Differential diagnosis of diffuse connective tissue diseases Tactics

Differential diagnosis of diffuse connective tissue diseases. Tactics GPs. Lecturer: Assoc. Akhmedov H. S.

diffuse connective tissue diseases (DCTD) Autoimmune precces Lack of mono etiological factor Polysyndromic flow The similarity of the pathological changes Loss (signs of inflammation) of blood vessels БТДК articular syndrome The effectiveness of glucocorticoids, cytotoxic drugs and NSAIDs Multifactorial predisposition to a certain type of role immunogenetic factors related to the sixth chromosome

SPECIFIC IMMUNE RESPONSE В- site ho p lym elimination AG A B IC С A B AG Тlymphocyt e С

AUTOIMMUNE PROCCES В- cyte o ph m ly elimination AG A B IC Immunoinflammatory process connective tissue Тlymphocyt e С

DCTD List 1. SLE - idiopathic; drug lupus syndrome 2. SSD - idiopathic; induced (chemical, pharmaceutical) 3. Diffuse eosinophilic fastsiit 4. Dermatomyositis (polymyositis) - idiopathic, paraneoplastic, juvenile 5. Sjogren's syndrome - primary (Sjogren's disease), secondary. 6. Cross 7. syndrome. Relapsing polychondritis relapsing panniculitis (Weber-Christian disease). 8. Antiphospholipid syndrome

SYSTEMIC LUPUS ERYTHEMATOSUS The disease occurs predominantly in women (90%), middle-aged (30 -40 years).

Leading clinical features that are crucial in the diagnosis of SLE: derm atiti s Articular syndrome sero (poly sitis sero sitis) ØLupus "butterfly" Ødiscoid lupus ØRaynaud's syndrome Øalopecia Øphotosensitization Østomatitis ØNephritis, cardio, ØPsychosis and (or) seizures seizure ØLE-cell test, false-positive test Wasserman Øsyndrome thrombocytopenic purpura, hemolytic anemia, and (or) leukopenia and (or) thrombocytopenia. Øprolonged fever ØAccelerated erythrocyte sedimentation rate, hypergammaglobulinemia, RF pleurisy pericarditis The defeat of the peritoneum

Features articular syndrome in SLE Migratory arthralgia and arthritis Symmetrical joint disease Morning stiffness (less than 30 minutes) Contracture (due to the destruction of muscles or ligaments) Articular syndrome Often in combination with myalgia, myositis, and ossalgiya Predominant involvement of small and medium-sized joints, and sometimes large Deformation and ankylosis are not observed

Diagnostic criteria articular syndrome in systemic lupus erythematosus (ARA) erythema on the face - "Butterfly"; discoid lupus; Raynaud's syndrome, alopecia, photosensitivity, ulceration in the mouth, nose and throat, arthritis without deformation; LE-cells, false-positive Wasserman, proteinuria more than 3. 5 g per day; cilindruria, pleurisy, pericarditis, psychosis, convulsions, hemolytic anemia, and (or) leukocytosis and (or) thrombocytopenia.

Factors associated with poor prognosis include: kidney disease (especially diffuse proliferative glomerulonephritis), hypertension, male gender, onset before the age of 20 years; antiphospholipid syndrome; high disease activity; severe internal organ; accession of infection, complications of drug therapy.

SYSTEMIC SCLERODERMIA Prevalence - 12 per 1 million population, mostly women.

Leading clinical features that are crucial in the diagnosis of SSD: Skin defe a t • "Solid" edema • induration • atrophy • pigmentation • "Mask-like" face Articular syndrome Ray phe naud's nom eno n • sclerodactyly • CREST- syndrome • Anemia and (or) leukopenia, thrombocytopenia • Osteolysis of the terminal phalanges of hands and feet • The defeat of the internal organs (esophagitis with dysphagia, basal pulmonary fibrosis, macrofocal cardio, true scleroderma kidney) • Peripheral hyperpigmentation, trophic changes • Lymphadenopathy, polyserositis, polyneuritis • Increased erythrocyte sedimentation rate, hyperproteinemia, hypergammaglobulinemia, RF

Diagnosis of SSc criteria (American Associations for Rheumatology): 1. Main • scleroderma skin lesions proximal to the metacarpophalangeal or metatarsophalangeal joints 2. Minor criteria: • sclerodactyly ("bird's foot" because of flexion contracture); • scars on the distal phalanges; • bilateral basal pulmonary fibrosis.

