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Lecture notes, cheat sheets
Childhood diseases. Diffuse connective tissue diseases. Systemic lupus erythematosus. Dermatomyositis. Systemic scleroderma (lecture notes)
Directory / Lecture notes, cheat sheets Table of contents (expand) LECTURE № 13. Diffuse connective tissue diseases. Systemic lupus erythematosus. Dermatomyositis. Systemic scleroderma 1. Systemic lupus erythematosus Systemic lupus erythematosus is a chronic polysyndromic disease of connective tissue and blood vessels that develops due to genetically determined imperfection of immunoregulatory processes. Etiology. The significance of a viral infection against the background of genetically determined immune disorders is assumed. Pathogenesis. The formation of circulating autoantibodies, of which antinuclear antibodies are of the most important diagnostic and pathogenetic importance; the formation of circulating immune complexes, which, deposited on the basement membranes of various organs, cause their damage and inflammation. This is the pathogenesis of nephritis, dermatitis, vasculitis, etc. This hyperreactivity of humoral immunity is associated with disorders of cellular immunoregulation. Recently, importance has been attached to hyperestrogenemia, accompanied by a decrease in the clearance of circulating immune complexes, etc. A family genetic predisposition has been proven. Mostly young women and teenage girls are ill. Provoking factors are insolation, pregnancy, abortion, childbirth, the onset of menstrual function, infections (especially in adolescents), drug or post-vaccination reactions. Clinic. The disease begins gradually with recurrent polyarthritis and asthenia. Less common is an acute onset (high fever, dermatitis, acute polyarthritis). Subsequently, a relapsing course and characteristic polysyndromic character are noted. Polyarthritis and polyarthralgia are the most common and early symptom of the disease. Mostly small joints of the hands (wrist, ankle) are affected, and less commonly the knee joints. A non-erosive type of polyarthritis is characteristic, even in the presence of deformation of the interphalangeal joints, which develops in 10-15% of patients with a chronic course. Erythematous rashes on the skin of the face in the form of a butterfly, on the upper half of the chest in the form of a décolleté, on the extremities are also a common sign of systemic lupus erythematosus. Polyserositis is considered a component of the diagnostic triad along with dermatitis and polyarthritis. It is observed in almost all patients in the form of bilateral pleurisy and pericarditis, less commonly perihepatitis and (or) perisplenitis. Characterized by damage to the cardiovascular system with the development of pericarditis, which is accompanied by myocarditis. Relatively often observed warty endocarditis Libman-Sacks with damage to the mitral, aortic and tricuspid valves. Signs of vascular damage are included in the picture of damage to individual organs. Nevertheless, it should be noted the possibility of developing Raynaud's syndrome (long before the typical picture of the disease), damage to both small and large vessels with corresponding clinical symptoms. Lung lesions may be associated with the underlying disease in the form of lupus pneumonitis, characterized by cough, shortness of breath, unvoiced moist rales in the lower parts of the lungs. X-ray examination in such patients reveals an increase and deformation of the pulmonary pattern in the basal sections of the lungs, at times focal-like tones can be detected. Since pneumonitis usually develops against the background of current polyserositis, the described radiographic symptoms are supplemented by a high standing of the diaphragm with signs of pleurodiaphragmatic and pleuropericardial adhesions and discoid atelectasis (linear shadows parallel to the diaphragm). In the study of the gastrointestinal tract, aphthous stomatitis, dyspeptic syndrome and anorexia are noted. Painful abdominal syndrome can be associated both with the involvement of the peritoneum in the pathological process, and with vasculitis proper - mesenteric, splenic, etc. Segmental ileitis develops less frequently. The defeat of the reticuloendothelial system is expressed in an increase in all groups of lymph nodes - a very frequent and early sign of the systemic nature of the disease, as well as in an increase in the liver and spleen. Actually lupus hepatitis develops extremely seldom. However, liver enlargement may be due to heart failure with pancarditis or severe effusion pericarditis, as well as the development of fatty liver. Lupus diffuse glomerulonephritis (lupus nephritis) develops in half of the patients, usually during the period of generalization of the process. There are various variants of kidney damage - urinary, nephritic and nephrotic syndromes. For the recognition of lupus nephritis, intravital puncture biopsy with immunomorphological and electron microscopic examination of the kidney biopsy is of great importance. The development of renal pathology in patients with recurrent articular syndrome, fever and persistently elevated ESR requires the exclusion of lupus nephritis. It should