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Renal involvement in systemic diseases

Renal involvement in systemic diseases. Lívia Jánoskúti. Introduction. A variety of systemic conditions can affect the function of the kidneys, from acute illnesses (including for example prolonged hypotension ) to drugs and more insidious illnesses.

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Renal involvement in systemic diseases

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  1. Renal involvement in systemic diseases Lívia Jánoskúti

  2. Introduction A variety of systemic conditions can affect the function of the kidneys, from acute illnesses (including for example prolonged hypotension) to drugs and more insidious illnesses. Overview of the potential renal consequences of some of the commoner/more important systemic diseases.

  3. Renal involvement in systemic diaseses • Hypertensive nephropathy/nephrosclerosis • Metabolic disorders (Diabetes mell., gout) • Immunologically mediated diseases • Diseases associated with paraproteinaemia and neoplasia 5. Hereditary disorders

  4. 1.Hypertensive nephropathy • ¼ of patients with renal failure has hypertensive nephropathy • Chr.hypertension damages the renal vasculature Ischaemia and hyperfiltration cause nephrosclerosis • Hypertension is a risk of Cholesterol embolisation stenosis of renal arteries • More than 10 years history of hypertension

  5. Think of hypertensive nephropathy,if • The patient african or karibien origin • The patient has hypertensive retinopathy • There is left ventricular hypertrophy on the ECG • There is a long history of malignant hypertension/hypertension • The proteinuria<0,5gr/die • Renal biopsy showes nephrosclerosis

  6. What to do in hypertensive nephropathy? • Blood pressure control with ACEI /ARB, diuretics and other drugs. • No ACEI in bilateral art. renal stenosis! • Control of renal function if you change the dose of a drug. • Treat cardiovascular risk factors (cholesterol, obesity, smoking etc.) • In significant renal art. stenosis- renal angioplastic.

  7. 2.Metabolic disorders/ Diabetes mell. • 30-40% of patients with end stage renal disease have DM. • In type I.DM- common, in type II.-not common • 3% of patients with >10 years history of DM. have D. nephropathy • Nephropathy is a risk factor for other micro-and macrovascular complications .

  8. 2.Characteristics of diabetic nephropathy • Microalbuminuria 20-300mg/die- incipiens sign of nephropathy • >300mg/die – diabetic nephropathy. • GFR gradually decreases • Blood pressure increases

  9. 2.What to do in diabetic nephropathy? • Tight glycemic control with diet, drugs ( insulin) and regular physical activity. • Tight RR control 130/80 with ACEI/ARB ± diuretics/BB • ACEI improves the prognosis of patients with microalbuminuria in normotensives as well. • Treat other cardiovascular risk factors( aspirin, statin) • Pancreas and renal trasplantation

  10. 2. Metabolic disorders/Gout Cause:10% uric acid overproduction, 90% decreased renal excretion 1.Acute uric nephropathy-urate deposition in renal tubules- lympho.,-myeloprolif. diseases – prevention:increased fluid intake ,+ 600-900mg/die, later 300mg/die allopurinol 2.Chronic uric stone -nephropathy- uricosuric treatment: probenecid 2x250mg /die ,or sulfinpyrazon 2x500mg/die later 300-400mg/die.

  11. Tophus in the kidney

  12. 3.Immunmediated kidney diseases. • SLE • Goodpasture syndrome • Vasculitic syndromes • Rheumatoid arthritis • Scleroderma • Sjögren syndrome

  13. SLE

  14. 3/1.SLE and the kidney • Common:35-90% • Clinical symptoms:mild abnormalities of the urinary sediment to massive proteinuria and from chronic indolent GN-itis to rapidly progressive renal failure • Clinical types:nephrosis( 50%), nephritis, mixed forms,some % rapidly progressiveGN

  15. RPGN- crescent formation

  16. SLE-kidney-treatment • Milder forms-treatment directed to control of the extrarenal manifestations( steroids in modest doses, salicylates(!),antimalarials.Serologic parameters should be followed –antiDNS,C3,4. • Severe forms(IV):high dose iv metilprednisolon, adjunctive cytotoxic agents( cyclophosphamide, azathioprine) • Uremia:chr.dialysis, kidney transplant.(uncommon the recurrence of the SLE in the allograft)

