Use of Pulmonary Function Test Parameters to Identify Echocardiographic Pulmonary Hypertension in Interstitial Lung Disease
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At the beginning of this presentation, the pulmonary hypertension was classified into three groups as follows : 1) Pulmonary pulmonary hypertension 2) Pulmonary hypertension in congenital cardiovascular disease 3) Pulmonary hypertension due to the left heart disease This (3) pulmonary hypertension due to the left heart disorder corresponds to the passive pulmonary hypertension (Wood, P.) and is accompanied by the elevation of the pulmonary venous pressure. Pulmonary hypertension due to left to right shunt in congenital cardiovascular disease (2) is equivalent to the hyperkinetic pulmonary hypertension. And pulmonary pulmonary hypertension (1) is caused by the disease of the lung and/or thorax and has possibility to develop the pulmonary heart disease defined by WHO.
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AIM: To explore the pathophysiological significance of nitric oxide(NO) and nitric oxide synthase(NOS) in congenital heart defects with pulmonary hypertension. METHODS: Twenty-four patients with congenital heart defects were divided into 3 groups: mild pulmonary hypertension group(n=8), moderate or severe pulmonary hypertension group(n=9), and non-pulmonary hypertension group(n=7). NOS mRNA expression was detected in lung tissues with reverse transcriptase polymerase chain reaction (RT-PCR). RESULTS: The NOS mRNA expression levels in the patients with moderate or severe pulmonary hypertension were much lower than those in the ones with mild-pulmonary hypertension or non-pulmonary hypertension (P 0.05). There was an inverse correlation between the NOS mRNA expression level and the pulmonary arteriopathy in the patients with pulmonary hypertension (r=-0.833, P0.01). CONCLUSION: In patients with congenital heart defects associated with pulmonary hypertension, NO and NOS play a role in pathological process of pulmonary arteriopathy, and the NOS mRNA expression level was proportional to the severity of pulmonary hypertension or pulmonary arteriopathy.
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Increased post-marketing reports of interstitial lung disease in Japan have been recognized. An understanding of its regional groundings can be important for the global pharmacovigilance community. The objective of this study was to explore the correlation between high rates of interstitial lung disease reporting and regulatory actions in Japan. Post-marketing interstitial lung disease-related label changes and interstitial lung disease reports were classified by the anatomical therapeutic chemical classification groups of the suspected drugs. Regulatory actions for the top interstitial lung disease-reporting drugs were compared. The interstitial lung disease reporting patterns of protein kinase inhibitors were compared to those of methotrexate. Interstitial lung disease-related label changes predominantly occurred for drugs in the anatomical therapeutic chemical classification groups L, J, C, and herbal medicines. Interstitial lung disease was reported most frequently for L group, especially for the protein kinase inhibitors. The regulatory actions for those drugs with the highest number of interstitial lung disease reports (methotrexate, protease kinase inhibitors, gemcitabine, docetaxel) plus monoclonal antibodies were analyzed. The ratio of interstitial lung disease reports to all reports over time was initially high in the re-examination period, while it was constantly low after the period expired. The increase in interstitial lung disease reporting was observed for the drugs for which interstitial lung disease was designated as a priority item in the use-results survey. Methotrexate had more interstitial lung disease reports with multiple suspected drugs and fewer reports with high completeness than the protease kinase inhibitors. The high rates of interstitial lung disease reporting derived from mainly the anatomical therapeutic chemical classification group L drugs. Interstitial lung disease is the targeted adverse drug reaction in the use-results survey mandated in the re-examination of those drugs. This system provides at least one explanation for the high reporting of interstitial lung disease in Japan.
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The aim of this study was to identify risk factors of percent predicted forced vital capacity (ppFVC) decline in patients with SSc-associated interstitial lung disease (SSc-ILD).We identified 484 patients with SSc who had HRCT Chest, of which 312 with ILD. Those with serial pulmonary function tests were included in a longitudinal analysis (n = 184). Linear mixed effect models were fitted to assess the decline in ppFVC over time, and to explore the effect of demographics and baseline characteristics on ppFVC decline.The majority of SSc-ILD patients were female (76.3%) and 51.3% had diffuse cutaneous subset. The mean (s.d.) age was 53.6 (12.7) years, median disease duration since first non-RP symptoms was 2.6 years, and 48.4% of the patients had ILD extent >20% on HRCT. In the univariate analysis, longer disease duration (>2.37 years), ILD extent >20%, and anti-topoisomerase I (ATA) positivity were significantly associated with ppFVC decline. In the multivariate analysis, the only statistically significant variable associated with ppFVC decline was ATA positivity. The overall group's mean decline in ppFVC was -0.28% (P-value 0.029), with -0.13% (n = 163) in those who were alive and -8.28% (P-value 0.0002 for the change in ppFVC trajectory) in patients who died within 2 years.Our study confirms that ppFVC is a marker of survival in SSc-ILD, supporting its use for risk stratification to identify patients who may benefit from earlier interventions and treatment. Our study also supports the role of ATA positivity as a predictive marker for ppFVC decline in this population.
Univariate analysis
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Objectives. Interstitial lung disease (ILD) is a rare complication of juvenile dermatomyositis (JDM). The aim of this study was to clarify the clinical features of JDM‐associated ILD and to evaluate the efficacy of cyclosporin A (CSA).
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Clinical efficacy
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Interstitial lung disease occurs in the majority of patients with systemic sclerosis. Although interstitial lung disease is the number one cause of death in systemic sclerosis, interstitial lung disease progression rates vary considerably among patients with systemic sclerosis. Some patients with systemic sclerosis–associated interstitial lung disease have sub-clinical disease and may not derive benefit from immunosuppression, while others have a more aggressive interstitial lung disease phenotype. Reliable predictors of interstitial lung disease progression are lacking. The present review describes our current approach to monitoring systemic sclerosis–associated interstitial lung disease progression in clinical practice. To illustrate the marked heterogeneity that exists in interstitial lung disease progression rates in systemic sclerosis, this review presents the individual disease course of five unique patients with systemic sclerosis–associated interstitial lung disease who participated in the Scleroderma Lung Study II. These cases illustrate that treatment response rates vary in systemic sclerosis–associated interstitial lung disease and more research is needed to determine how to predict treatment response in systemic sclerosis–associated interstitial lung disease and to develop personalized treatment approaches for patients with this devastating disease.
Scleroderma (fungus)
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In Brief The association of pulmonary hypertension (PH) and interstitial lung disease (ILD) is well known. However, the etiology is likely multifactorial and the prevalence varied. When PH presents in a patient with ILD, it is associated with increased morbidity and mortality. Noninvasive diagnostic tools for PH in advanced lung disease have emerged but definitive diagnosis requires right heart catheterization. No guidelines currently exist for therapy in this secondary form of PH. Small case series and studies have shown mixed results in the use of therapy for Group I pulmonary arterial hypertension in secondary PH. Further studies are needed to define the role of these therapies in PH of ILD. Pulmonary hypertension is commonly found in association with interstitial lung disease. This finding heralds increased mortality and increased symptoms but also presents potential targets for therapy.
Etiology
Right heart catheterization
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