be remembered that almost every fifth patient with nephrotic syndrome has systemic lupus erythematosus. The defeat of the neuropsychic sphere occurs in many patients in all phases of the disease. At the onset of the disease - asthenovegetative syndrome, subsequently develop signs of damage to all parts of the central and peripheral nervous system in the form of encephalitis, myelitis, polyneuritis. Damage to the nervous system in the form of meningoencephalomyelopolyradiculoneuritis is characteristic. Epileptiform seizures are less common. Hallucinations (auditory or visual), delusional states, etc. are possible. Diagnostics is carried out on the basis of clinical and laboratory data that are of diagnostic value: the determination of a large number of LE cells, high titers of antibodies to DNA, especially to native DNA, to deoxyribonucleoprotein, Sm antigen. Treatment. Non-steroidal anti-inflammatory drugs and aminoquinoline derivatives are indicated. The first ones are recommended for articular syndrome. Immunosuppressants: B vitamins, ascorbic acid in the form of a spring-autumn course. Patients are shown treatment in sanatoriums of the local type (cardiological, rheumatological). Climatobalneological, physiotherapeutic treatment is contraindicated, since ultraviolet irradiation, insolation and hydrotherapy can exacerbate the disease. 2. Scleroderma localized Localized scleroderma is a chronic systemic disease of connective tissue and small vessels with widespread fibro-sclerotic changes in the skin and stroma of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome. Big Criterion. Scleroderma-like skin changes proximal to the major finger joints. Small criteria. 1. Sclerodactyly. 2. Dimpled scars or loss of soft tissue substance at the fingertips and/or feet. 3. Bilateral basal pulmonary fibrosis. The diagnosis of scleroderma is considered reliable if either a major criterion or at least two minor criteria are met. Etiology. Unknown. Provoking factors are cooling, trauma, infection, vaccination, etc. Pathogenesis. Of leading importance is a violation of collagen metabolism associated with the functional hyperactivity of fibroblasts and smooth muscle cells of the vascular wall. Also a factor in pathogenesis is a violation of microcirculation caused by damage to the vascular wall and changes in the intravascular aggregate properties of blood. Systemic scleroderma is a typical collagen disease associated with excessive collagen formation (and fibrosis), functionally defective fibroblasts and other collagen-forming cells. Family genetic predisposition matters. Women get sick 3 times more often than men. Clinic. The clinical picture usually begins with Raynaud's syndrome (vasomotor disturbances), trophic disorders and persistent arthralgia, weight loss, increased body temperature, and asthenia. Having begun with any one symptom, systemic scleroderma gradually or quite quickly acquires the features of a multi-syndromic disease. Skin lesions are a pathognomonic sign of the disease. This is a common dense edema, followed by thickening and atrophy of the skin. More often, changes are localized on the skin of the face and extremities, often the skin of the entire body is dense. At the same time, focal or widespread pigmentation develops with areas of depigmentation, telangiectasia. Ulcerations and pustules on the fingertips are characteristic, which do not heal for a long time and are extremely painful, deformation of the nails, hair loss up to baldness. Fibrosing interstitial myositis often develops. Muscular syndrome is accompanied by myalgia, progressive thickening, then muscle atrophy, and a decrease in muscle strength. And rarely there is acute polymyositis with pain, muscle swelling, etc. Fibrosing changes in the muscles are accompanied by tendon fibrosis, which leads to muscle-tendon contractures. Joint damage is mainly associated with pathological processes in periarticular tissues (skin, tendons, articular bags, muscles). Arthralgias are accompanied by a pronounced deformity of the joints due to proliferative changes in the periarticular tissues. X-ray examination does not reveal significant destruction. An important diagnostic sign is osteolysis of the terminal, and in severe cases, the middle phalanges of the fingers, less often the legs. The deposition of calcium salts in the subcutaneous tissue is localized mainly in the area of the fingers and periarticular tissues, expressed in the form of painful uneven formations, sometimes spontaneously opening with rejection of crumbly calcareous masses. Damage to the cardiovascular system observed in almost all patients: the myocardium and endocardium are affected. Scleroderma cardiosclerosis is clinically characterized by pain in the region of the heart, shortness of breath, extrasystole, muffled tones and systolic murmur at the apex, expansion of the heart to the left. In X-ray examination, a weakening of the pulsation and smoothness of the contours of the heart are noted, with X-ray kymography - silent zones in areas of macrofocal cardiosclerosis, and in the most severe cases, an aneurysm of the heart is formed due to the replacement of muscle tissue with fibrous tissue. On the ECG, a decrease