CLASSIFICATION OF DERMATOMYOSITIS A. Origin: idiopathic, paraneoplastic B. Current: acute, subacute, chronic B. Periods: prodromal, symptomatic, dystrophic G. The extent of activity: 1, 2, 3 D. The main clinical signs (syndromes)

Leading clinical features that are crucial in the diagnosis of diabetes: Skin defe a t • "Typical skin changes - periorbital edema and erythema (symptom score) • telangiectasia muscle syndrome • Progressive weakness, myalgia, edema and further muscle atrophy (proximal parts of the limbs) Changes in the blood The increase in performance: creatine phosphokinase, aldolase transaminase

Diagnostic criteria for dermatomyositis (ARA) The main characteristic skin lesions: periorbital edema and erythema (symptom of "points"); telangiectasia, erythema on the open areas of the body (face, neck, upper chest, limbs). affected muscles (mainly proximal extremities), which is expressed in muscle weakness, myalgia, edema, and later - atrophy. Patomorphology characteristic muscle biopsy (degeneration necrosis, basophils, inflammatory infiltrates, fibrosis). Increase of serum enzymes - creatine kinase, aldolase, transaminases 50% or more compared to the norm. Typical data electromyographic studies. Additional calcification. Dysphagia.

Diffuse eosinophilic fasciitis- It is a systemic disease of the connective tissue with a primary infiltrative lesions of fibrous fascia limbs, accompanied by dense sclerodermic swelling of the skin, flexion contracture, eosinophilia and hyper gamma globulinaemia

Diagnostic criteria DEF acute onset with seal tissues in the forearms and shins, limitation of movement, flexion contractures, skin lesions by type "orange peel", eosinophilia in the peripheral blood cell infiltration fascia with a predominance of macrophages and lymphocytes and eosinophils admixture, the positive effect of GCS favorable prognosis

Mixed connective tissue disease clinical and immunological syndrome systemic connective tissue disorders, manifested by a combination of individual clinical signs of SSc, polymyositis, SLE and the presence of in the blood of patients with antibodies to ribonucleoprotein

get sick more often women. Patients of complainpain in the joints - polyarth-, muscle weakness, muscle compression, increase in blood creatine kinase. The skin telangiectasia, erythematous and hypo- or hyperpigmented spots, discoid lupus, alopecia, etc. . The lesions of the internalorgans: myocarditis, pericarditis, aortic insufficiency, enlargement of the liver, spleen, kidney-type glomerulonephritis. In the blood, signs of anemia, leukopenia, increased erythrocyte sedimentation rate. The main thing is biopsy - a picture of myositis and necrosis of muscle

Sjogren disease This is a systemic autoimmune disease characterized by chronic inflammation of the exocrine glands that secrete predominantly Ig A, especially the salivary and lacrimal, with the gradual development of secretory failure in combination with various systemic manifestations.

The clinical picture consists of: 1. The lesions of the salivary glands, cornea, conjunctiva, xerophthalmia as a dry ceratoconyuktivita that appears foreign body sensation in the eye, photophobia, burning sensation in the eyes, redness of eyes, lack of tears, erosion and corneal opacity. 2. The defeat of the salivary glands - mumps with decreased secretion of saliva, dry mouth (xerostomia). Parotid, submandibular gland is enlarged, vayutsya, little saliva, it is thick, develops cheilitis, glossitis, dental caries, stomatitis. 3. Defeat other exocrine glands with decreased function, as manifested by dryness of the skin, puts-conductive, esophagus, nose, throat, etc. 4. polyarth- or the development of arthritis by type RA 5. Systemic manifestations: fever, limfoadeno-Patiala, vasculitis, myositis, liver enlargement, village-Zenk, kidney disease, Raynaud's syndrome

DCTD APPLY TO 2 ND CATEGORY SERVICES GPs in conjunction with a dermatologist should Achieve clinical and laboratory remission. Prevent injury to vital organs and systems in the first place - the kidneys and central nervous system.

