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Glomerulonephritis nurse teaching jan 2017

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Glomerulonephritis nurse teaching jan 2017

  1. 1. GLOMERUL ONEPHRITI S AMBER Z JAFFERI EMERGENCY DEPARTMENT SIH
  2. 2. 2 PLAN General over view Little revision anatomy
  3. 3. 3 GLOMERULONEPHRITIS Nephros – kidney -itis – inflammation of Glomus – small round ball or knot Pathos – suffering or disease -osis – diseased condition Glomerulonephritis – inflammation of the glomeruli Glomerulopathy – disease of the glomeruli
  4. 4. 4 Light micrograph of a normal glomerulus. There are only 1 or 2 cells per capillary tuft, the capillary lumens are open, the thickness of the glomerular capillary wall (long arrow) is similar to that of the tubular basement membranes (short arrow), and the mesangial cells and mesangial matrix are located in the central or stalk regions of the tuft (arrows). Courtesy of Helmut G Rennke.
  5. 5. 5 Light micrograph in membranoproliferative glomerulonephritis showing a lobular appearance of the glomerular tuft with focal areas of increased glomerular cellularity (large arrows), mesangial expansion (*), narrowing of the capillary lumens, and diffuse thickening of the glomerular capillary walls (small arrows). Courtesy of Helmut Rennke, MD.
  6. 6. 6 Electron micrograph of a normal glomerular capillary loop showing the fenestrated endothelial cell (Endo), the glomerular basement membrane (GBM), and the epithelial cells with its interdigitating foot processes (arrow). The GBM is thin and no electron dense deposits are present. Two normal platelets are seen in the capillary lumen. Courtesy of Helmut Rennke, MD.
  7. 7. 7 Electron micrograph in dense deposit disease (DDD) showing dense, ribbon-like appearance of subendothelial and intramembranous material (arrow) and narrowing of the capillary lumen due to proliferation of cells (double arrow). Courtesy of Helmut Rennke, MD.
  8. 8. 8 GLOMERULAR DISEASE Primary – confined to the kidney Secondary – due to a systemic disease
  9. 9. 9 GLOMERULAR INJURY Impairment of selective filtering properties of the kidney leading to a decreased GFR Molecules normally not filtered such as constituents of the blood, pass into the urine and are excreted
  10. 10. 10 MD consult
  11. 11. 11 Anatomy of Kidney Note the positions of Glomerulus Loop of Henle PCT, DCT, CT Cortex, Medulla, Pelvis. MD consult
  12. 12. 12 JGA ↓GFR  Renin Angiotensin Blood Pressure MD consult
  13. 13. ULTRASTRUCTURE – GLOM. CAPILLARY
  14. 14. 14 POSSIBLE CLINICAL MANIFESTATIONS Proteinuria – asymptomatic Haematuria – asymptomatic Hypertension Nephrotic syndrome Nephritic syndrome Acute renal failure Rapidly progressive renal failure End stage renal failure
  15. 15. 15 GLOMERULONEPHRITIS Presence of glomerular disease as opposed to tubulointersititial or vascular disease is suspected from history Haematuria (especially dysmorphic red cells) Red cell casts Lipiduria (glomerular permeability must be increased to allow the filtration of large lipoproteins) Proteinuria (may be in nephrotic range of >3.5 g/24hours)
  16. 16. 16 Phase contrast microscopy showing dysmorphic red cells in a patient with glomerular bleeding. Acanthocytes can be recognized as ring forms with vesicle-shaped protrusions (arrows). Courtesy of Hans Köhler, MD.
