Amyloidosis

amyloidosis

class of disease

Subclass of	systemic disease, proteostasis deficiency, acquired metabolic disease, pathogenesis
Health specialty	endocrinology, cardiology, hematology
ICD-9-CM	277.3, 277.30
NCI Thesaurus ID	C2868

Amyloidosis be a group of diseases insyd wich abnormal proteins, dem know as amyloid fibrils, build up insyd tissue. Der be chaw non-specific den vague signs den symptoms wey be associated plus amyloidosis. Dese dey include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, den feeling faint with standing.

Insyd AL amyloidosis, specific indicators fi include enlargement of de tongue den periorbital purpura. Insyd wild-type ATTR amyloidosis, non-cardiac symptoms dey include: bilateral carpal tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, small fiber neuropathy, den autonomic dysfunction.

Der be about 36 different types of amyloidosis, each secof a specific protein misfolding. Within dese 36 proteins, 19 be grouped into localized forms, 14 be grouped as systemic forms, den three proteins fi identify as either. Dese proteins fi cam be irregular secof genetic effects, as well as thru acquired environmental factors. De four most common types of systemic amyloidosis be light chain (AL), inflammation (AA), dialysis-related (Aβ₂M), den hereditary den old age (ATTR den wild-type transthyretin amyloid).

Diagnosis fi be suspected wen dem find protein insyd de urine, organ enlargement be present, anaa dem find problems plus multiple peripheral nerves wey e be unclear why. Diagnosis be confirmed by tissue biopsy. Secof de variable presentation, a diagnosis often fi take sam time to reach.

Treatment be geared towards decreasing de amount of de involved protein. Dis sam times fi be achieved by determining den treating de underlying cause. AL amyloidosis dey occur insyd about 3–13 per million people per year den AA amyloidosis insyd about two per million people per year. De usual age of onset of dese two types be 55 to 60 years old. Widout treatment, life expectancy be between six months den four years. Insyd de developed world about one per 1,000 deaths be from systemic amyloidosis. Na dem describe amyloidosis since at least 1639.