  17. 3/2.Goodpasture syndrome • Antibody against glomerular basement membrane • Affects young men • Pulmonary hemorrhage, iron deficiency anaemia,progressive renal failure • AntiBM AB 90% positive, histology: IF-linear deposits in basement membrane • Early treatment is effective: plasmapheresis +high dose steroid, later dose reduction + cytotoxic drugs

  18. AGBM glomerulonephritisLinear deposits

  19. Schönlein Henoch purpura

  20. 3/3Schönlein Hennoch purpuraHypersensitivity vasculitis • Vascular purpura, abdominal pain arthralgias,hematuria, proteinuria • IgA increased ,IC contains IgA • Prognosis is usually good.Steroid,cyt.is not evidently helpful. In severe forms plasmapheresis +steroid, immunsuppression.

  21. 3/3 .Necrotizing vasculitis • Wegener granulomatosis( upper-lower airway granulomasa+ kidney) • Microscopic polyangiitis ( pulmonary infiltrates,RPGN, muscle-nerve abnormalities) • Churg-Strauss sy (allergic asthma ,eosinophilia, kidney) ANCA positivity • Polyarteritis nodosa (fever, headache, myalgia, hypertension, renal involvement) • Prognosis usually bad. • Treatmnet in severe cases: plasmapheresis, steroid cytostatic drugs.

  22. 1.Wegener gran.(No linear deposits)2.Goodpasture (Linear BM deposits)

  23. Rheumatoid arthritis

  24. 3/4.Rheumatoid arthritis and the kidney Several form of glomerular injury occure: • Amyloidosis • Nephrosis sy- complication of penicillinamin / gold therapy) • Papillary necrosis-analgetic drugs side effect • Vasculitis-mild proliferative or membranous GN

  25. Sjögren syndrome

  26. 3/5. Sjögren sy • Incidence:5% • Types: glomerulonephritis, interstitial nephritis ( polyuria, nephrolithiasis), renal tub. acidosis.

  27. Scleroderma

  28. 3/6. Scleroderma • Microangiopathy lead to acute or chronic renal failure • Acute form: sudden skin symptoms, hypertension, headache,oliguria,edema, renal failure. • In spite of ACEI treatment 50% renal failure. • Remission can occure .

  29. 4.Paraproteinaemias and the kidney Myeloma multiplex ( plasma cell tu): 50%of cases have kidney involvement Causes of renal failure: • Dehydration, hyperviscosity • hyperCa • NAIDS, biphosphonat side effect • Tumor lysis syndroma • Amyloidosis • Light chain deposition.

  30. 4.Paraproteinaemias and the kidney Amyloidosis( primer):deposits that stain with Congo red, nephrosis sy 5 years survival 20%, no effective treatment Cryoglobulinaemia Cryoprecipitable Ig-k, form IC(IgGpolyclon, IgM) Acute renal failure, RPGN Often associated with hepatitis C infection (interferonalfa) plasmapheresis,steroid ,cytostatic drugs

  31. Tumors and kidney Nephrotic syndrome is the most common clinical manifestation • Secunder amyloidosis • IC mediated membranosus GN,which regresses after removal of the tumor

  32. 5.Hereditary disease and kidney-Sickle cell disease • The disease causes a glomerulopathy with proteinuria and progressive renal insufficiency, leading to ESRF; renal papillary necrosis is another possible mechanism of acute renal syndromes. • Albuminuria is a sensitive marker of glomerular damage and precedes the onset of renal failure. • There are no effective therapies to prevent the onset of renal failure other than good management of the condition in order to reduce the incidence of, and ameliorate, sickling crises. • Great care should be taken to avoid or adjust the dose of nephrotoxic drugs which may precipitate acute or acute on chronic renal impairment. • Those with ESRF will require renal replacement therapy and should be considered for transplantation.

