Document Detail

Severe pulmonary hypertension in pediatric primary Sjögren syndrome: a case report.
MedLine Citation:
PMID:  17921797     Owner:  NLM     Status:  MEDLINE    
A 9-year-old girl with a history of xerostomia and recurrent bilateral parotid gland enlargement presented with purpura of the lower limbs and exertional dyspnea. She had hyperglobulinemia, positive ANA, SSA, and SSB and an abnormal Shirmer test leading to a diagnosis of primary Sjögren syndrome. Cardiologic and imaging investigations revealed severe isolated pulmonary hypertension and ruled out pulmonary fibrosis. Prednisolone and cyclophosphamide, together with anticoagulant and vasodilatory drugs therapy, induced a significant improvement of exertional dyspnea and lowered pulmonary artery pressure. This case and reports from the literature suggest that immune mechanisms, not just vasospasm, can be factors in some pulmonary hypertension.
Xuan Zhang; Xiaofeng Zeng
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases     Volume:  13     ISSN:  1076-1608     ISO Abbreviation:  J Clin Rheumatol     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-10-08     Completed Date:  2008-01-08     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  9518034     Medline TA:  J Clin Rheumatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  276-7     Citation Subset:  IM    
Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
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MeSH Terms
Antirheumatic Agents / therapeutic use
Cyclophosphamide / therapeutic use
Hypertension, Pulmonary / drug therapy,  etiology*,  immunology
Prednisolone / therapeutic use
Sjogren's Syndrome / complications*,  diagnosis,  drug therapy
Reg. No./Substance:
0/Antirheumatic Agents; 50-18-0/Cyclophosphamide; 50-24-8/Prednisolone

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