The new morphological criteries for prevention of amyloidogenesis and heart failure in cardiac amyloidosis

Abstract Background and objective Cardiac amyloidosis is a severe complication of periodic disease, a hereditary disorder of ancient nations in the Mediterranean basin. Early diagnosis of amyloidosis is only way to prevent an amyloidogenesis and accumulation of amyloid depositions in organs. Methods Clinical and morphological analysis of 120 patients and 107 sectional observations with diagnosis of periodic disease thoracic, abdominal and mixed forms (P±m=5,71±2,27; 22,86±4,09; 71,43±4,4) distributed in 3 groups, by age of first manifestation of disease during the life: under 5, 5-20 and above 20 years (P±m=28,57±4,4; 56,19±4,84; 15,24±3,5) respectively were done. For morphological studies Congo red, specific serum of Monoclonal Mouse Anti-Human Amyloid-A used. Results By immunocytological investigation of blood monocytes of patients, in amounts of 7,0±0,72 in the stage of chronic renal failure, 5,58±0,36 (p<0.05) in nephrotic, 5,55±0,42 in proteinuria, and 4,93±0,45 in latent stage, the fragments of amyloidal fibrillar protein were detected. Cardiac lesions in periodic disease in 47,6% (p<0,05) of cases were clinically latent, and morphological changes in sectional material in 52,4% (p<0,05) of cases were detected. Amyloid cardiopathy in the presence of morphologically expressed cardiomegaly with amyloid masses in the myocardium stroma in 23,8% (p<0,05), and in the background of amyloid cardiopathy atherosclerosis of the coronary vessels in 15,2% (p<0,05) of cases were detected. Amyloid angiopathies, leading to myocardial ischemia, in 3,8% (p>0,05) cases were observed. Conclusions Cardiac amyloidosis leads to the chronic heart failure prevailing in thanatogenesis of periodic disease. The presence of stained by immunochemical specific method of monocytes containing fragments of A-amyloid fibrillar protein, can testify the participance of these cells in the process of incomplete degradation of the serum amyloid-A protein and amyloidogenesis. The letter can use for prognosis and prevention of heart failure in periodic disease.