Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis.
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.
Yaqub
Sheraz
S
Division of Rheumatology and Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minn 55905, USA.
Moder
Kevin G
KG
Lacy
Martha Q
MQ
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