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Tuesday Clinical Case Conference

Tuesday Clinical Case Conference . 9/11/07 Zae Kim. Atheroembolic disease (Cholesterol Crystal Embolism) . Epidemiology Clinical and pathologic findings Diagnosis Pathogenesis Treatment. Atheroembolic Renal Disease (AERD). An underdiagnosed and increasing cause of renal failure

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Tuesday Clinical Case Conference

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  1. Tuesday Clinical Case Conference 9/11/07 Zae Kim

  2. Atheroembolic disease (Cholesterol Crystal Embolism) • Epidemiology • Clinical and pathologic findings • Diagnosis • Pathogenesis • Treatment

  3. Atheroembolic Renal Disease (AERD) • An underdiagnosed and increasing cause of renal failure • Caused by showers of cholesterol crystals from an atherosclerotic aorta that occlude small renal arteries • Often multisystemic • Iatrogenic complication • Treatment?

  4. Epidemiologyretrospective autopsy studyantemortem biopsy study

  5. Incidence:retrospective autopsy studies

  6. Incidence:antemortem biopsy studies

  7. Incidence:

  8. Risk factors

  9. Risk factors

  10. Risk factors

  11. Risk factors

  12. Age >60 Male gender White HTN Tobacco use DM Atherosclerosis CAD AAA PVD Risk factors

  13. Clinical and laboratory presentation

  14. Clinical Features • Atheroembolic renal disease is part of a multisystem • Renal • ~50% patients affected • Multiple presentation • Acute • Subacute • Chronic

  15. Clinical Presentation

  16. Clinical and Laboratory Presentation

  17. GI Gastric mucosal and submucosal biopsy -cholesterol crystals in the submucosal arterioles

  18. Clinical and Laboratory Presentation

  19. Cholesterol crystals lodged in the retinal vessels (Hollenhorst plaques) on funduscopic examination.

  20. outcome

  21. outcome

  22. Survival rate (Kaplan Meier) of 67 patients with disseminated CCE

  23. Laboratory Features • Variable and NONE ARE PATHOGNOMIC • Serum chemistry • Elevated BUN, creatinine • amylase, CPK, LFTs • Hematology • leukocytosis, thrombocytopenia, and eosinophilia • Elevated ESR, CRP • Serologic • Elevated ESR • Decreased serum complement • Urine (abnormal but nonspecific) • proteinuria, hematuria, eosinophilia

  24. Pathogenesis • Flory (1945) • 267 consecutive autopsies • 9 cases of cholesterol crystal embolism • 2/147 (1%) with moderate aortic plaque erosion • 7/57 (12%) with severe aortic plaque erosion • 0 in 63 cases with absence of aortic plaque ulceration http://www.mdconsult.com/das/book/body/77638334-4/620123283/1201/I4-u1.0-B0-7216-0164-2..50036-7--f10.fig?tocnode=50835407

  25. Atheroma • How vulnerable plaque is formed… • Fat droplet absorption • Cytokine release • Inflammation • Monocyte->macrophage • Further fat collection • The fat-filled cells form a plaque with a thin covering. http://heart.health.ivillage.com/cholesterol/heartattack3.cfm

  26. Pathology • CCE lodge in multiple small arteries (150-200 μm in diameter) • Interlobular, afferent arterioles, terminal arterioles, and glomerular capillaries Thin section, toluidine blue stain shows the characteristic cholesterol clefts (due to washout of the cholesterol crystals during histologic processing) of an atheroembolus in the small renal artery

  27. Histologic features • In acute lesion • Occlusion of lumen of small vessel • Inflammatory response: PMN leukocytes and eosinophils • Later stage • Foreign-body giant cells • Endothelial proliferation • Fibrous tissue surrounding the crystals

  28. Diagnosis • “great masquerader” • CCE is ubiquitous with random and variable distributions in the body • Mimic many other clinical syndromes • Ddx • Vasculitis • Subacute endocarditis • Polymyositis • Myoglobinuric renal failure • Drug-induced interstitial nephritis • Renal artery thrombosis or thromboembolism

  29. Definitive diagnosis - biopsy • Biopsy • Characteristic needle-shaped empty clefts within arterioles • “ghost cells” because crystals are dissolved during tissue fixation • Muscle, kidney, or skin • Cutaneous biopsy with 92% yield

  30. Treatment • No effective treatment available • Secondary Prevention • Avoid precipitating factors • aggressive risk factor modification, and • optimal medical mgmt of CVD • smoking cessation, anti-platelet tx, and bp control, cholesterol and glucose • Statin – uncertain? • Steroid? • Surgical – with clear embolic source

  31. What is the implication of eosinophilia/-uria and hypocomplementemia?

  32. Eosinophilia Eosinophiluria

  33. Hypocomplementemia • Complement and inflammatory response may play a role in pathogenesis of AERD. • Hammerschmidt (J lab Clin med 1981)…

  34. Generation of PMN-aggregating activity in plasma incubated with lipids extracted from atheromatous aortas Aggregating activity of PMN

  35. Role of steroid? • Use of corticosteroid was associated with 100% mortality (Fine, Agiology, 1987) • Belenfant’s experience (1999) • N=18 • Patients with laboratory evidence of inflammation • Corticosteroid treatment using prednisolone 0.3mg/kg • Outcome: • Therapy credited with the relief of lower limb and/or gastrointestinal pain and definite improvement in food intake and clinical status

  36. conclusion • Under-recognized cause of kidney failure • Think about it before precipitating risk • Think about it in your differential • Look for it

  37. Belenfant: Supportive treatment improves survival in multivisceral cholesterol crystal embolism. Am J Kidney Dis 1999, 33:840-850. • Highlighted the potential benefits of avoiding further precipitating insults in conjunction with optmal medical management

  38. Belenfant • Larges series to date, n=67, w catastrophic atheroembolism • Prospective with f/u to 4 yrs • N=2102 admit to renal intensive care unit over 11-yr period • Dx • Based on clinical and histologic findings • Excluded patients with other causes of acute or acute on chronic renal impairment (also excluded CIN or perioperative associated renal failure)

  39. The end

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