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LECTURE № 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 (Shekleyn — Genokh);

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

3) Takayasu arteritis.

7. Dermatomyositis.

8. Scleroderma.

9. Difficultly classified rheumatic syndromes.

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

1) benign rheumatoid nodules;

2) erythema nodosa;

3) Lyme disease;

4) sarcoidosis;

5) Stephen-Johnson syndrome;

6) Goodpasture syndrome;

7) symptoms of fibrositis and fibromyalgia;

8) Behcet syndrome;

9) Sjogren's syndrome.

11. Non-rheumatic diseases similar to rheumatic in the clinic.

Diffuse diseases of the connective tissue (DBST, collagenosis) is an immunopathological lesion of the connective tissue with systemic damage to the vessels and various organs, with a progressive course.

In the etiology of DBST, there are 3 leading factors.

The 1st factor is a genetic predisposition, as evidenced by epidemiological studies, the disease of close relatives, twins and the detection of markers of certain diseases in the HLA system.

The 2nd factor is the trigger mechanism, which is represented by viruses containing RNA and slow-reacting (retroviruses), and chronic persistent infection transmitted transplacentally.

The third factor is resolving: stressful situation, hyperinsolation, hypothermia.

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

In the DBST group, 4 “large collagen diseases” are distinguished.

1. Systemic lupus erythematosus (SLE).

2. Systemic scleroderma (SSD).

3. Progressive sclerosis.

4. Dermatomyositis (DM).

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

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