THIS IS ACHIEVED suppression of immune inflammation and immunocomplex pathology; prevention of complications of immunosuppressive therapy; Direct impact on the pathological manifestations treatment of complications arising during the course of immunosuppressive therapy; the impact on the individual, pronounced syndromes; removing from the body of circulating immune complexes and antibodies.

Suppression of immune inflammation and immunocomplex pathology 1. 2. glucocorticoids cytostatics Directly affect the pathological manifestations (DMARDs) 1. 2. 3. antifibrotic therapy aminohinolinovogo drugs Inhibitors of TNF alpha

WITH THE DEVELOPMENT OF COMPLICATIONS APPOINT: Antibacterials (with intercurrent infection); TB drugs (with the development of tuberculosis, often pulmonary localization); Formulations of insulin diet (at development of diabetes); Antifungal agents (candidiasis); The course of anti-ulcer therapy (when a "steroid" ulcers).

NSAID’s basic drugs Phase mucoid swelling Phase fibrinoid swelling granulation phase fibrosis

CYTOSTATICS В- cyte ho p lym AG A B IC Тlymphocyt e С

EDUCATION OF THE PATIENT The patient must be advised of the need to long (lifelong) treatment, as well as directly dependent on the accuracy of the results of treatment compliance recommendations. Should explain the negative effect of sunlight on the course of the disease (acute provocation) The importance of contraception and pregnancy planning under medical supervision in view of disease activity and functional state of vital organs. Patients should be aware of the need for regular monitoring of clinical and laboratory manifestations and know the side effects of drugs used.

MEDICAL REHABILITATION AT DCTD Indications for physical rehabilitation and spa treatment at DCTD: mainly peripheral manifestations of the disease; chronic or subacute with active pathological process is not higher than I degree; functional failure of the musculoskeletal system are not above II degree.

CONTRAINDICATIONS TO THE PHYSIO-FUNCTIONAL AND SANATORIUM TREATMENT AT DCTD: predominantly visceral forms of systemic connective tissue diseases; The high activity of (II and IIIstepeni) expressed functional disorders of the musculoskeletal system with the loss of self-care and independent movement; treatment with high doses of corticosteroids (more than 15 mg of prednisone per day) or receiving cytotoxic drugs.

INDICATIONS FOR HOSPITALIZATION A Fever. Signs of diffuse CNS. Hemolytic crisis. Active forms. Severe comorbidities (pulmonary hemorrhage, myocardial infarction, gastrointestinal bleeding, and others. ).

Keep in mind! If the patient is observed • Articular syndrome (especially visceral); • Prolonged fever of unknown origin; • serozity; • Rash manifestations of vasculitis; • weight loss; • Raynaud's phenomenon; • The appearance of seals Should be deleted DCTD

PREVENTION primary prevention identification of persons threatened by this disease (mainly relatives of patients). Upon detection of them even one of the symptoms - persistent leukopenia, antibodies to DNA, increasing the ESR, hypergammaglobulinemia, or other signs of pre-disease - should warn them against excessive sun exposure, hypothermia, vaccinations, physiotherapy (eg, ultraviolet radiation, mud). Particular attention should be given to patients with discoid lupus. To prevent the generalization of the pathological process, such patients should not receive ultraviolet irradiation, treatment with gold, spa treatment.

secondary prevention • a thorough clinical examination; • permanent and long-term daily use of hormonal drugs in maintenance doses • Glucocorticosteroids and aminohinolinovogo drugs can be canceled only upon the occurrence of complete remission; • mode patient should be protective, lightweight, but, if possible tempering (morning gymnastics, tiring exercise and workout, wiping with warm water, long walks in the fresh air). • readjustment of foci of infection

tertiary prevention social and vocational rehabilitation psychological adjustment