in voltage, conduction disturbances up to atrioventricular blockade are usually observed; heart attack-like ECG occurs with the development of massive foci of fibrosis in the myocardium. If the process is localized in the endocardium, the development of scleroderma heart disease and damage to the parietal endocardium is possible. The mitral valve is usually affected. Scleroderma heart disease is characterized by a benign course. Damage to small arteries, arterioles causes such peripheral symptoms of scleroderma as Raynaud's syndrome, gangrene of the fingers. Damage to the vessels of internal organs leads to severe visceral pathology - hemorrhages, ischemic and even necrotic changes with a clinical picture of severe visceritis (decay of lung tissue, "true scleroderma kidney", etc.). Vascular pathology determines the speed of the process, its severity and often the outcome of the disease. At the same time, it is possible to damage large vessels with a clinical picture of thromboangiitis obliterans; ischemic phenomena develop, and often gangrene of the fingers, toes, migrating thrombophlebitis with trophic ulcers in the feet and legs, etc. Lung injury in the form of diffuse or focal pneumofibrosis, accompanied by emphysema and bronchiectasis. Complaints of shortness of breath, difficulty in taking a deep breath, hard breathing, when auscultating the lungs, wheezing is heard, a boxy tint of percussion sound, a decrease in vital capacity to 40-60% of the expected value, bilateral strengthening and deformation of the pulmonary pattern, sometimes with a fine-mesh structure (honeycomb); X-ray examination shows signs that usually characterize scleroderma pneumofibrosis. Kidney damage manifests itself more often with focal nephritis, and diffuse glomerulonephritis with hypertension and renal failure may develop. With a rapidly progressive course of systemic scleroderma, a true scleroderma kidney often develops, leading to focal necrosis of the cortex and renal failure. Esophageal injury, manifested by dysphagia, expansion, weakening of peristalsis and rigidity of the walls with a slowdown in the passage of barium during X-ray examination, is observed very often and is of great diagnostic value. In connection with the defeat of the vessels, the development of ulcers, hemorrhages, ischemic necrosis and bleeding in the digestive tract is possible. Damage to the nervous system manifested by polyneuritis, vegetative instability (impaired sweating, thermoregulation, vasomotor skin reactions), emotional lability, irritability, tearfulness and suspiciousness, insomnia. Only in rare cases does a picture of encephalitis or psychosis occur. Possible symptoms of sclerosis of cerebral vessels due to their scleroderma lesions, even in young people. There is damage to the reticuloendothelial (polyadenia, and in some patients hepatosplenomegaly) and endocrine (pluriglandular insufficiency or pathology of one or another endocrine gland) systems. More often there is a chronic course, the disease lasts for decades with minimal activity of the process and the gradual spread of lesions to various internal organs, the function of which is not disturbed for a long time. Such patients suffer mainly from lesions of the skin, joints and trophic disorders. Within the framework of chronic systemic scleroderma, CRST syndrome (calcinosis, Raynaud's syndrome, sclerodactyly and telangiectasia) is distinguished, characterized by a long benign course with an extremely slow development of visceral pathology. In the subacute course, the disease begins with arthralgia, weight loss, visceral pathology quickly increases, and the disease takes on a steadily progressive course with the spread of the pathological process to many organs and systems. Death usually occurs 1-2 years from the onset of the disease. Forms of focal scleroderma: plaque - puffiness, erythema, violations of the skin pattern and pigmentation with a lilac-pink "corolla", induration, fibrosis, atrophy. Keloid form - dense strands resembling a keloid scar. Linear form - a lesion along the neurovascular bundle, affecting the fascia, muscles and bones; areas of lipodystrophy, amyotrophy, shortening of the tendons and dysplasia of the limb. Deep subcutaneous nodular form is localized on the thighs and buttocks with the involvement of the fascia. Isolated nodules along the tendons, resembling rheumatoid, and transforming calcifications. Atypical forms - idiopathic atrophoderma (bluish-violet spots on the body without previous seals); Buschke's scleroma is a pseudoscleroderma disease (dense swelling of the dermis and subcutaneous tissue). Diagnostics. Based on clinical and laboratory data: moderate, normo- or hypochromic anemia, moderate leukocytosis and eosinophilia, and transient thrombocytopenia are usually observed. ESR is normally or moderately increased in chronic cases and significantly increased (up to 50-60 mm/h) in subacute cases. Treatment. Antifibrotic agents (D-penicillamine, diuciphone, colchicine, enzymatic drugs, dimethyl sulfoxide). Use of NSAIDs. Treatment with immunosuppressants (suppress the autoimmune inflammatory process in connective tissue, inhibit excessive fibrosis formation), prescription of glucocorticoids. The use of antihypertensive and microcirculation-improving agents. Local therapy, massage, exercise therapy. Symptomatic treatment for damage to the digestive organs. Spa treatment. 