Amyloidosis is the extracellular deposition of insoluble protein fibrils. AL- Primary systemic amyloidosis AA- Secondary amyloidosis AF- Familial Aβ2M- In ESRD Diagnosed by abdominal fat pad aspiration showing apple green birefringence by polarized light microscopy with Congo red stain High ESR, High ALP Serum Immunofixation electrophoresis (SIFE), UIFE-sensitive to detect light chains (Lamda more common) Kidneys- most common involved. Nephrotic range proteinuria, azotemia seen Heart- second common organ; restrictive cardiomyopathy, low voltage complexes in ECG, sparkly appearance on ECHO heart Peripheral sensory neuropathy,carpal tunnel syndrome Large tongue, raccoon-eye sign-Factor X deficiency^ bleeding) Treatment- AL- Melphalan. dexamethasone, lenalidomide & bortezomib; HDM/SCT- Autologous stem cell transplantation after high-dose melphalan High BNP. pro-BNP, troponin T & I in cardiac involvement, used for prognosis too. AA Amyloidosis: secondary to chronic infections - tuberculosis, SABE or inflammatory conditions like RA, IBD, Castleman's disease Treat the primary disease, TNF & IL -1 antagonist. The new drug - Eprodisate for renal AA AF Amyloidosis: AD, mutant TTR (transthyretin/prealbumin)° Peripheral & autonomic neuropathy ° Cardiac conduction defectsAβ2M/ Amyloidosis: associated with ESRD. on hemodialysis Carpal tunnel syndrome, joint effusions, cystic bone lesions Decreases with stopping dialysis