LECTURE No. 9. Differential diagnosis of diffuse connective tissue diseases in children. Clinic, diagnosis, treatment

Classification of rheumatic diseases.

1. Rheumatism.

2. Juvenile rheumatoid arthritis.

3. Ankylosing spondylitis.

4. Other spondyloarthropathies.

5. Systemic lupus erythematosus.

6. Vasculitis:

1) hemorrhagic vasculitis (Sheklein-Genoch);

2) periarteritis nodosa (polyarteritis in young children, Kawasaki disease, Wegener's disease);

3) Takayasu's arteritis.

7. Dermatomyositis.

8. Scleroderma.

9. Difficult to classify rheumatic syndromes.

10. Various diseases associated with rheumatic symptoms and signs in children:

1) benign rheumatoid nodules;

2) erythema nodosum;

3) Lyme disease;

4) sarcoidosis;

5) Steven-Johnson syndrome;

6) Goodpasture's syndrome;

7) symptoms of fibrositis and fibromyalgia;

8) Behçet's syndrome;

9) Sjögren's syndrome.

11. Non-rheumatic diseases, clinically similar to rheumatic ones.

Diffuse connective tissue diseases (DCCT, collagenoses) are an immunopathological lesion of connective tissue with systemic damage to blood vessels and various organs, a progressive course.

In the etiology of DBST, 3 leading factors can be distinguished.

The 1st factor is genetic predisposition, which is confirmed by epidemiological studies, illness of close relatives, twins and the detection of markers of certain diseases using the HLA system.

The 2nd factor is a trigger mechanism, which is viruses containing RNA and slowly reacting (retro viruses), and a chronic persistent infection transmitted transplacentally.

3rd factor - permissive: stressful situation, hyperinsolation, hypothermia.

For all DBST, the degree of disease activity is determined; the nature of the flow; the presence of visceral lesions: the functional ability of the patient in terms of the state of the organs and systems involved in the process; nature of complications. In the prodrome of DBST there are a number of common signs that unite various nosological forms: unmotivated weakness, physical inactivity, decreased appetite and body weight; fever resistant to antibiotics, non-steroidal anti-inflammatory drugs and responsive to treatment with GCs; damage to the skin and mucous membranes; articular syndrome; lymphadenopathy; enlarged liver and spleen; persistent mono- or polyviscerites. In laboratory diagnostics, most DBST (except for SSD) are typical: high acute phase indicators (ESR, sialic acids CRP, etc.); some are more specific (LE cells, HLA, etc.).

In the DBST group, 4 "large collagenoses" are distinguished.

1. Systemic lupus erythematosus (SLE).

2. Systemic scleroderma (SSD).

3. Progressive sclerosis.

4. Dermatomyositis (DM).

1. Systemic lupus erythematosus

Systemic lupus erythematosus is a chronic polysyndromic disease of connective tissue and blood vessels that develops due to a genetically determined imperfection of immunoregulatory processes.

Etiology. The significance of a viral infection against the background of genetically determined immune disorders is assumed.

Pathogenesis: circulating antibodies are formed, of which antinuclear antibodies are of important diagnostic and pathogenetic importance; the formation of circulating immune complexes that are deposited on the basement membranes of various organs, causing their damage and inflammation. Hyperreactivity of humoral immunity is associated with a violation of cellular immunoregulation. Recently, importance has been attached to hyperestrogenemia, accompanied by a decrease in clearance. Family genetic predisposition; girls and women are more often affected. Provoking factors: insolation, pregnancy, abortion, childbirth, onset of menstruation, 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). In the future, a relapsing course is noted. Polyarthritis, polyarthralgia are an early symptom of the disease. Damage to small joints of the hands, wrists, ankles, and less commonly, knee joints. Erythematous rashes on the skin of the face in the form of a “butterfly”, in the upper half of the chest in the form of a décolleté, and on the extremities are also a characteristic sign of systemic lupus erythematosus. Polyserositis, dermatitis, polyarthritis - a diagnostic triad. Characterized by damage to the cardiovascular system with the development of pericarditis, which is accompanied by myocarditis. Warty Libman-Sachs endocarditis with damage to the mitral, aortic and tricuspid valves is relatively often observed. Damage to blood vessels with the development of Raynaud's syndrome (long before the typical picture of the disease), damage to small and large vessels with corresponding clinical symptoms. Lung damage may be associated with the underlying disease in the form of lupus pneumonitis, manifested by cough, shortness of breath, and moist rales on auscultation in the lower parts of the lungs. An X-ray examination can reveal an enhanced pulmonary pattern and deformation of the pulmonary pattern in the basal parts of the lungs, focal-like shadows are revealed. When examining the gastrointestinal tract, aphthous stomatitis is revealed, dyspeptic syndrome and anorexia develop. Abdominal pain syndrome develops when the peritoneum is involved in the pathological process, mesenteric and splenic vasculitis develops, and segmental ileitis develops. Damage to the reticuloendothelial system manifests itself in the form of enlargement of all groups of lymph nodes, enlargement of the liver, and spleen. Lupus hepatitis develops very rarely, but an enlarged liver can be detected in heart failure, pancarditis, severe effusion pericarditis, and with the development of fatty liver. Lupus diffuse glomerulonephritis (lupus nephritis) develops in some patients during the generalization of the process. You can find different types of kidney damage: urinary syndrome, nephritic syndrome, nephrotic syndrome. When recognizing lupus nephritis, a puncture biopsy with immunomorphological and electron microscopic examination of a kidney biopsy is of great importance. The development of renal pathology with recurrent articular syndromes, fever, increased ESR, which requires the exclusion of lupus nephritis. It must be remembered that every fifth patient with nephrotic syndrome may have systemic lupus erythematosus and damage to the neuropsychic sphere in patients can develop in all phases of the disease. At the initial stage, asthenovegetative syndrome is diagnosed, then characteristic signs of damage to the central and peripheral nervous system appear in the form of encephalitis, polyneuritis, and myelitis. Very rarely, epileptiform seizures may develop. The development of hallucinations (auditory or visual), delusional states, etc. is possible.