  17. 17. 17 DIAGNOSIS Look for clues • History • Haematuria • Proteinuria • Azotemia • Azote – nitrogen • A – without • Zoe – life • “The gas does not support life” (French chemists Gayton de Morveau (1737-1816) and Antoine Lavoisier (1743-1794) ) McCarthy ET, November 2008
  18. 18. 18 DIAGNOSIS Can be difficult to distinguish between Glomerular disease and tubulo-interstitial disease Tubular disease does not directly increase protein excretion but nephron loss due to the disease can have the same end result
  19. 19. 19 CLINICAL PATTERNS Patients age and characteristics of the urine sediment can allow narrowing of the differential diagnosis options prior to biopsy ‘URINE IS THE LIQUID BIOPSY OF THE KIDNEY’ Walter Piering MD – Prof Med Wisconsin
  20. 20. 20 URINARY PATTERNS 3 different patterns • Focal nephritic • Diffuse nephritic • Nephrotic
  21. 21. 21 URINARY PATTERNS Focal nephritic • Associated with inflammatory to less than half of the glomeruli on light microscopy • Red cells – often dysmorphic • Occasional red cell casts • Mild proteinuria (<1.5g/day)
  22. 22. 22 URINARY PATTERNS Diffuse nephritic • Damage to all or almost all of the glomeruli • Similar to focal disease but may also have heavy proteinuria (even nephrotic range), oedema, hypertension and/or renal insufficiency • - ‘full house’ urinary sediment – red cells, white cells, red cell casts, white cell casts, hyaline casts
  23. 23. 23 URINARY PATTERNS Nephrotic • Heavy proteinuria • Lipiduria – refractile fat bodies that look like a maltese cross under polarised light • Few cells • Few casts – but those present are hyaline and granular
  24. 24. 24 NON SPECIFIC NATURE OF HISTOLOGIC PATTERNS Membranous GN – usually Immune complex disease (infective endocarditis, SLE, Hepatitis C) Membranous nephropathy – drugs (gold, penicillamine), SLE, Hepatitis B, malignancy Focal glomerulosclerosis can be primary ( minimal change), or secondary (intraglomerular hypertension, or healing previous glomerular injury)
  25. 25. 25 PATTERN DIAGNOSIS FOCAL GN <15 years – mild post infectious GN, IgA nephropathy, thin basement membrane disease, hereditary nephritis, Henoch Schonlein Purpura, mesangial proliferative GN 15-40 years – IgA nephropathy, thin basement membrane disease, lupus hereditary nephritis, mesangial proliferative GN >40 years – IgA nephropathy
  26. 26. 26 PATTERN DIAGNOSIS DIFFUSE GN Post infectious GN, lupus GN, membranoproliferative GN, mixed cryoglobulinaemia Often associated with decreased complement Classic findings • PSGN (anti strep antibodies) • Lupus nephritis (ANA) • Anti-GBM disease (anti GBM Abs) • Mixed cryoglobulinaemia (circulating cryoglobulins) • Wegener's granulomatosis (anti neutrophil cytoplasmic abs)
  27. 27. 27 PATTERN DIAGNOSIS DIFFUSE GN <15 years – Post infectious GN, membranoproliferative GN 15-40 years – Post infectious GN, rapidly progressive GN, fibrillary GN, membranoproliferative GN >40years – rapidly progressive GN, vasculitis, fibrillary glomerulonephritis
  28. 28. 28 PATTERN DIAGNOSIS NEPHROTIC SYNDROME <15 years – minimal change disease, focal glomerulosclerosis, mesangial proliferative GN 15-40 years – focal glomerulosclerosis, minimal change disease, membranous nephropathy including lupus, diabetic nephropathy, preeclampsia, post infectious GN >40 years – focal glomerulosclerosis, membranous nephropathy, diabetic nephropathy, minimal change disease, IgA nephropathy, primary amyloidosis or related disorder – light chain deposition disease (up to 20% of pts over 60), benign nephrosclerosis, post infectious GN
  29. 29. 29 GENERAL WORKUP ? GLOMERULAR DISEASE History • Family history kidney disease and hearing trouble (Alport’s syndrome) • Medications that can damage the kidney (NSAID’s, ACEI, penicillamine, gold, mercury in some skin lightening creams) • Recent throat infection - ? Strep- PSGN or viral – Wegener's granulomatosis, IgA • Cancer – solid tumours, Hodgkin’s (minimal change) or non Hodgkin’s (MPGN)
