title: Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis.
abstract: 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 5 years after treatment was discontinued.
authors.affiliation: Division of Rheumatology and Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minn 55905, USA.
authors.forename: Sheraz
authors.initials: S
authors.lastname: Yaqub
authors.forename: Kevin G
authors.initials: KG
authors.lastname: Moder
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
mesh_terms: D000328:Adult; D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012720:Severity of Illness Index