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Y4C-parenchymal kidney diseases.ppt

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Y4C-parenchymal kidney diseases.ppt

  1. 1. Parenchymal kidney diseases Umm Al-Qurra university- Faculty of medicine- 4th year Department of pathology Dr.Raid Jastania Dr. Abeer Shaker
  2. 2. Learning Outcomes At the end of this lecture the student should be able to • Discuss the most common systemic disease that affect the kidney illustrating pathology of lupus nephritis • Discuss the most common causes of glomerular diseases • Understand the difference between nephrotic and nephritic syndrome 3/18/2023 (C)Year4_UQUMED 2
  3. 3. Introduction  Glomerular diseases constitute some of the major problems in nephrology.  Glomerulonephritis is an inflammation of the glomerulus, while glomerulopathy is a term for disorder affecting this structure.  Glomeruli may be injured by variety of factors and in course of several systemic diseases.  Most of the glomerular diseases are immunologically mediated 3/18/2023 (C)Year4_UQUMED 3
  4. 4. Glomerular diseases Glomerulopathies in Systemic diseases  SLE  Diabetes mellitus  Goodpasture  Bacterial endocarditis  Amyloidosis  Vascular disorders • Hypertension • PAN • Wegener’s granulomatosis • Henoch-Schönlein purpura. 18/03/2023 4 Primary Glomerular diseases - Minor Glomerular abnormalities: Minimal Change disease - Focal and/or segmental lesions: - Focal glomerulosclerosis - Focal proliferative glomerulonephritis - Diffuse glomerulonephritis - Chronic GN (most common cause of CRF)
  5. 5. Clinical classification of renal diseases 1. Nephritic syndrome 2. Nephrotic syndrome 3. Asymptomatic hematuria or proteinuria, 4. Rapidly progressive glomerulonephritis 5. Acute renal failure : oliguria or anuria, with recent onset of zotemia. 6. Chronic renal failure 7. Urinary tract infection 8. Nephrolithiasis (renal stones) 18/03/2023 5
  6. 6. Immune mechanisms underlie most forms of primary glomerulopathies and many of secondary glomerular disorders. ANTIBODY MEDIATED IN SITU IMMUNE COMPLEX DEPOSITION CIRCULATING IMMUNE COMPLEX MEDIATED CYTOTOXIC ANTIBODIES CELL MEDIATED IMMUNE INJURY ACTIVATION OF ALTERNATIVE COMPLEMENT PATHWAY Pathogenesis of glomerular injury
  7. 7. Immune mechanism in immune glomerulonephritis 1. Antigen or Antibody or immune complex deposition. 2. Immune reaction 3. Activation of complement system 4. Destruction of glomerular structure 5. Inflammation 6. Affect renal function 18/03/2023 7
  8. 8. Clinical-Pathological correlations  Almost any variety of histopathologic findings can be found in any of the clinical syndromes and vice versa  There are broad correspondences between prognosis and histologic findings.  Each morphologic type of glomerular disease has a clinical correlation as well as etiology and pathogenesis. 18/03/2023 8
  9. 9. What is Nephrotic syndrome? marked Proteinurea ≥3.5 g/day (protein: creatinine ratio >3-3.5) Hypoalbuminaeia <3g/L Hyperlipidemia & hyperlipiduria Generalized Oedema Clinical complex that includes the following:
  10. 10. The most important causes that characteristically lead to the nephrotic syndrome are Primary (idiopathic): •Membranous nephropathy •Minimal change disease •Focal segmental glomerulosclerosis •Membranoproliferative GN •IgA nephropathy (rare cause) 18/03/2023 10
  11. 11. Secondary to: Diabetes mellitus Amyloidosis Systemic lupus erythrematosus Ingestion of drugs (gold, penicillamine , "street heroin") Infections (malaria, syphilis, hepatitis B, HIV) Malignancy (carcinoma & melanoma) Miscellaneous (bee-sting allergy, hereditary nephritis) 18/03/2023 11
  12. 12. Case1# A 5 years old child presented to pediatric clinic by his parents suffering from swelling which was noticed around the eyes, legs and abdomen with recurrence of the symptoms. The swelling was noticeable in the morning. The color of urine had turned straw colored. It is not associated with burning or pain during urination. 18/03/2023 12
  13. 13. Case 2# 6 years old male child presented to pediateric clinic with burning urination and hematuria. His mother noticed that the child has 2 attacks of follicular tonsillitis in previous 3 months before the presenting symptoms go through 18/03/2023 13 As a future doctor can you give us the main difference between case1# & 2
