Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome

Ohashi, Kazuyo; Nishimura, Rintaro; Sugimoto, Shunsuke; Sakao, Seiichiro; Tanabe, Nobuhiro

doi:10.1002/rcr2.411

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…Besides, it's been reported that thalidomide can reduce the expression of IL-18 and TNF-α in the alveolar lavage fluid in patients with IPF (41). It can be seen that thalidomide potential provides a good antipulmonary fibrosis effect (42,43). However, the underlying mechanism remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Thalidomide inhibits the gene promoter of connective tissue growth factor in human embryonic lung fibroblasts

Wu¹,

Liu²,

Zhang³

et al. 2020

Ann Palliat Med

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Background: The etiology and pathogenesis of idiopathic pulmonary fibrosis (IPF) remain unclear, and the early detection and treatment are vital to the prognosis of IPF patients. It's necessary to investigate the effect of thalidomide on the gene promoter activation of connective tissue growth factor (CTGF) induced by transforming growth factor-β1 (TGF-β1) in human embryonic lung fibroblast (HELF). Methods:The gene vector of pGL3-CTGFP containing human CTGF gene promoter was constructed and transfected into HELF cells. We used different TGF-β1 (0, 2.5, 5, 10 and 20 µg/L respectively) to stimulate HELF to identify the optimal concentration for gene promoter of CTGF. The activity of luciferase was measured to observe the effect of TGF-β1 and THALIDOMIDE on the activity of CTGF gene promoter. Results:The relative luciferase activity increased significantly with the stimulation of TGF-β1, and the relative luciferase activity peaked in the 5 µg/L TGF-β1 group (all P<0.01). The thalidomide inhibited the TGF-β1-induced activation of CTGF gene promoter in HELF, and the effect peaked in the 25 µg/L group (all P<0.001).Conclusions: Thalidomide produces a significant inhibitory effect on the gene promoter activation of CTGF induced by TGF-β1 in HELF, it may be a potentially effective drug for the treatment of pulmonary fibrosis.

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Section: Discussionmentioning

confidence: 99%

Thalidomide inhibits the gene promoter of connective tissue growth factor in human embryonic lung fibroblasts

Wu¹,

Liu²,

Zhang³

et al. 2020

Ann Palliat Med

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Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases

Huang,

Luo,

Hou

et al. 2023

BMC Pulm Med

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Background Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic clonal plasma cell disorder. Pulmonary involvement is frequently found in patients with POEMS syndrome, manifesting various clinical features. Therefore, to improve diagnostic accuracy and provide treatment strategies, a comprehensive analysis of pulmonary manifestations of POEMS syndrome is needed. Methods This retrospective study included patients with POEMS syndrome at Peking Union Medical College Hospital, a major referral medical center in China, between June 1, 2013, and June 1, 2023. Demographic data, laboratory findings, pulmonary function test results, echocardiograms, and chest imaging data were extracted. Continuous variables were compared using the t-test or Mann–Whitney method. Pearson’s chi-square test or Fisher’s exact test was conducted to compare categorical data. Results Overall, 282 individuals diagnosed with POEMS syndrome were included in this study, of which 56% were male with an average age of 48.7 years. Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). Chest computed tomography and echocardiography findings showed that 56.4% of patients exhibited pleural effusion, 62.8% displayed mediastinal or hilar lymphadenopathy, 46.5% presented pleural thickening, 27.3% demonstrated bone lesions of the ribs or thoracic vertebra, 7.8% showed lung interstitial abnormalities, and 35.5% had pulmonary hypertension. Decreased diffuse capacity and restrictive ventilatory patterns were identified in 85.2% (115 cases) and 47.4% (64 cases) of patients, respectively. Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities. Conclusion Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem.

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Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome

Cited by 2 publications

References 5 publications

Thalidomide inhibits the gene promoter of connective tissue growth factor in human embryonic lung fibroblasts

Thalidomide inhibits the gene promoter of connective tissue growth factor in human embryonic lung fibroblasts

Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases

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