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Introduction. Mixed connective tissue disease (MCTD) is a rare autoimmune disorder that has 5 other
connective tissue diseases: systemic lupus erythematosus, systemic sclerosis, polymyositis,
dermatomyositis and rheumatoid arthritis. A sixth - Sjögren's syndrome, is commonly associated with each
of these diseases. Current clinical records often note that one autoimmune rheumatic disease seems to
evolve into another over the course of several years and this occurs in approximately 25 percent of patients.
Case presentation. We report a case of a female patient A, 43 y.o., she is complains started with the
insidious onset manifested by inflammatory joint syndrome of bilateral radiocarpal, talocrural, synovitis of
knee joints and decreased muscle strength, morning stiffness > 30 minutes with relief from exercise and
movement, swelling of the proximal and distal metacarpophalangeal joints, skin hyperpigmentation on the
trunk, heart palpitations, positive Shirmer test, weight loss 10 kg, severe fatigue by VAS - 82/100 mm.
Discussion. Radiography features showed erosions and deforming like in rheumatoid arthritis. Echo
showed moderate pulmonary hypertension, mitral and tricuspid valve regurgitation stage II. Serological
examination showed (anti-CCP, ScL-70, dsDNA -negative, C3, C4- norma, rheumatoid factor, anti-RNP
are positive. A diagnosis of MCTD was made, based on the criteria of Alarcón-Segovia and Villareal;
patients met both clinical and serological criteria, with Anti-RNP values in high titers.
Conclusion. MCTD is a well-defined entity using well-defined criteria. The complications of the disorder
have already been described, most deaths from MCTD are due to heart failure caused by pulmonary arterial
hypertension and ILD because it is important to note that both comorbidities worsen patient prognosis. The
medications and dosage will depend on the severity of activity and damage disease. |
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