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

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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.

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Yaqub, S., Moder, K. G., & Lacy, M. Q. (2004). Severe, Reversible Pulmonary Hypertension in a Patient with Monoclonal Gammopathy and Features of Dermatomyositis. Mayo Clinic Proceedings, 79(5), 687–689. https://doi.org/10.4065/79.5.687

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