Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension

被引：0

作者：

Johnson, Sindhu R.

Patsios, Demetris

Hwang, David M.

Granton, John T.

机构：

[1] Univ Toronto, Div Rheumatol, Dept Pathol, Toronto, ON, Canada

[2] Univ Toronto, Dept Med Imaging, Toronto, ON, Canada

[3] Univ Toronto, Pulm Hypertens Program, Div Respirol, Toronto, ON, Canada

来源：

JOURNAL OF RHEUMATOLOGY | 2006年 / 33卷 / 11期

关键词：

pulmonary veno-occlusive disease; pulmonary arterial hypertension; scleroderma; systemic sclerosis; endothelin receptor antagonist; SYSTEMIC-SCLEROSIS; AUTOANTIBODIES; PROSTACYCLIN; FEATURES; PATIENT;

D O I：

暂无

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presurned PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect.

引用

页码：2347 / 2350

页数：4

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