Severe, Reversible Pulmonary Hypertension in a Patient With Monoclonal Gammopathy and Features of Dermatomyositis

Yaqub, Sheraz; Moder, Kevin G.; Lacy, Martha Q.
May 2004
Mayo Clinic Proceedings;May2004, Vol. 79 Issue 5, p687
Academic Journal
Pulmonary hypertension is an extremely serious and potentially fatal disorder. Although pulmonary hypertension is a potential complication of connective tissue disease, it has been reported rarely in patients with dermatomyositis. Similarly, multiple myeloma is rare in patients with dermatomyositis. We describe a patient with severe pulmonary hypertension who also had features of dermatomyositis and monoclonal gammopathy. To our knowledge, this is the first reported case of a patient in whom all 3 disorders occurred concurrently. Even more striking is the fact that the patient responded to treatment with cyclophosphamide and prostacyclin. He is asymptomatic more than S years after treatment was discontinued.


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