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Differential diagnosis of systemic connective tissue diseases in Tashkent

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Differential diagnosis of systemic connective tissue diseases in Tashkent

Differential diagnosis of systemic connective tissue diseases in Tashkent

Diagnostics

- Analysis for antinuclear antibodies (ANA) to extractable nuclear antigen, antibodies to Smith antigen (Sm) and antibodies to DNA

- Organ pathology is determined in accordance with clinical signs

MCTD should be suspected in all patients with SLE, systemic scleroderma, or polymyositis who develop additional clinical overlap.

First of all, it is necessary to determine ANA, antibodies to U1 ribonucleoprotein. Almost all patients have high titers of mottled ANA. Antibodies to U1 ribonucleoprotein are usually present in very high titer. Antibodies to the ribonuclease-resistant component of the extractable nuclear antigen (CM antigen) and double-stranded DNA (negative in CCTD) are measured to rule out other diseases.

Rheumatoid factor (RF) is often detected, the titers of which should be high. ESR levels are often elevated.

Pulmonary hypertension should be detected as early as possible by pulmonary function test and echocardiography. The plan for further research depends on the existing symptoms of damage to organs and systems: the presence of signs of myositis, damage to the kidneys and lungs requires appropriate diagnostic methods (in particular, creatine kinase, MRI, electromyography, muscle biopsy).

Forecast

In general, ten-year survival is about 80%, but the prognosis largely depends on the prevailing symptoms. It is worse for patients with signs of systemic scleroderma and polymyositis. Patients have an increased risk of atherosclerosis. The main causes of death are pulmonary hypertension, renal failure, myocardial infarction, colon perforation, disseminated infections, and cerebral hemorrhage. Some patients remain in remission for many years without treatment.

Treatment
- NSAIDs and aminoquinoline drugs for mild disease

- Corticosteroids and other immunosuppressants (eg, methotrexate, azathioprine, mycophenolate mofetil) for moderate to severe disease

- Calcium channel blockers (eg nifedipine) and phosphodiesterase inhibitors (eg tadalafil) for Raynaud's phenomenon

The initial and maintenance treatment for patients with CTD is chosen according to the clinical picture; it may be similar to treatment for SLE or depend on the dominant clinical phenotype.

All patients should be monitored for signs of atherosclerosis. During long-term treatment with glucocorticoids, prevention of osteoporosis should be carried out.
Some experts recommend periodic screening for pulmonary hypertension with lung function and/or echocardiography every 1 to 2 years, depending on symptoms.

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