  30. 30. 30 GENERAL WORKUP ? GLOMERULAR DISEASE Physical Examination • Inspection – appearance, colour, pitting oedema, xanthelasma, alopecia, facial rash, purpura, clubbing, livedo reticularis • Palpation – pulse, hepatomegaly, palpable kidneys, splenomegaly, palapable bladder • Percussion – hepatomegaly, splenomegaly • Auscultation – renal artery bruits, other bruits, cardiac lesions, hypertension,
  31. 31. 31 GENERAL WORKUP ? GLOMERULAR DISEASE Laboratory work • UECB • LFT • BSL • FBC • Urine microscopy and culture • ACR • Serum and urine protein electrophoresis • Renal ultrasound
  32. 32. 32 GENERAL WORKUP ? GLOMERULAR DISEASE Laboratory work • Specific serology • For a nephrotic type picture • HIV, HCV, HBV, ANA, serum cryoglobulins, anti DNA ab, complements • For a nephritic type picture • Blood cultures, ASOT, AntiDNAse B, ANA, Anti DNA ab, anti GBM ab, anti neutrophil cytoplasmic ab, complements
  33. 33. 33 Pre urinalysis
  34. 34. 34 IGA NEPHROPATHY – BUERGER’S DISEASE Most common cause of GN in Asia but uncommon in Sth America or Africa 15-40% of all biopsy proven GN Male > Females 2nd -3rd decade Most commonly asymptomatic with serendipitous finding of haematuria and mild proteinuria Another classic presentation is macroscopic haematuria in conjunction with a viral infection Renal function is usually normal but occasionally a patient will present with acute renal failure due to acute tubular necrosis secondary to the gross haematuria Biopsy – mild to moderate mesangial cell proliferation, IgA deposits in the mesangium on immunofluorescence, often with C3 deposition also
  35. 35. 35 IGA NEPHROPATHY – BUERGER’S DISEASE Slowly progressive By 20 years, 50% have end stage kidney disease Worse prognosis if >1g/day proteinuria, hypertension, increased creatinine of glomerular fibrosis at biopsy, on presentation
  36. 36. 36 IGA NEPHROPATHY – BUERGER’S DISEASE Management • Aggressive control of blood pressure and proteinuria with ACEI’s or AR2B’s • Corticosteroids +/- azathiprine – varied schools of thought • However if rapidly progressive GN with crescent deposition treatment should be aggressive with high dose steroids and cyclophosphamide • Consult the Nephrologist
  37. 37. 37 RAPIDLY PROGRESSIVE GN (PRGN) Medical emergency ‘full house’ nephritic urinary sediment Immediate hospitalisation and biopsy Crescentic GN – proliferation of cells outside the glomerulus, but within Bowman’s space If IgG present in linear stain along the basement membrane – consistent with anti glomerular basement membrane antibiodies (AGBM ab’s) which is a marker of Goodpasture’s syndrome Presence of a linear pattern or complement in a granular pattern on the capillary wall suggests an immune complex associated disease such as lupus, IgA nephropathy of PSGN Absence of immune deposition suggests a vasculitic process such as Wegener’s granulomatosis or microscopic polyangiitis
  38. 38. 38 RPGN – EG GOODPASTURE’S Autoimmune Commonly 2nd -3rd decade and second peak in 60+ age group Some present with renal involvement (Goodpasture’s disease) Some present with pulmonary haemorrhage and nephritis (Goodpasture’s syndrome) Rarely some present with only pulmonary involvement
  39. 39. 40 GOODPASTURES
  40. 40. 41 RPGN – EG GOODPASTURE’S Classic – haemoptysis after upper respiratory infection and have nephritic urinary sediment History of smoking or hydrocarbon exposure is common CXR – pulmonary haemorrhage Lab- iron deficiency anaemia and renal dysfunction, circulating anti-GBM antibodies Kidney biopsy crescentic GN with linear staining IgG and C3 along the glomerular basement membrane
  41. 41. 42 RPGN – EG GOODPASTURE’S Treatment • High dose IV steroids (methyl pred 500mg daily for 3 days) followed by oral prednisolone and cyclophosphamide • Plasma exchange every other day until anti-GBM Ab titire is negative • Px guarded (if present with oliguria and elevated creatinine, or severe scarring – unlikely to recover renal function)