3. Dermatomyositis Dermatomyositis is a systemic disease of the skeletal and smooth muscles and skin. Etiology. Unknown. Suspected viral (Coxsackie B2) the etiology of dermatomyositis. Provocative factors - cooling, insolation, trauma, pregnancy, drug intolerance, vaccination, stressful situations. Pathogenesis. Various immunopathological disorders. Clinic. The disease begins acutely or subacutely with muscle syndrome (myasthenia gravis, myalgia), arthralgia, fever, skin lesions, dense widespread edema. Skin lesions are varied. More often it is purple paraorbital erythema with or without edema, erythema over the extensor surfaces of the joint, sometimes with atrophic scars. Damage to skeletal muscles is observed in the form of myalgia during movement and even at rest, with pressure and increasing muscle weakness. The muscles of the shoulder and pelvic girdle become denser, increase in volume, active movements are significantly impaired, the patient cannot sit down independently, lift his limbs, lift his head from the pillow, or hold it while sitting or standing. With a significant spread of the process, patients are essentially completely immobilized, and in severe cases they are in a state of complete prostration. Damage to skeletal muscles (weakness, pain, swelling, induration, malnutrition, calcification). Visceral-muscular syndrome, damage to symmetrical (mainly proximal) muscle groups, facial muscles leads to masking of the face, damage to the pharyngeal muscles leads to dysphagia, and damage to the intercostal and diaphragm muscles leads to respiratory failure, a decrease in lung capacity, and hypoventilation. The oculomotor muscles may be affected with the development of diplopia, strabismus, bilateral ptosis of the eyelids, etc. Polyarthralgia during movement and limitation of joint mobility up to the development of ankylosis are mostly due to muscle damage. An inflammatory or dystrophic myocardial injury is observed, which is manifested by persistent tachycardia and pulse lability, enlargement of the heart to the left, muffled tones, systolic murmur at the apex, arterial hypotension. Lung damage is associated with the underlying disease, more often it is due to infection, to which patients are predisposed due to hypoventilation of the lungs. The gastrointestinal tract is involved in the process: anorexia, abdominal pain, symptoms of gastroenterocolitis, hypotension of the upper third of the esophagus are noted. Damage to the peripheral and central nervous system. Diagnostics. Weakness of the proximal muscles for at least 1 month, myalgia for 1 month in the absence of sensory impairment, the ratio of the concentration of creatine in the urine to the sum of the concentrations of creatine and creatinine in the urine exceeds 40%, A significant increase in the blood level of creatine phosphokinase or transaminases in the absence of other causes, degenerative changes muscle fibers on biopsy. Four signs - a reliable diagnosis, three signs - a probable diagnosis, two signs - a possible diagnosis. The diagnosis is made on the basis of clinical, laboratory data. In the blood, moderate leukocytosis with severe eosinophilia, a moderate increase in ESR, and hypergammaglobulinemia are usually observed. Biochemical studies of blood and urine and muscle biopsy are of diagnostic importance, especially in chronic and subacute course (thickening of muscle fibers is detected with loss of transverse striation, fragmentation and dystrophy up to necrosis, a significant cellular reaction is observed - accumulation of lymphocytes, plasma cells, etc. ). Treatment. In acute and subacute cases, glucocorticoids are indicated in large daily doses (prednisolone). After achieving the effect, the dose of corticosteroids is reduced very slowly (0,5 tablets every 7-10 days) to a maintenance dose against the background of delagil (0,25 g), plaquenil (0,2 g) 1 tablet after dinner. With the development of stable remission, glucocorticoids can be completely discontinued. In complex treatment, B vitamins and ascorbic acid are recommended. With severe muscle fatigue, prozerin and its analogues in normal doses, ATP are indicated. With early treatment with adequate doses of corticosteroids in patients with acute dermatomyositis, a stable recovery occurs. In the subacute course, it is usually possible to achieve only remission, supported by glucocorticoids. In chronic dermatomyositis, the disease becomes undulating. Author: Gavrilova N.V. << Back: Kidney diseases in children. Acute renal failure (ARF). Chronic renal failure (CRF) (Kidney failure. Prerenal acute renal failure. Renal acute renal failure. Partial chronic renal failure) >> Forward: Hemorrhagic diseases in children, hemorrhagic diathesis, thrombocytopenic purpura, hemorrhagic vasculitis. Hemophilia, emergency treatment for bleeding (Hemorrhagic diathesis and syndromes. Types of bleeding. Thrombocytopenic purpura. Hemorrhagic vasculitis (Schönlein-Henoch disease). Hemophilia A and B)
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