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 Oliguric/anuric renal failure due
Thrombotic
Thrombocytopenic
Purpura (TTP) 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.

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
Connective tissue disease that involves skin and organ fibrosis
Autoimmune
Glomerular lesions: proteinuria
/nephritic. Arrythmias, angina, rest cardiomyopathy, Carpal tunnel, malabsorption, hepatomegaly, periorbital purpura
Unknown. Possibly similar to myeloma??
Biposy affected tissue and positive congo red stainig with red-green biregfringence under light microscopy.
Oedema, malaise, fatigue, anorexia, headaches, pruritis.
These symptoms don’t prt=esent until later stages.
Earliest sign microalbuminuria, followed by proteinuria.
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.
Earliest sign is microalbuminuria which should be tested every
6 months with EMU
(early morning urines) to test albumin:creatinine ratio.
FBC: norm/norm anaemia, ↑ ESR, ↑urea and creatinine, increase
Ca. X rays show lytic punced out lesions and osteoporosis.
If secondary disease treat primary cause and may improve. Primary may respond to therapy as for myeloma. Otherwise 1-2 year survival rate.
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.
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.

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.
Acute flares require IV cyclophosphamide.
Maintenance with NSAIDS, methotrexate, hydrochloroquine. If nephritis vital to control
BP – ACE In, Ca channel blockers.