| Severe pulmonary hypertension in pediatric primary Sjögren syndrome: a case report. | |
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MedLine Citation:
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PMID: 17921797 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Xuan Zhang; Xiaofeng Zeng |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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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:
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Created Date: 2007-10-08 Completed Date: 2008-01-08 Revised Date: 2009-11-03 |
Medline Journal Info:
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Nlm Unique ID: 9518034 Medline TA: J Clin Rheumatol Country: United States |
Other Details:
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Languages: eng Pagination: 276-7 Citation Subset: IM |
Affiliation:
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Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Antirheumatic Agents
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therapeutic use Child Cyclophosphamide / therapeutic use Female Humans Hypertension, Pulmonary / drug therapy, etiology*, immunology Prednisolone / therapeutic use Sjogren's Syndrome / complications*, diagnosis, drug therapy |
| Chemical | |
Reg. No./Substance:
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0/Antirheumatic Agents; 50-18-0/Cyclophosphamide; 50-24-8/Prednisolone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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