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...

Inflammation-related (AA) amyloidosis is a severe clinical disorder characterized by the systemic deposition of the acute-phase reactant serum amyloid A (SAA). SAA is normally associated with the high-density lipoprotein (HDL) fraction in plasma, but under yet unclear circumstances, the apolipoprotein is converted into amyloid fibrils. AA amyloid and heparan sulfate (HS) display an intimate rel...

Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sjögren's syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75%. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is u...

BACKGROUND Autologous hematopoietic cell transplantation (THA) in patients with multiple myeloma and amyloidosis is the standard of care to promote disease free survival and quality of life. AIM To report our experience with THA in patients with multiple myeloma. MATERIAL AND METHODS Retrospective review of the hematopoietic cell transplantation database of a hospital of a Medical School. F...

AL amyloidosis is caused by clonal plasma cells that produce immunoglobulin light chains which misfold and get deposited as amyloid fibrils. Therapy directed against the plasma cell clone leads to clinical benefit. Melphalan and corticosteroids have been the mainstay of treatment for a number of years and the recent availability of other effective agents (IMiDs and proteasome inhibitors) has in...

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...

progress in Kumamoto, Japan (1967–2010). Proc Jap Acad Ser B Phys Biol Sci Nippon Gakushiin 2010;86:694–706. 4. Rapezzi C, Quarta CC, Obici L, Perfetto F, Longhi S, Salvi F, Biagini E, Lorenzini M, Grigioni F, Leone O, Cappelli F, Palladini G, Rimessi P, Ferlini A, Arpesella G, Pinna AD, Merlini G, Perlini S. Disease profile and differential diagnosis of hereditary transthyretin-related amyloid...

INTRODUCTION Amyloidosis in elderly individuals can be an independent alteration and a characteristic of aging. However, the clinical, pathophysiologic, and biochemical characteristics of amyloidosis related to age remain uncertain. OBJECTIVE The purpose of this study was to determine the extent to which the heart and/or the brain of individuals aged 60 years or over exhibits amyloid deposits...