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Lecture 3. Secondary glomerular diseases and diseases of large blood vessels

Lecture 3. Secondary glomerular diseases and diseases of large blood vessels. Crescentic Glomerulonephritis and Vasculitis. VASCULITIS: Inflammation and necrosis of blood vessels Large vessel vasculitis eg Giant cell (Temporal) arteritis

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Lecture 3. Secondary glomerular diseases and diseases of large blood vessels

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  1. Lecture 3. Secondary glomerular diseases and diseases of large blood vessels

  2. Crescentic Glomerulonephritis and Vasculitis • VASCULITIS: Inflammation and necrosis of blood vessels • Large vessel vasculitis eg Giant cell (Temporal) arteritis • Medium sized vessel arteritis eg Polyarteritis Nodosa • Small vessel vasculitis* • *Capillaries, venules, arterioles +/- small arteries • Skin only e.g. drugs • Multiple organs, tissues, kidney: systemic vasculitis • Crescentic glomerulonephritis is typical of systemic vasculitis involving the kidney • (renal-limited crescentic GN also occurs)

  3. Vasculitis

  4. Vasculitis - artery in Polyarteritis Nodosa Lumen

  5. Crescentic Glomerulonephritis, vasculitis • Crescent: Proliferation of cells inside Bowman’s capsule - obliterates urinary space CRESCENT Glom

  6. Systemic small vessel Vasculitis • Microscopic polyangiitis (micro PAN) • Vasculitis in small, med blood vsls; glomerulonephritis • Wegener’s granulomatosis • Necrosis and granulomas in upper/lr resp tract • Vasculitis in small, med blood vsls; glomerulonephritis • Henoch-Schlonlein purpura • Cryoglobulinemia • Goodpasture’s syndrome and anti-GBM nephritis • Anti-GBM, anti-alveolar BM antibodies • Pulmonary haemorrhage, glomerulonephritis

  7. Wegener’s granulomatosis • Multinucl Giant cell • Granulomas, necrosis, vasculitis in respiratory tract • Crescents, focal necrosis in glomeruli

  8. Vasculitis - clinical • Clinical signs vary: Fever, rash, arthralgias, pulmonary infiltrates or nodules, haemorrhage, ENT, GI, musculoskel or neurological symptoms, • Acute renal failure, oliguria over wks, months (dialysis); hypertension, haematuria, proteinuria • Investigations: Renal function, imaging, ESR, serology, ANCA

  9. Anti-neutrophil cytoplasmic antibodies (ANCA) • Serum antibodies to enzymes in neutrophil granules, monocyte lysosomes • Immunofluorescence: Cytoplasmic or Perinuclear • P-ANCA (anti-myeloperoxidase) in 80% of micro polyangiitis; more often indolent, renal limited • C-ANCA (anti-proteinase 3) in 90% of Wegener’s • Very useful in diagnosis; follow up of disease activity in Wegener’s with C-ANCA

  10. Crescentic GN, vasculitis • Crescent • Necrosis and crescent N Cr

  11. Crescentic GN, vasculitis: Immunofluorescence findings . .. ,. 65% “pauci-immune” • pauci = few or no IC • 20% immune complex • 15% anti-GBM

  12. Crescentic GN, vasculitis - pathology • LM: Glomerular crescents, inflammation and necrosis • Crescent: Proliferation of cells inside Bowman’s capsule - obliterates urinary space (-> oliguria) • Focal glomerular inflammation, necrosis • Immune complex deposits FM & EM 20% e.g. SLE, cryoglob • anti-GBM 15% Goodpature’s syndrome, anti-GBM nephritis • pauci-immune 65% (in ANCA+ microscopic polyangiitis, Wegener’s) • Adverse prognosis: >80% crescents, anti-GBM • Summary: Crescentic GN typical of systemic small vessel vasculitis; sometimes limited to kidney

  13. Secondary glomerular diseases • Glomerular disease an important feature of multisystem diseases - vasculitis, diabetes, SLE, amyloidosis • DIABETES MELLITUS • Relative/absolute deficiency of insulin secretory response; leading cause of end-stage renal disease in US, Europe (40%) • Type 1: autoimmune destruction of insulin secreting beta cells • Type 2 (90%) decreased insulin secretion; insulin resistance • Gradual onset of proteinuria, glycosylation of proteins, microangiopathy thick leaky GBM, and increased mesangial matrix synthesis; also suscept to pyelonephritis

  14. Glomerular and vascular lesions in Diabetes Art Art Art KW

  15. SLE (Lupus) nephritis

  16. SLE (Lupus) nephritis • Chronic auto-immune disease; females 20-30 yrs • Rash, connective tissues, kidney etc • Antinuclear (e.g. anti-ds DNA), anticytoplasmic & antiphospholipid antibodies • Lupus nephritis in 90% - variable proteinuria, haematuria • Immune Complex Glomerulonephritis ranges from very mild to severe • Glomerulonephritis a major cause of morbidity, mortality

  17. Lupus nephritis

  18. Amyloidosis • Abnormal beta-fibrillar protein (15 classes) • (A-beta in Alzheimer’s) • AA amyloid (chronic infection, inflammation) or AL amyloid (Ig derived) deposited in glomeruli, renal vessels • Proteinuria • Poor prognosis

  19. Amyloidosis

  20. Clinical Presentations of glomerular disease

  21. Clinical Presentations of glomerular disease • CRESCENTIC GN and VASCULITIS (“Rapidly progressive GN”) • HAEMATURIA • IgA nephropathy • PROTEINURIA & NEPHROTIC SYNDROME • Minimal change disease • Membranous glomerulonephritis • SLE, diabetes, renal amyloidosis • ACUTE NEPHRITIS • Post-streptococcal glomerulonephritis - • Haematuria, hypertension, raised serum creatinine, oedema • Immune complexes to streptococcal antigen • Most patients recover fully • CHRONIC RENAL FAILURE • Abnormal renal function tests. Raised se Cr, reducd Cr Cl & clinical signs • Develops slowly, chronically over years • Glomerular disease not the only cause (nephrosclerosis, APCKD)

  22. Lesions of large blood vessels

  23. Lesions of Renal Blood Vessels • Thrombi, emboli, infarcts • Renal artery thrombosis • Left heart, heart valves e.g. mitral vegetations • Atheroemboli from aorta -> interlobular arteries in kidneys

  24. Lesions of Renal Blood Vessels • Vasculitis (other than small vessel vasculitis) • Large and medium sized blood vessels, small arteries. • Temporal (Giant cell) arteritis - head and neck • Takayasu’s - coronaries • (Vasculitis a/w infection include fungal) • Polyarteritis Nodosa • aneurysms in coeliac, mesenteric, coronary and renal arteries; hepatitis B in 30%; ANCA negative

  25. Lesions of Renal Blood Vessels • Renal artery stenosis • Proximal renal artery atherosclerosis • Fibromuscular dysplasia • GFR afferent arteriole pressure -> Renin, BP • Nephrosclerosis • Age change worsened by hypertension • Thick arterioles (“hyaline” arteriolosclerosis) & thickened small, medium sized arteries • Small kidneys, granular suface +/- larger scars • Malign BP and thrombotic microangiopathies • Endothelial injury, necrosis of media, thrombi

  26. Nephrosclerosis • Granular renal cortical surface & scars • Thickened artery

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