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Glomerulopathies –IgA nephropathy

Glomerulopathies –IgA nephropathy. IgA nephropathy - Pathogenesis. Glomerulopathies –IgA nephropathy. IgA nephropathy - Pathogenesis Mesangial IgA  promotes glomerular inflammation  a series of events  …….  can cause end stage renal disease. Glomerulopathies –IgA nephropathy.

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Glomerulopathies –IgA nephropathy

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  1. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis

  2. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis Mesangial IgA  promotes glomerular inflammation  a series of events  …….  can cause end stage renal disease

  3. Glomerulopathies –IgA nephropathy

  4. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis What specific mechanisms influence the deposition of IgA and its initiation of inflammation?

  5. Glomerulopathies –IgA nephropathy

  6. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis tightly T cell controlled processes in health most polymeric IgA is produced in the mucosal immune system is involved in mucosal defense the bone marrow produces monomeric IgA, which reaches the circulation

  7. Glomerulopathies –IgA nephropathy

  8. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis in IgA nephropathy synthesis of polymeric IgA is increased in the bone marrow and decreased in the mucosa

  9. Glomerulopathies –IgA nephropathy

  10. Glomerulopathies –IgA nephropathy

  11. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis Yes it is

  12. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis Why might this happen? This process is very tightly T cell regulated In response to a mucosal antigen, T cells reach the circulation, but will return to the mucosa to synthesize polymeric IgA if they express alpha 4 beta 7, the mucosal homing receptor If on the other hand, a systemic antigen is confronted, T cells will home to the marrow if they express the alpha 4 beta 1 systemic homing receptor

  13. Glomerulopathies –IgA nephropathy

  14. Glomerulopathies –IgA nephropathy

  15. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis in IgA nephropathy - these processes is abnormal there is an exaggeration of systemic homing receptor expression, which would drive T cells not back to the mucosa but towards the marrow  the abnormal site of IgA1 production, which is one of the features of IgA nephropathy

  16. Glomerulopathies –IgA nephropathy

  17. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis in IgA nephropathy - these processes is abnormal but not only there is an exaggeration of systemic homing receptor expression, which would drive T cells not back to the mucosa but towards the marrow  the abnormal site of IgA1 production, which is one of the features of IgA nephropathy structural abnormalities of the IgA molecule itself

  18. Glomerulopathies –IgA nephropathy

  19. Glomerulopathies –IgA nephropathy

  20. Glomerulopathies –IgA nephropathy IgA nephropathy - Pathogenesis Abnormal IgA1 molecule promote IgA1 self-aggregation  production of macromolecular IgA1 act as an antigen  production of IgA1-IgG complexes, which could be deposited influence interaction directly with matrix components and particularly with mesangial cells

  21. Glomerulopathies –IgA nephropathy

  22. Glomerulopathies –IgA nephropathy

  23. Glomerulopathies –IgA nephropathy

  24. Glomerulopathies –IgA nephropathy IgA nephropathy-a great clinical heterogeneity

  25. Glomerulopathies –IgA nephropathy

  26. Glomerulopathies –IgA nephropathy IgA nephropathy is often presenting as recurrent visible hematuria coinciding with mucosal infection which is almost never seen over the age of 40

  27. Glomerulopathies –IgA nephropathy

  28. Glomerulopathies –IgA nephropathy IgA nephropathy a few patients will present nephroticproteinuria

  29. Glomerulopathies –IgA nephropathy

  30. Glomerulopathies –IgA nephropathy IgA nephropathy other patients will be picked up because they have an asymptomatic urine abnormality

  31. Glomerulopathies –IgA nephropathy

  32. Glomerulopathies –IgA nephropathy IgA nephropathy others will already have advanced kidney disease when they’re first picked up

  33. Glomerulopathies –IgA nephropathy

  34. Glomerulopathies –IgA nephropathy “Is this one disease? Is this the same disease we’re picking up at different stages?” Prof J. Feehally Dept of NephrologyLeicester General Hospital Leicester, United Kingdom

  35. Glomerulopathies –IgA nephropathy IgA nephropathy in a global view of this disease it’s extremely different in its prevalence

  36. Glomerulopathies –IgA nephropathy

  37. Glomerulopathies –IgA nephropathy IgA nephropathy also a very variable prognosis

  38. Glomerulopathies –IgA nephropathy

  39. Glomerulopathies –IgA nephropathy IgA nephropathy - prognosis proteinuria, hypertension and GFR at the time of diagnosis predict outcome that’s true for any glomerular disease

  40. Glomerulopathies –IgA nephropathy

  41. Glomerulopathies –IgA nephropathy IgA nephropathy – prognosis glomerulosclerosis, interstitial fibrosis, tubular atrophy predict outcome that’s true for any glomerular disease

  42. Glomerulopathies –IgA nephropathy

  43. Glomerulopathies –IgA nephropathy IgA nephropathy – prognosis proteinuria you have not just at presentation but during prolonged follow up is a very powerful predictor of outcome !!

  44. Glomerulopathies –IgA nephropathy IgA nephropathy – prognosis how much proteinuria you have during follow up combined with mean arterial pressure during follow upare the most powerful predictor of outcome

  45. Glomerulopathies –IgA nephropathy

  46. Glomerulopathies –IgA nephropathy IgA nephropathy and Henoch Schonlein Purpura

  47. Glomerulopathies –IgA nephropathy

  48. Glomerulopathies –IgA nephropathy IgA nephropathy and Henoch Schonlein Purpura What is IgA nephropathy? characterised by predominant mesangial IgA deposits by immunofluorescence on the renal biopsy there is no clinical nor serological features, which are specific for IgA nephropathies to make the diagnosis, you really need a renal biopsy with appropriate immunofluorescence What is Henoch Schonlein Purpura? a true clinical syndrome characterised by palpable purpura, arthritis, and abdominal pain, and of course, a glomerulonephritis characterised by predominant IgA deposits

  49. Glomerulopathies –IgA nephropathy IgA nephropathy and Henoch Schonlein Purpura a more accurate definition of H.S.Purpura a small vessel vasculitis with IgA dominant deposits involving skin, gut, and glomeruli associated with arthritis

  50. Glomerulopathies –IgA nephropathy

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