Document Detail

Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis.
MedLine Citation:
PMID:  15132415     Owner:  NLM     Status:  MEDLINE    
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.
Sheraz Yaqub; Kevin G Moder; Martha Q Lacy
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Mayo Clinic proceedings     Volume:  79     ISSN:  0025-6196     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  2004 May 
Date Detail:
Created Date:  2004-05-10     Completed Date:  2004-05-28     Revised Date:  2013-12-13    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  687-9     Citation Subset:  AIM; IM    
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MeSH Terms
Dermatomyositis / complications*,  diagnosis,  therapy
Hypertension, Pulmonary / complications*,  diagnosis,  therapy
Paraproteinemias / complications*,  diagnosis,  therapy
Severity of Illness Index

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