  33. Main message If you detect • Urine abnormality • Renal function abnormality Always look for • Urological disease • Systemic disease with renal involvement • Intrinsic renal disease

  34. Case: B.A.(17 years old female) • 1989.Small joint pain, hair loss • Physical examination: small joint involvement, RR 120/80 P:100/min No edema. • Labor: spec. grav 1015 protein:+++ Sed:6-8wbc,10-15 rbc 24h prot: 2,6gr ELFO albumin, transferrin.Se prot:53g/l Alb 53% kreat 93uM/l , ANF.antiDNS pos Cryoglob pos C3 decreased • Kidney biopsy: mesangioprolif.GN • Therapy:250mg Metypred • Proteinuria decreased to 1,2g/24h, hematuria stopped

  35. Case: SZ.M.(32 years old female) • 1984.weakness, fatigue,small joints pain,back pain, alopecia, photosensitivity,periorbital, hand leg edema for 2 month. • Physical examination:pallor,leg and hand edema,RR 120/80 P:90/min • Labor: urin 1022 prot+++ Ül: 2-4wbc 80-100rbc Ht 0,29 WE:104mm/h 24h prot 16gr Se prot.43g/l Alb 33% Se chol:7,2 urine ELFO: alb, trasferrin, IG-k kreat 65uM/l ANF , antiDNS poz.,C3 decreased • Biopsy: membranoprolif.GN. Th 50mg Prednisolon+100mg Imuran After 6 m. 24h prot.6gr, Se prot.59gr/l Ül:1-1 rbc

  36. Case:K.L.(65 years old male) • 1991.Back pain X ray:diffuse osteoporosis,Th7 compres.fract. We:24mm/h Ht 0,31 Treatment:Ca.D3 Miacalcic • 1992.febr. Dysuria,problems with stool-spinal MRI- tu-200Gy telecobalt irrad.

  37. K.L. • 1992. July Pallor, painfull ribs and dorsal vertebrae • Phys. exam:pallor,RR 70/90 P:94/min • Labor: fs 1020 prot:+++ Sed: neg Sü:70mm/h Ht 0,31 fvs 5000 24h prot 3,6gr ELFO: kappa light chain, Se ELFO: M comp.5,4g/l BUN 22 kreat 480 Bone marrow: 20% atypical plasma cell. Treatment:BCNU,Cyclo.,Vincrs. Prednisolon After 3 course of treatment: urin prot..0,58g/24h kreat 176uM/l,normal urination, and stool, bone pain decreased

  38. S.E. • 59 years old man • 2002.AMI ,hypertension • 2014. Juny: macroscopic hematuria,- cystoscopy revealed urin bladder tu. • He is on 25ug Thyroxin therapy • 2014.July. Surgery was planned, but, because of pleural effusion, generalized edema, dyspnoe,anaesthesiology contraindicated the operation.With the suspition of heart failure patient was transported to our department.

  39. S.E Physical examination. • Obes (110kg 178 cm), around the umbilicus 20 cm diameter red plaque(itching,for a month it showes a growing tendency), pitting leg edema, 4 cm bilateral pleural effusion • RR 160/90 P 80/min rhythmic, aeq.

  40. SE imaging results • Chest Xray: pleural effusion. No heart enlargement. • Echocardiography: EF54% LVH (Se, LWPD 13) relaxation abnormality. • Abdominal US: normal kidneys norm.Tumor in the urin bladder.

  41. S.E. Lab tests • BNP 145pg/L • Ht 0,41 cu 7,1 cholesterol 8,18 LDL 5,7) CRP 3,6mg/ml • SGOT,GPT SAP norm • creatinin 263-270-231 • Urin test:spec.grav 1015, protein ++++ ubg norm Sediment 80-100rbc, 20-25 wbc, bact • Se ELFO: 37,5 g/L Albumin 16,8 gr/L • 24h urine protein: 8 gr

  42. S.E.Suspected diagnosis • Tumor associated (paraneoplastic) nephrotic syndrome

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