  14. 14. Clinical picture of nephritic syndrome Abrupt onset of acute nephritic syndrome 1- 4 weeks after upper respiratory tract or skin infection leading to: Gross hematuria (urine appears smoky brown) Oliguiria and azotemia Hypertension Proteinuria and peri-orbital edema High titre of anti-streptolysin O (ASO) in serum Low serum complement level (hypocomplementenemia) 18/03/2023 14
  15. 15. Causes of Nephritic syndrome 1. Infection inducing acute diffuse proliferative GN (primary glomerular diseases) 2. Post-streptococcal GN 3. Non-streptococcal GN (certain pneumococcal and staphylococcal infections, some viral infection as HCV, and HBV) 4. Secondary to systemic diseases as SLE 18/03/2023 15
  16. 16. Diagnostic features of glomerular diseases Light microscopy Cellularity Extracellular matrix Special stains (PAS, amyloid) Immunofluorescence microscopy (Linear, granular, mesangial, irregular deposits) Electron microscopy (deposits of immune complexes, BM changes, amyloid) 18/03/2023 16
  17. 17. The kidney can be affected in different diseases including autoimmune diseases diabetes infections cardiac and liver diseases Kidney involvement usually affect mode of therapy response to therapy and outcome The kidney in systemic disease
  18. 18. Systemic Lupus Erythrematosus (SLE)  A recent study on Taif University in 2015 showed that a high percentage of familial lupus in Saudi patients may be a consequence of high consanguinity rate in Saudi Arabia*  Another study in Western Saudi Arabia found that lupus nephritis (LN) in 2014 that compare with other results from other series a high prevalence of LN in Saudi population especially Class IV was the most frequent type of nephritis. * * 3/18/2023 (C)Year4_UQUMED 18 * Albishri, J.A., Alshehri, S.S., Altowairqi, A.M. and Aljuaid, R.M. (2015) Familial Lupus and Clinical Characteristics in Saudi Arabia. International Journal of Clinical Medicine, 6, 899-905. http://dx.doi.org/10.4236/ijcm.2015.612117 ** Wafaey .G, Sami.B, Wael.H, Maimoona.M, Saeed.A.G., Jaudah.A.M: (2014) Clinicopathological characteristics of lupus nephritis in Western region of Saudi Arabia: An experience from two tertiary medical centres. Journal of Microscopy and Ultrastructure, Volume 2, Issue 1, March 2014, Pages 12-19 open access. https://doi.org/10.1016/j.jmau.2014.02.001
  19. 19. Lupus Nephritis Renal involvement is variable from mild asymptomatic proteinuria and hematuria to ever renal impairment that may require dialysis The clinical picture can change rapidly to a very aggressive disease There might be a discrepancy between the clinical picture and histological findings 3/18/2023 (C)Year4_UQUMED 19
  20. 20. Pathophysiology Autoimmunity plays a major role in the pathogenesis of lupus nephritis. The histologic type of lupus nephritis that develops depends on numerous factors, including the antigen specificity and other properties of the autoantibodies and the type of inflammatory response that is determined by other host factors. 3/18/2023 (C)Year4_UQUMED 20 Immunologic mechanism Production of antibodies Against nuclear elements
  21. 21. Pathogenesis of lupus nephritis 3/18/2023 (C)Year4_UQUMED 21
  22. 22. Lupus Nephritis ISN/RPS classification Class I: minimal mesangial lupus glomerulonephritis (LGN) Class II: mesangial proliferative LGN Class III: focal LGN (involving less than 50% of the total number of glomeruli) Class IV: diffuse LGN (involving 50% or more of the total number of glomeruli) Class V: membranous LGN Class VI: advanced sclerotic LGN (>90% of glomeruli globally sclerosed without residual activity) 3/18/2023 (C)Year4_UQUMED 22 International Society of Nephrology (ISN)/Renal Pathology Society (RPS) Classification of lupus nephritis (LN)
  23. 23. Lupus nephritis
  24. 24. 21 year old lady known to have SLE and lupus nephritis for the last 4 years . Biopsy was done at the time of diagnosis and showed class IV with active disease but no chronic changes . Which one of the following histopathologic finding will be found in her renal biopsy? A.Diffuse LGN B.Focal LGN C.Membranous LGN D.Mesangial proliferative LGN E.Minimal mesangial lupus glomerulonephritis 3/18/2023 (C)Year4_UQUMED 24 Can you answer this question
  25. 25. References: Robbins Basic Pathology 10th Edition; Chapter 5, 121-188 & Chapter 14, 549-581 3/18/2023 (C)Year4_UQUMED 25

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