Treatment. Non-steroidal anti-inflammatory drugs and aminoquinoline derivatives are indicated. Nonsteroidal anti-inflammatory drugs are prescribed for articular syndrome. Immunosuppressants, B vitamins, ascorbic acid during the spring-autumn course. Patients are prescribed treatment in local sanatoriums (cardiological, rheumatological). Climatobalneological and physiotherapeutic treatment is contraindicated, since ultraviolet irradiation, insolation and hydrotherapy can cause an exacerbation of the disease.

2. Scleroderma (Morphea) localized

This is a chronic systemic disease of connective tissue, small vessels with widespread fibrous-sclerotic changes in the skin and stroma of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome.

Major criteria: scleroderma-like skin changes proximal to the major finger joints. Minor criteria:

1) sclerodactyly;

2) dimpled scarring or loss of soft tissue substance in the tips of the fingers, hands and/or feet;

3) bilateral basal fibrosis of the lungs. The diagnosis of scleroderma is considered reliable if either a major criterion or at least two minor criteria are present. Etiology. Etiology unknown.

Provoking factors are cooling, injury, infection, vaccination, etc.

Pathogenesis. Of particular importance is the disruption of collagen metabolism, which is associated with the functional hyperreactivity of fibroblasts and smooth muscle cells of the vascular wall. Also a factor in pathogenesis is a violation of microcirculation associated with damage to the vascular wall and changes in the intravascular aggregate properties of blood.