  42. 42. 43 NEPHROTIC SYNDROME Can be due to systemic or local renal disease Diabetic nephropathy most common cause Other common causes include amyloidosis (often secondary to multiple myeloma), light chain deposition disease, minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, membranoproliferative glomerulonephritis, fibrillary glomerulonephritis
  43. 43. 44 NEPHROTIC SYNDROME EG MINIMAL CHANGE DISEASE Other name lipoid nephrosis or nil disease Most common cause of nephrotic syndrome in kids 2-12 years but also in adults Onset often acute and precipitant my be beesting, viral infection, allergy or immunization Association with Hodgkin’s lymphoma and other T cell malignancies
  44. 44. 45 NEPHROTIC SYNDROME EG MINIMAL CHANGE DISEASE Clinical – dramatic weight gain, pitting oedema, normal blood pressure. Urine – proteinuria, hyaline casts, oval fat bodies. Usually no red cells. Normal renal function but sometimes failure secondary to severe hypoalbuminaemia or prerenal azotemia leading to volume contraction. Children don’t need biopsy unless hypertensive or other complications If biopsy done, EM fusion of podocytes (foot processes of glomerular visceral epithelial cells)
  45. 45. 46 NEPHROTIC SYNDROME EG MINIMAL CHANGE DISEASE Treatment • Oral corticosteroids – prednisolone 2mg/kg/day • Cyclophosphamide if relapsing diseas
  46. 46. 47 NEPHROTIC SYNDROME EG FSGN Most common cause of nephrotic syndrome in young adults Classic nephrotic syndrome and a small amount of blood in the urine Can occur in minimal change disease which becomes resistant to prednisolone Can be secondary heroin use Can be secondary to HIV infection Associated with other diseases (morbid obesity, persistent reflux nephropathy, sickle cell disease , cyanotic congenital heart disease)
  47. 47. 48 NEPHROTIC SYNDROME EG FSGN Diagnosis – biopsy – light microscopic pattern of segmental or total sclerosis of glomerular tufts Treatment – prednisolone 1mg/kg/day often for 6-8 months Complete remission only in 50% ACEI Poor prognosticators – tubulointerstitial disease, increased creatinine, marked proteinuria
  48. 48. 49 NEPHROTIC SYNDROME EG MEMBRANOUS NEPHROPATHY Most common cause of nephrotic syndrome in 40- 60 yo’s Usually frank nephrotic syndrome, low grade microhaematuria, relatively preserved renal function Some people asymptomatic Others can lose 10-20g of protein a day and be quite sick Associated with certain medications eg penicillamine, gold, captopril, NSAID’s, certain viral infections eg Hep B and HCV and malignancies
  49. 49. 50 NEPHROTIC SYNDROME EG MEMBRANOUS NEPHROPATHY Diagnosis is on kidney biopsy; glomeruli appear normocellular with thickening of the GBM, immune deposits on outer side of GBM Mx – rule out secondary causes Mx – supportive – ACEI/AR2B for proteinuria, statins for hypercholesterolaemia, prophylactic warfarin (if very low albumin markedly increased risk of venous thrombosis) Prednisolone may be used Some may not progress over 10 years, but marked proteinuria and increased creatinine – 40 % progress to ESKD
  50. 50. 51 NEPHROTIC SYNDROME EG MEMBRANOPROLIFERATIV E GLOMERULONEPHRITIS Idiopathic if between 10-30 year Between 35-60 years usually secondary to Hepatitis C Clinical- hypertension, mild nephrotic syndrome, microhaematuria, relatively preserved renal function Pts with HCV may have circulating cryoglobulins including triad of weakness, arthralgias and palpable purpura In kids 2 forms • MPGN 1 – circulating immune complexes passively trapped in glomeruli • MPGN 2 – circulating IgG (nephritic factor) that activates complement via the alternative pathway
  51. 51. 52 NEPHROTIC SYNDROME EG MEMBRANOPROLIFERATIV E GLOMERULONEPHRITIS Diagnosis – serum complement (depressed), hepatitis serologies, biopsy – glomeruli are hypercellular, often lobular in appearance – more detailed changes. Treatment – manage hypertension, ACEI/AR2B, salt restriction, diuretics, treat HCV with interferon 50% progress to ESKD Tends to recur in a kidney transplant
