Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis

Mayo Clin Proc. 2004 May;79(5):687-9. doi: 10.4065/79.5.687.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Dermatomyositis / complications*
  • Dermatomyositis / diagnosis
  • Dermatomyositis / therapy
  • Humans
  • Hypertension, Pulmonary / complications*
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / therapy
  • Male
  • Paraproteinemias / complications*
  • Paraproteinemias / diagnosis
  • Paraproteinemias / therapy
  • Severity of Illness Index