BACKGROUND AND OBJECTIVES Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis. SUBJECTS AND METHODS We retrospectively studied all patients diagnosed with systemic amyloidosi...

OBJECTIVE To investigate if serum apolipoprotein A-I and A-II (apoAI and apAII) concentrations change in subjects with systemic amyloidosis secondary to underlying disorders. METHODS Serum concentrations of apoAI and apoAII were measured in 21 multiple myeloma patients, including eight with amyloidosis; 95 rheumatoid arthritis patients, including 45 with amyloidosis; and 73 haemodialysis pati...

AMYLOIDOSIS is caused by the accumulation of amyloid fibrils in vital organs, leading to functional disturbances. Approximately 40% of patients with primary amyloidosis have had cardiac amyloidosis. In cardiac amyloidosis, right heart failure is the predominant sign, whereas left heart function is preserved until late in the disease. Because of the cardiac involvement, when patients with cardia...

Amyloidosis includes a spectrum of diseases that involve the deposition of misfolded proteins in various tissues throughout the body. There are many different proteins known to cause amyloidosis and the etiology of the amyloid subtype determines where the abnormal proteins deposit. AL, ATTRwt (formerly senile systemic amyloidosis), and ATTRm (formerly familial amyloidosis) are the most common a...

Abstract Background Bone scintigraphy is extremely valuable when assessing patients with suspected cardiac amyloidosis (CA), but the clinical significance and associated phenotype of different degrees myocardial tracer uptake across types yet to be defined. Purpose We sought define phenotypes varying on bone scintigraphy, multiple systemic using extensive characterisation comprising biomarkers,...

Amyloidosis cutis dyschromica is a rare type of primary cutaneous amyloidosis characterized by reticulate hyper-pigmentation with discrete hypopigmented macules. Up to date, about 50 cases of amyloidosis cutis dyschromica have been reported and the majority are familial cases of Asian ethnicity. Various diseases, particularly autoimmune diseases such as systemic sclerosis and systemic lupus ery...

In several families, multiple endocrine neoplasia type 2A (MEN 2A) has been found in association with cutaneous lichen amyloidosis. It has been debated, however, whether the skin amyloidosis found in MEN 2A families, localized exclusively in the interscapular area, represents the same anomaly as that found in autosomal dominant familial cutaneous lichen amyloidosis, which is more generalized. W...

OBJECTIVE To clarify the clinical significance of the SAA1.3 allele in the development and outcome of AA amyloidosis in Japanese patients with rheumatoid arthritis (RA). METHODS One hundred and twenty RA patients (60 alive and 60 dead) fulfilling the 1987 ACR criteria and 62 RA patients with biopsy-confirmed amyloid A (AA) amyloidosis (36 alive and 26 dead) were enrolled. The SAA1 genotypes w...

protein aggregation is a serious problem for both biotechnology and cell biology. diseases such as prion misfolding, alzheimer’s, and other amyloidosis are phenomena for which protein aggregation in our living cells is of considerable relevance. human lysozyme has been shown to form amyloid fibrils in individuals suffering from nonneuropathic systemic amyloidosis, all of which have point mutati...