Amyloidosis

Amyloidosis (amyloidosis; from the Greek. amylon - starch and eidos - view) - violation of protein metabolism, accompanied intercellular deposition in tissues and organs are dense homogeneous spherical or tsilindroidnym protein mass. These extracellular deposits are stained with iodine as starch and that was Virchow (R. Virchow) a base to suggest the term "amyloid" (kamalodine matter). According to modern concepts amyloid in its chemical structure - glycoprotein, which consists of protein and polysaccharide components are closely connected with each other and with strong resistance to enzymatic action. The protein component is presented by globulins: A1, A2, b and ' symbol. The ratio between globulins vary. From other structural proteins in the amyloid found fibrin, and some amino acids - arginine, tryptophan, histidine, casein, cystine. Polysaccharide component represented by glucosamine associated with chondroitinase acid. Amyloid positive at colouring of PAS. Toluidine blue is blue; when processing sections Genzianella stands out for its red color on the background of surrounding tissues, assumed blue. Amyloid becomes red when exposed to iodine green, logoiski solution paints it in a reddish-brown color. Most commonly used as a reagent for the amyloid Congo red: amyloid becomes red. However, all these tinctorial properties amyloid is not specific: staining of the result can be negative, which gave occasion to speak about the so-called achromatic the amyloid (framilode). Amyloid has positive anisotropy, dikhroizma; electron microscopy of the amyloidosis found many thin fibrous threads that do not have a specific orientation. Next to the fibrous structures visible melkorazmernye.
The pathogenesis of amyloidosis is unclear. Established only two facts: 1) dysproteinemia with the accumulation in the blood plasma coarse proteins (A1, A2, b, V-globulins)that participate in the formation of the amyloid, and 2) transformation of the cells of the reticuloendothelial system - proliferation, plasmafysica and other Remains controversial, which of these phenomena is primary.
The formation of amyloid has the character autoimmunological process in which we can distinguish two stages: 1) appearance of the autoantigens, and related autoantibodies as a result of perversion of the protein synthetic function of the reticuloendothelial system and 2) the reaction of precipitation (antigen-antibody) (VEB).
Amyloidosis in laboratory animals can be reproduced by subcutaneous and intramuscular casein, bacterial toxins, nutrose, ribonuclease sodium, gelatin, proteolytic enzymes, sulphur, cholesterol, methylcholanthrene etc. Serological changes and transformation of cells of the reticuloendothelial system, the experimental data, precede the deposition of amyloid in the tissue (called prednisone stage). The deposition of amyloid contribute to increased permeability of the vascular wall, and inactivation here tissue enzymes (usually these enzymes fix amyloid directly under the endothelium, extracellular).
The first clinical-anatomic classifications of amyloidosis was proposed in 1935 and since that time it was repeatedly modified. However, today there is no single classification that would take into account all forms of amyloidosis. From the most simple modern structure is the classification of Cruche, contributing in humans: 1) General amyloidosis type A-I - secondary; 2) amyloidosis B-II - primary peramelids (may be); 3) amyloidosis type, including transitional forms, and all kinds of atypical A. According to the classification of Briggs form A. are subdivided as follows: 1) primary: a) generalized, b) the family, in) respiratory (focal and diffuse); 2) secondary; 3) senile; 4) A. in myeloma; 5) granulomas A. (all varieties except respiratory).
The division of amyloidosis in the primary and secondary relative and conditional, as all varieties A. common for them by the properties of the amyloid, the reaction of the reticuloendothelial system in the event of amyloid and, finally, serological changes that occur in this disease.
Clinical and pathological characteristics. General amyloidosis type A-I (secondary, typical, Hesse, visceral, parenchymatous) is a consequence of long-term recurrent chronic diseases accompanied suppuration or tissue disintegration and absorption of the decay products (tuberculosis of the lungs, bones, chronic pneumonia with bronchiectasis, chronic osteomyelitis, tertiary syphilis, chlamydia, etc.,). The most characteristic lesions of the spleen, liver, kidneys, adrenal glands, intestines; rarely deposits of amyloid can be observed in the heart, breast, lymph nodes, skin. For a long period of amyloidosis this type flows clinically asymptomatic. Macroscopically bodies, which postponed the amyloid, look dense, increased, have a greasy appearance.
The spleen is the deposition of amyloid in its pulp looks in the context of increased with smooth-dull, greasy surface of red color (so-called sebaceous, or ham, spleen); the deposition of amyloid in the follicles of the last stand above the dark red with the cut surface in the form of translucent grey bumps, reminding grain sago (called the Saga spleen). Deposits of amyloid kidney - see Nephritic syndrome. The deposition of amyloid in the liver under the endothelium mitakovich capillaries can be diffuse and focal, accompanied by atrophy of the parenchyma, although the weight of the body due to fat mass amyloid increases. In the adrenal glands amyloid deposited diffuse, captures all areas of the cortex and its causes atrophy. Macroscopically cortical layer of pale gray, semi-transparent.
