemoptysis, macroscopic haematuria, oliguria, ARF

Malaise, fatigue, myalgia, fever, weight loss, alopecia, lymphadenopathy, Raynauds,

Malar rash, photosensitivity, arthritis, oral ulcers.

Renal nephritis causes persistent proteinuria or cellular casts.

Unknown, possibly similar to below.

P-ANCA antibodies bind and degranulate neutrophils which release toxins causing endothelial and glomerular injury

Development of autoantibodies to type

4 collagen (in BM and also in lung)

1/3 of patients will have renal involvement with vascular, glomerular and tubulointerstitial damage. Due to pathogenic autoantibodies which form immune complexes that deposit in sites such as kidneys.

Urinalysis: protein and heam. CXR. cANCA confirmed with raised

Proteinase3. Renal biopsy.

P-ANCA positive, ↑ESR and CRP, ↑ creatinine, proteinuria, haematuria, casts on microscopy. Biopsy Dx.

CXR (pulm haem) renal biopsy (crescentic GN) ,

AntiGBM

ANA + and high double stranded DNA (dsDNA) in 60% patients.

Standard therapy is steroids and IV cyclophosphamide.

Methotrexate as maintenance

Standard therapy is steroids and IV cyclophosphamide

Plasma exchange and corticosteroids.

Also hyperparathyroidism: renal manifestations are from hypercalaceamia.

Rapidly Progressive GN (RPGN): Potential to cause ESRF over days, Biopsy shows crescents affecting most glomeruli. 3 Categories:

Acute flares require IV cyclophosphamide.

Maintenance with NSAIDS, methotrexate, hydrochloroquine. If nephritis vital to control

BP – ACE In, Ca channel blockers.

1.

2.

3.

Immune complex: post infectious (endocarditis), multisystem (SLE), Iga nephropathy, Henoch-Schonlein purpure.

Pauci-immune disease: Wegener’s, microscopic polyangiitis, Churg-Strauss

Anti-GBM (Goodpastures disease).

Download