Snapshot A 31-year-old woman presents to her primary care physician's office with general malaise, myalgias, and low-grade fever. She notices that during the winter months the tips of her fingers turn blue and then returns to her baseline color when in a warm environment. On physical exam, there is a malar rash, sclerodactyly, and joint tenderness. Laboratory testing is positive for anti-nuclear antibodies (ANA) and anti-U1 RNP antibodies. Introduction Clinical definition generalized connective tissue disorder with clinical features of systemic lupus erythematosus, systemic sclerodermia, and polymyositis login to view 1 more bullet Epidemiology demographics login to view 2 more bullets Pathogenesis autoimmune process Prognosis generally a favorable outcome Presentation Symptoms Raynaud phenomenon arthralgia arthritis myositis sclerodactyly hand swelling Imaging Echocardiography indication login to view 1 more bullet Studies Labs positive for anti-U1-RNP antibodies Diagnostic criteria clinical diagnosis supported by anti-U1-RNP antibodies on serology Differential Systemic lupus erythematosus (SLE) Scleroderma Polymyositis Rheumatoid arthritis Idiopathic pulmonary arterial hypertension Treatment Management approach treatment is aimed at controlling symptoms and is targeted to organ involvement Medical prednisone login to view 3 more bullets calcium channel blockers login to view 3 more bullets Complications The major cause of death is pulmonary hypertension in MCTD