A special kind of secondary amyloidosis is A. if multiple myeloma (see).
Primary peramelids B-II (idiopathic, system, diffuse peramelids, atypical amyloidosis, mesenchyme, mesenchimal, autoimmunity) occurs in the absence of any prior chronic process in the body and is characterized by a primary lesion of derivatives mesoderm. Describes the following types of paramilita: Portuguese A. (type Andrade), Indian A. and A. if "familial Mediterranean fever". The first two types are very close to each other: they are characterized by General clinical, morphological, serological changes and may be hereditary (for an autosomal dominant type). Clinical manifestations also similar: a sharp pain in skeletal muscles of lower limbs with symptoms of giperestesia and parestesia, increased temperature sensitivity and phenomena heavy infants with atrophy and paralysis of the muscles, reduction or complete disappearance of tendon reflexes and development of trophic ulcers. Pain in the abdominal cavity are determined in the course of the intestine and are accompanied by indigestion and diarrhea. Picture of heart failure accompanied by disorders of the heart rhythm. Changes in the urinary system are expressed in albuminuria, zilindruria, falling libido menopause and azoospermia. The characteristic symptoms of the Indian version of paramilita is to change from the side view (periarteriit, retinitis).
For paramilita malignant; Indian amyloidosis flows relatively long time - up to 25 years. Death occurs from cachexia, collapse or acceded intercurrent infections. Damaged muscle looks dull, thick, matted and greasy kind of grayish colour. Microscopically identifies the deposition of amyloid in the course of the vessels, connective tissue strips of striated muscle, nerve trunks, sometimes in the thick bundle of nerve fibers. In the heart of amyloid deposited in the stroma of the myocardium, in the course of the vessels, in the sarcolemma muscle fibers and especially abundant under the endocardium, as parietal and valve, which is accompanied by thickening and hardening of the two - and tricuspid valves. It can simulate a picture of Vice. In the stomach and intestines deposits of amyloid observed in the course of the vessels, in the smooth muscle layer. If this is blocked intramural nervous apparatus and impaired absorption capacity of the mucous. Damage to the kidneys and spleen similarly secondary amyloidosis. In the sex glands amyloid deposited in stroma, and in the vessels.


A. if "familial Mediterranean fever is characterized by lesions of the kidneys, liver and spleen. Bouts of fever preceding these lesions or follow them (different phenotypes). The nature of the deposition of amyloid twofold: periradicular (under the intima or the muscular layer of the blood vessels) and peritonectomy (adventitia vessels with the involvement of the media).
For amyloidosis type include: 1) isolated tumor A. (see Amyloid tumor), 2) family allergic urticaria with hyperglobulinemia and amyloid nephropathy; 3) senile amyloidosis, which afflicts the heart, lungs, seminal vesicles, skin and bone (called senile spots). The latter kind clinically often bessimptomnom and is found at autopsy in patients over 70 years of age, died from any random reason.
The diagnosis of secondary A. based on the anamnesis, the analysis of urine and serological tests. Used diagnostic test with Congo red (primary amyloidosis it doesn't matter). Recognition of the primary A. used cytological examination of mucous membranes of the mouth and rectum, x-ray examinations of gastrointestinal tract, a biopsy of the liver and spleen, punctata which found glybki amyloid.
The prognosis is poor. Experimental data indicate the possibility of reverse development A. However, in the clinic of such observations are rare.
Cases resorption of amyloid in the treatment of hormone (ACTH and cortisone) and hepatic drugs, probably due to the fact that amyloidosis was recognized in the early stages of development.
Important in practical terms, the question about the conditions conducive to the resolution of amyloid masses, and the extent to which it can be done, remains open.
amyloidosis skinAmyloidosis skin - amyloid deposits in the skin is manifested in the following three options.
1. Lichen amyloidosus [Freundenthal; synonym amyloidosis cutis localis nodularis et disseminata (Gutmann)] reminds atopic dermatitis: a rich semi-transparent, thick, itchy, shiny flesh-colored nodules (Zwettl., Fig. 5), less bluish. Lichenification when it itches.
2. Amyloidosis cutis localis secundaria (secondary amyloidosis skin); there are knots, infiltrates, warty and knotted elements. Accompanies A. internal organs in patients with chronic diseases.
3. Amyloidosis cutis metabolica (Konigstein - system, General A. skin (rare): xanthomatose plaques, yellow nodules on pale porcelain skin, larger under the Breasts in women; in the inguinal folds where the lesions coalesce, skin darkens. Characteristic macroglossia, language becomes dense, loss of motion, pale mucosa arise yellow nodules, petechiae.
The diagnosis is confirmed in red areas defeat when injected into the skin of a solution of the Congo red, which is not observed in a similar medicine (knotty prurigo, lichen obtusus and others). The deposition of amyloid in the skin on histological preparations are identified by the same methods and dyes, and that the deposition of amyloid in the internal organs.
Treatment - vitamin a (up to 100 000 ME a day), treatment of diseases, against which arose amyloidosis skin.