The clinical guidelines summarize the current methods of diagnosis and treatment of systemic amyloidosis, including AA (secondary to chronic inflammatory diseases, that is, rheumatoid arthritis, ankylosing spondylitis, autoinflammatory disorders, osteomyelitis, malignancy, etc.), AL (a monoclonalplasma cell disorder that can be primary or associated withmultiple myeloma and Waldenström's macroglobulinemia) and ATTR (transthyretin amyloidosis associated with abnormal metabolism of wild-type transthyretin or with hereditary variants in the transthyretin gene). Diagnosis of amyloidosis thatcan be suspected clinically should be verified by tissue biopsy (Congo red staining and apple-green fluorescence under apolarizing microscope). The treatment goal in any type ofamyloidosis is to reduce production of amyloid precursor proteins via treatment of chronic inflammation in AA-amyloidosisor eradication of the amyloidogenic plasma cell clone in AL-amyloidosis. Both variant and wild-type ATTR patients can be treated with TTR tetramer stabilizers, e.g., tafamidis, thatinterfere with the deposition of misfolded transthyretin.
Systemic amyloidosis, AL, AA, ATTR, clinicalguidelines, diagnosis, treatment.