  52. 52. 53 NEPHROTIC SYNDROME EG FIBRILLARY GLOMERULONEPHRITIS Recently recognised – 40-60 years Similar to MPGN but serum complement normal and microscopy of biopsy demonstrates fibrilllar deposist in the mesangium. Prognosis guarded
  53. 53. 54 Diseases PSGN IgA Nephropathy MPGN RPGN Age and Sex All ages, mean 7 years, 2:1 male 2:1 male, 15-35 yrs 6:1 male, 15-30 yrs Mean 51yrs, 2:1 male Clinical Manifestations 90% 50% 90% 90% Acute nephritic syndrome Occasionally 50% Rare rare Asymptomatic haematuria 10-20% Rare Rare 10-20% Nephrotic syndrome 70% 30-50% Rare 25% Hypertension 50% Rare 50% 60% Acute renal failure Latent 1-3 weeks Follows viral infection Pul haemorrhage, iron def none Lab findings ASOT IgA +anti GBM membrane + ANCA Positive streptozyme IgA in dermal caps C3-C9 N C1 and C4 Immunogenetics HLA B12 Light microscopy Diffuse proliferation Focal proliferation Focal- diffuse crescentic Crescentic GN Immunoflourescence Granular IgG and C3 Diffuse mesangial IgA Linear IgG and C3 No immune complexes Electron microscopy Subepithelial humps Mesangial deposits No deposits No deposits Prognosis 95% cure 5% progress Slow progression in 25- 50 years 75% stabilise or improve if treated early 75% stabilise or improve if treated early Treatment Supportive None established Plasman exchange, cyclosphosphamide, steroids Pulsed steroid therpy
  54. 54. 55 CONCLUSIONS Take a history Do a urine test If haematuria and proteinuria - ?GN Exclude secondary causes Biosy is the definitive way to diagnose but some hints from history and
  55. 55. 56 ACKNOWLEDGEMENTS Handbook of nephrology…..Wilcox et al Up to date MD Consult

Notas do Editor

  • Patients who also have oedema and hyperlipidaemia (nephrotic syndrome) have a more marked glomerular leak
    Some form of secondary focal glomerulosclerosis (eg reflux nephropathy) is more likely in patients with nephrotic range proteinuria (&amp;gt;3.5G/24 hrs).
    Relatively bland sediment (few cells or casts) in nephrotic disorders results from lack of inflammatory cell infiltration in the glomeruli. Largely due to absence of immune complex deposition in most of these conditions such as minimal change disease, diabetic nephropathy and amyloidosis. Immune complexes are depositied in membranous neprhopathy but this occurs across the glomerular basement membrane in the sub-epithelial space. So complement can be activated but the chemoattractants (C3a and C5a) are separated from the vascular space by the basement membrane and don’t have access to the circulating mononuclear cells and neutrophils.
    This lack of inflammation also results in the plasma creatinine concentration being normal or only slightly elevated at presentation.
    Acute renal failure can occur but usually only in a limited setting eg
    Concurrent acute tubular necrosis usually in minimal change disease and usually over 50years
    Tubular injury in collapsing focal glomerulosclerosis either idiopathic or associated with HIV
    Minimal change disease with acute interstitial nephritis induced by non steroidal antiinflammatory drugs
    Crescentic glomerulonephritis superimposed upon membranous nephropathy
    Nephrotic syndrome secondary ton monoclonal immunoglobulin deposition disease may also develop myeloma cast nephropathy and acute renal failure
    Triad of nephrotic syndrome, a nephrotic sediment and acute renal failure narrows the differential DX
  • Varies between geographical locations but this is a broad brush stroke
  • Xanthelasma, pitting oedema – nephrotic syndrome,
    Alopecia, facial rash – lupus
    Clubbing in hepatits related kidney disease
    Livedo reticularis – vasculitis
    Purpura – endocarditis associated GN, lupus, cryogolbulinaemia
  • anti neutrophil cytoplasmic ab-wegener’s or idiopathic, microscopic polyangiitis

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