Systemic scleroderma is a collagen disease that is associated with increased collagen formation and fibrosis, functionally defective fibroblasts and other collagen-forming cells. The predisposition is family-genetic; women are affected 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. Systemic scleroderma begins with one symptom and quickly acquires the features of a multisyndromic disease. Skin lesions are a characteristic symptom of the disease. The most common is dense swelling, which subsequently leads to thickening and atrophy of the skin. More often, the changes are localized on the skin of the face and limbs; very rarely, the skin of the entire body becomes dense. At the same time, focal or widespread pigmentation develops with areas of depigmentation and telangiectasia. The characteristic ulcerations and pustules on the fingertips do not heal for a long time, they are very painful, deformation of the nails, hair loss and even baldness appear. Fibrosing interstitial myositis often develops. Muscle syndrome manifests itself in the form of myalgia, characterized by progressive hardening, then muscle atrophy, and a decrease in muscle strength. And acute polymyositis with pain and muscle swelling is rarely observed. Fibrosis changes in muscles can be accompanied by tendon fibrosis, which leads to muscle-tendon contractures. Joint damage is associated mainly with the pathological process of peri-articular tissue (skin, tendons, joint capsules, muscles). Arthralgia is accompanied by severe deformation of the joint due to proliferative changes in the periarticular tissue; X-ray examination does not reveal significant destruction. A special diagnostic sign is osteolysis of the terminal, and also in severe cases, the middle phalanges of the fingers, and very rarely of the toes. The deposition of calcium salts in the subcutaneous tissue is most often localized in the areas of the fingers and periarticular tissues, which is expressed in the form of painful uneven formations that can spontaneously open with the deposition of crumbly calcareous masses. Damage to the cardiovascular system is observed in almost all patients; the myocardium and endocardium are affected. Sclerodermic cardiosclerosis is clinically characterized by pain in the heart, shortness of breath, and extrasystole. muffled tones, systolic murmur at the apex, expansion of the borders of the heart to the left. X-ray examination reveals a weakening of pulsation, smoothness of the contours of the heart; X-ray kymography can reveal silent zones in areas of large-focal cardiosclerosis, and in more severe cases, a cardiac aneurysm may form due to the replacement of muscle tissue with fibrous tissue. The ECG shows a decrease in voltage, conduction disturbances up to atrioventricular block; an infarction-like ECG is recorded with the development of massive foci of fibrosis in the myocardium. When 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 defect is characterized by a benign course. When small arteries and arterioles are damaged, the following peripheral symptoms of scleroderma are revealed: Raynaud's syndrome, gangrene of the fingers. Damage to the vessels of internal organs leads to severe visceral pathology of hemorrhage, ischemic, even necrotic changes with the clinical picture of visceritis (decay of lung tissue, “true scleroderma kidney”), With vascular Pathology determines the speed of the process, its severity and outcome of the disease. At the same time, damage to large vessels with the clinical manifestations of thromboangiitis obliterans is also possible; ischemic symptoms can be identified, namely gangrene of the fingers, toes, migrating thrombophlebitis with trophic ulcers in the feet, legs, etc. n Lung damage in the form of diffuse or focal pneumofibrosis is accompanied by emphysema and bronchiectasis. Complaints of shortness of breath. difficulty in taking a deep breath, hard breathing, wheezing is heard during auscultation of the lungs, with percussion there is a boxy tint of the percussion sound, with FVD a decrease in the vital capacity of the lungs to 40-60% of the proper one, bilateral strengthening and deformation of the pulmonary pattern, you can sometimes identify a fine-mesh structure in the form of a “honeycomb” ", an X-ray examination of the chest organs reveals signs that are characteristic of scleroderma pneumofibrosis. Kidney damage is most often manifested by focal nephritis and the development of diffuse glomerulonephritis with hypertensive syndrome and renal failure is possible. With the rapid progression of systemic scleroderma, a “true scleroderma kidney” sometimes develops, leading to focal necrosis of the cortex and the development of renal failure. When the esophagus is damaged, the clinical picture is in the form of dysphagia, expansion and weakening of peristalsis, rigidity of the walls with a slowdown in the passage of barium during X-ray examination, which is often observed and has important diagnostic significance. In connection with the affected vessels, the development of ulcerations, hemorrhages, ischemic necrosis and bleeding in the digestive tract is possible. Damage to the nervous system is clinically manifested by polyneuritis, vegetative instability (impaired sweating, thermoregulation, vasomotor reactions of the skin), emotional lability, irritability, tearfulness, suspiciousness, insomnia. Sometimes, in rare cases, a picture of encephalitis or psychosis appears. It is possible to develop a clinical picture of cerebral vascular sclerosis in connection with their sclerodermatous lesions, even in young people. Damage to the reticuloendothelial system in the form of polyadenia is detected, and in some patients hepatosplenomegaly and damage to the endocrine system in the form of pathology of one or another endocrine gland. Systemic scleroderma often has a chronic course, and the disease continues for several decades with minimal process activity and gradual spread of damage to various internal organs, the functions of which are not impaired for a long time. These patients suffer mainly from diseases of the skin and joints with trophic disorders. In chronic scleroderma, CRST is distinguished - a syndrome that manifests itself as calcification, Raynaud's syndrome, sclerodactyly, telangiectasia, characterized by a long-term benign course with an extremely slow development of visceral pathology. In the subacute course, systemic scleroderma begins with arthralgia, weight loss, visceral pathology quickly increases, and the disease begins to steadily progress, acquiring a widespread pathological process in many organs and systems. The death of such patients occurs within 1-2 years from the onset of the disease.

1. Plaque form - swelling, erythema, disturbances in skin pattern and pigmentation with a lilac-pink “corolla”, induration, fibrosis, atrophy.

2. Keloid form - dense strands resembling a keloid scar.

3. Linear form - damage along the neurovascular bundle, affecting fascia, muscles and bones; areas of lipodystrophy, amyotrophy, tendon shortening and impaired limb growth.

4. Deep subcutaneous nodular form - localized on the thighs and buttocks with involvement of the fascia. Isolated nodules along the tendons, resembling rheumatoid and transforming calcifications.

5. Atypical forms - idiopathic atrophoderma (bluish-violet spots on the body without previous compactions); Buschke's scleredema is a pseudoscleroderma disease (dense swelling of the dermis and subcutaneous tissue).

