Currents Concepts on the Immunopathology of Amyloidosis

Abstract

Amyloidosis is defined as the extracellular accumulation at systemic or organ-specific level of insoluble low molecular weight protein fibrils manifesting a beta pleated sheet configuration and a characteristic staining pattern. Several different types of proteins may lead to this phenomenon, and amyloidosis is defined by the biochemical nature of the protein in the deposits and further classified according to whether the deposits are localized or systemic, acquired or inherited, and by the resulting clinical phenotype. Amyloidosis includes subtypes such as light chain, associated with serum amyloid A protein, heritable and familial forms, dialysis-related disease, and organ-specific conditions. The pathogenesis and clinical features of these clinical and pathological entities will be critically discussed in this review article.

Structural mechanism of serum amyloid A-mediated inflammatory amyloidosis

“…Secondary amyloidosis, characterized by the deposition of AA amyloid in tissues and organs, is a major complication of many chronic inflammatory diseases (1,2). However, the structural basis of AA amyloidosis remains largely unknown.…”

“…The overall incidence of systemic secondary amyloidosis in Western nations ranges from 0.5 to 0.86%, but increases significantly in certain patients (2)(3)(4). Deposition of the amyloid fibril in secondary amyloidosis (AA amyloid) can lead to progressive loss of splenic and renal functions.…”

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

“…Although recognized in less than 10% of patients [48], a positive Congo red tissue with no evidence for systemic disease may be encountered. Localized amyloidosis is typically seen in mucosal surfaces, such as the gastrointestinal tract (GIT), urinary tract, breasts, lungs and upper airways, as well as the skin [49]. It probably stems from a localized production of monoclonal LCs, and is thought to involve local amyloid phagocytic processing which provides the amyloidogenic properties of the LCs [50].…”

Abdominal distension and diarrhea as the main symptoms of primary amyloidosis: A case report and literature review

“…A total of 6 types of amyloidosis exist; these are primary, secondary, hemodialysis-associated, hereditary, senile and localized (2). The most common of these 6 types is primary amyloidosis, the prevalence of which is ~4.5 in 10,000 cases (3).…”