Collagen Diseases

Kollagena sjukdomar

Svensk definition

Historiskt en heterogen grupp akuta och kroniska sjukdomar, inklusive reumatoid artrit, systemisk lupus erythematosus (SLE), progressiv systemisk skleros, dermatomyosit osv. Denna indelning baserades på uppfattningen att kollagen var liktydigt med bindväv, men den nuvarande kännedomen om de olika typerna av kollagen och deras distinkta derivat hänförs begreppet kollagena sjukdomar uteslutande till sådana ärftliga tillstånd, där defekten förekommer på gennivå och påverkar kollagenbiosyntes, posttranslationsändringar eller extracellulära processer.

Engelsk definition

Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494)

Svenska synonymer

Kollagenoser

Engelska synonymer

Collagen Disease Disease, Collagen Diseases, Collagen