Amyloidosis

Amyloidosis is a violation of protein metabolism, accompanied by the formation in the tissues of a specific protein-polysaccharide complex (amyloid) and the defeat of many organs and systems.

ICD-10 code

• E85 Amyloidosis.
• E85.0 Hereditary family amyloidosis without neuropathy.
• E85.1 Neurotic hereditary family amyloidosis.
• E85.2 Hereditary family amyloidosis, unspecified.
• E85.3 Secondary systemic amyloidosis.
• E85.4 Limited amyloidosis.
• E85.8 Other forms of amyloidosis.
• E85.9 Amyloidosis, unspecified.

Epidemiology of amyloidosis

The incidence of the disease among men and women with primary amyloidosis is the same. Age of onset of the disease varies from 17 to 60 years, and the duration of the disease - from several months to 23 years. The timing of the onset of the disease is difficult to establish, since the first clinical manifestations do not correspond to the onset of amyloid deposition.

[1], [2], [3], [4], [5], [6], [7],

Causes and pathogenesis of amyloidosis

Depending on the etiology and features of pathogenesis, idiopathic (primary), acquired (secondary), hereditary (genetic), local amyloidosis, amyloidosis in myeloma and APUD-amyloidosis are isolated. The most common secondary amyloidosis, which is by origin approaching nonspecific (in particular, immune) reactions. It develops with rheumatoid arthritis, ankylosing spondylitis, tuberculosis, chronic osteomyelitis, bronchiectasis, less often with lymphogranulomatosis, kidney, lung and other organs, syphilis, ulcerative colitis, Crohn's disease and Whipple, subacute infective endocarditis, psoriasis, etc.

What provokes amyloidosis?

[8], [9], [10], [11], [12], [13], [14]

Symptoms of amyloidosis

The clinical manifestations of amyloidosis are diverse and depend on the location of amyloid deposits, their prevalence. Localized forms of amyloidosis, for example amyloidosis of the skin, are asymptomatic for a long time, like senile amyloidosis, in which deposits of amyloid in the brain, pancreas, heart are often found only at the dissection.

Symptoms of amyloidosis

Classification of amyloidosis

According to the classification of the Nomenclature Committee of the International Union of Immunological Societies (WHO Bulletin, 1993) five forms of amyloidosis are distinguished.

• AL-amyloidosis (A- amyloidosis, amyloidosis, L- light chains, light chains) - primary, associated with myeloma (amyloidosis is recorded in 10-20% of cases of myeloma).
• AA-amyloidosis (acquired amyloidosis, acquired amyloidosis) is a secondary amyloidosis against a background of chronic inflammatory, rheumatic, diseases, as well as with Mediterranean family fever (periodic disease).
• ATTR-amyloidosis (A- amyloidosis, amyloidosis, TTR- transthyretin, transthyretin) - hereditary-familial amyloidosis (familial amyloid polyneuropathy) and senile systemic amyloidosis.
• Aβ ₂ M-amyloidosis (A- amyloidosis, amyloidosis, β ₂ M-β _2- microglobulin) -amyloidosis in patients on planned hemodialysis.
• Localized amyloidosis often develops in people of senile age (AIAPP-amyloidosis - with insulin-independent diabetes mellitus, AV-amyloidosis - in Alzheimer's disease, AANF-amyloidosis - senile amyloidosis of atria).

[15], [16], [17], [18]

Diagnosis of amyloidosis

Amyloidosis should be suspected in nephropathy, persistent severe heart failure, malabsorption syndrome, or polyneuropathy of unclear etiology. In nephrotic syndrome or chronic renal failure, it is necessary to exclude amyloidosis in addition to glomerulonephritis. The probability of amyloidosis increases with hepato- and splenomegaly.

Diagnosis of amyloidosis

[19], [20], [21], [22]

Treatment of amyloidosis

Secondary amyloidosis treats the underlying disease: for rheumatic diseases, immunosuppressive therapy is chosen to suppress the activity of the disease, in chronic purulent processes, antibiotics or surgical treatment are used, cancer is removed from oncological diseases, etc.

How is amyloidosis treated?

Prevention of amyloidosis

Prevention of primary amyloidosis has not been developed. In secondary amyloidosis, prevention is reduced to the timely detection and adequate treatment of diseases that can cause its development.

Forecast

The prognosis for developing amyloidosis is unfavorable. Most patients die within a few years from renal insufficiency. In some cases, the development of remission is possible against the background of taking colchicine and treating the underlying disease.

[23], [24]