Mixed Connective Tissue Disease Flashcards
What is the autoantibody affiliated with mixed connective tissue disease?
Anti-U1 RNP
What is the gender distribution in patients with mixed connective tissue disease?
Females account for 80% of cases.
How do patients with mixed connective tissue disease typically present (6 findings)?
They typically present with Raynaud phenomenon, fever, arthritis, dorsal hand edema, rash, and myositis. Joint abnormalities are seen in 60-90% of children.
What is the main cause of mortality in mixed connective tissue disease?
Mortality most often occurs due to chronic interstitial lung disease or pulmonary HTN.
How does mixed connective tissue usually progress over time?
Over time, MCTD progresses like scleroderma, and many patients develop restrictive lung disease.
What are the typical GI manifestations of mixed connective tissue disease?
Esophageal disease, especially dysphagia and abnormal esophageal function, is the most typical of the GI manifestations.
What are the most common cardiac findings in mixed connective tissue disease?
Acute pericarditis with pericardial effusion and mitral valve prolapse.
Describe the association between mixed connective tissue disease and renal disease.
Renal disease occurs in about 25% of pediatric patients, and the nephritis can be membranous, membranoproliferative, or mesangioproliferative.
What are some (2) less common causes of mortality in patients with mixed connective tissue disease?
Severe thrombocytopenia and infectious complications of immunosuppression.
Compare the prognosis in patients with mixed connective tissue disease with that of patients with SLE or scleroderma.
Patients with MCTD generally have a better prognosis than for patients with SLE or scleroderma.
What (4) lab findings would one expect in a patient diagnosed with mixed connective tissue disease?
High-titer speckled ANA, anti-U1 RNP antibodies, RF, and hypergammaglobulinemia.
What is the typical treatment regimen in patients diagnosed with mixed connective tissue disease?
Antimalarials are first-line. In patients with severe disease, corticosteroids may be used as well.
