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 along with high levels of anti-U1-RNP antibodies Epidemiology demographics more common in women 15-25 years of age but can occur at any age Pathogenesis autoimmune process Prognosis generally a favorable outcome Presentation Symptoms Raynaud phenomenon arthralgia arthritis myositis sclerodactyly hand swelling Imaging Echocardiography indication for early diagnosis of pulmonary arterial hypertension and for the evaluation of valvular disease 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 indications responsive in patients presenting with symptoms consistent with SLE in patients with fatigue, myositis, myalgias, arthralgias, pleuritis, pericarditis, autoimmune anemia, and thrombocytopenia calcium channel blockers indications pulmonary arterial hypertension Raynaud phenomenon Complications The major cause of death is pulmonary hypertension in MCTD