Diagnostics. Diagnosis based on clinical and laboratory data: moderate normo- or hypochromic anemia, moderate leukocytosis and eosinophilia and transient thrombocytopenia are usually observed. ESR is normal or moderately increased in chronic cases and significantly increased to 50-60 mm/hour in subacute cases.

Treatment. The principles of treatment are as follows.

1. Antifibrotic agents (D-penicillamine, diuciphone, colchicine, enzymatic drugs, dimethyl sulfoxide).

2. The use of NSAIDs.

3. Treatment with immunosuppressive drugs (suppress the autoimmune inflammatory process in connective tissue, inhibit excessive fibrosis formation); prescription of glucocorticoids.

4. The use of antihypertensive and microcirculation-improving agents.

5. Local therapy, massage, exercise therapy.

6. Symptomatic treatment for lesions of the digestive system.

7. Spa treatment.

3. Dermatomyositis

Dermatomyositis is a systemic disease of the skeletal, smooth muscles and skin.

Etiology. The etiology is unknown. A viral (Coxsackie B2) etiology of dermatomyositis is suggested. Provoking factors are insolation, cooling, trauma, pregnancy, drug intolerance, vaccination, and stressful situations.

Pathogenesis. Various immunopathological disorders.

Clinic. The disease begins with an acute or subacute course, with a muscle syndrome in the form of myasthenia gravis, myalgia, arthralgia also develops, fever appears, and when the skin is affected, the clinical picture is in the form of dense, widespread edema.

Skin lesions are varied, most often 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 at rest, manifested in the form of pain with pressure and increasing weakness. The muscles of the shoulder and pelvic girdle are compacted, increased in volume, active movements are significantly impaired, the patient cannot sit down independently, raise his head from the pillow and hold it while sitting or standing, or raise his limbs. With a large widespread process, patients are completely immobilized, and in severe cases they are in a state of complete prostration. When skeletal muscles are damaged, weakness, pain, swelling, muscle hardening, muscle wasting, and calcification appear. Visceral muscular syndrome - damage to symmetrical, mainly proximal, muscle groups, facial muscles leads to a mask-like appearance of the face, when the pharyngeal muscles are affected, dysphagia develops, and when the intercostal muscles and muscles of the diaphragm are affected, breathing is impaired and the vital capacity of the lungs decreases, hypoventilation. The oculomotor muscles may be affected with the development of diplopia, strabismus, bilateral ptosis of the eyelids, etc. Polyarthralgia during movement is manifested by limited joint mobility up to the development of ankylosis, mostly due to muscle damage. Myocardial damage of an inflammatory or dystrophic nature is observed, which is manifested by persistent tachycardia and pulse lability, expansion of the borders of the heart to the left, muffled heart sounds on auscultation, systolic murmur at the apex, arterial hypotension. Lung damage is associated with the underlying disease, most often it is caused by an infection, to which patients are predisposed due to pulmonary hypoventilation. The gastrointestinal tract is also involved in the process, anorexia, abdominal pain are noted, symptoms of gastroenterocolitis appear, and hypotension of the upper third of the esophagus. Damage to the peripheral and central nervous system.

Diagnostics. Criteria for the diagnosis of dermatomyositis are as follows.

1. Proximal muscle weakness for at least one month.

2. Myalgia within one month in the absence of sensitivity disorders.

3. The ratio of the concentration of creatine in the urine to the sum of the concentrations of creatine and creatinine in the urine, exceeding 40%.

4. A significant increase in the blood level of creatine phosphokinase or transaminases in the absence of creatine phosphokinase or transaminases in the absence of other causes.

5. Degenerative changes in muscle fibers during biopsy, 4 signs - a definite diagnosis, 3 signs - a probable diagnosis

diagnosis, 2 signs - a possible diagnosis.

Diagnosis: based on clinical and laboratory data, moderate leukocytosis with pronounced eosinophilia, a moderate increase in ESR, and hypergammaglobulinemia are usually observed.

The following studies are of great diagnostic importance: biochemical examination of blood and urine, muscle biopsy, especially in subacute and chronic cases (thickened muscle fibers with transverse striations, fragmentation and dystrophy up to necrosis are found, a significant cellular reaction appears in the form of accumulation of leukocytes, 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 (half a tablet 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 discontinued completely. In complex treatment, B vitamins and ascorbic acid are prescribed. In case of severe muscle fatigue, proserin and its analogues are prescribed in normal doses, ATP.

With early treatment with adequate doses of corticosteroids, patients with acute dermatomyositis experience sustained recovery. In subacute cases, remission can be achieved with a maintenance dose of glucocorticoids. In chronic dermatomyositis, the disease takes on a wave-like course.

Author: Pavlova N.V.

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