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The Amyloidoses

The Amyloidoses. Douglas A. Stahura 4/7/99. Amyloidoses History. Virchow described a homogenous eosinophilic material which binds Congo red stain. Associated with inflammatory joint disease and amyloid deposition in kidney, liver, spleen. Amyloidoses Definition. Criteria for definition

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The Amyloidoses

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  1. The Amyloidoses Douglas A. Stahura 4/7/99

  2. AmyloidosesHistory • Virchow described a homogenous eosinophilic material which binds Congo red stain. • Associated with inflammatory joint disease and amyloid deposition in kidney, liver, spleen.

  3. AmyloidosesDefinition • Criteria for definition • Homogenous, hyaline eosinophilic on H+E stain • Crystal violet metachromasia • Stains with alkaline Congo red • Apple green birefringence on polarized light • Fibrillar structure on microscopy • Salt extraction yields p-component forms • Fibril is unique for all forms of amyloid

  4. Amyloidoses • AL L-chain primary, MM • AH H-chain primary, MM • ATTR transthyretin senile systemic • Abeta2M Beta2 microglobulin dialysis • Abeta beta-protein precursor Alzheimer • Ascr Scrapie Creutzfeldt-Jakob

  5. Amyloidoses • Most common clinical form beta protein placque of Alzheimer’s • for rheumatoligist AL disease primary amyloidoses or MM

  6. AmyloidosesClinical Presentation • Renal: nephrotic range protenuria or mild • must be distinguished from monoclonal light chains • protein may diminish with renal insufficiency • HTN • Enlargement occurs early

  7. AmyloidosesClinical Presentation • Cardiac: Restrictive cardiomyopathy- noncompliant hemodynamics, thickening of septum • on Echo- “sparkling” of echoes • endomyocardial biopsy shows AL deposition on immunohistochemical staining • Digoxin and Nifedipine toxicity reported due to collective binding to fibrils.

  8. AmyloidosesComplication • AL disease - acquired deficiency of clotting factor X • Periarticular amyloid deposition presenting as pseudoarthritis = shoulder pad sign

  9. AmyloidosesTreatment • Melphalan and Prednisone • 2 controlled trials with no statiscally significant improvement in survival • 20% of patients did have improvement

  10. AmyloidosesB2 Microglobulinemia • significant articular presentation with chronic renal disease • all reported pt’s presented with • joint pain • carpal tunnel • osteonecrosis • dialysis for mean of 7 years • B2 microglobulin is polypeptide of class I protein of MHC

  11. AmyloidosesAA Amyloid • AA deposition in tissues, associated with • rheumatoid arthritis • familial Mediterranean fever • Recent series 5% of RA have tissue deposits and 2% have cinically evident disease, usually renal or hepatic. • Worldwide, AA deposition common in leprosy, tuberculosis, osteomyelitis.

  12. AmyloidosesAA Amyoid • Clinical - episodic fever, arthritis, abdominal/pleuritic inflammation • Treatment - Colchicine • abortive of acute episodes • abortive of amyloid formation

  13. AmyloidosesAging • 5 sites identified with aging • Brain: beta protein of Alzheimer’s • Pancreas: islet associated polypeptide • ANF: fibril of isolated atrial amyloid • Cardiac: transthyretin, senile systemic amyloid • Aorta: fibril not identified

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