Connective Tissue Disease Associated Pulmonary Hypertension

2008 ◽  
pp. 145-171
Author(s):  
Kimberly A. Fisher ◽  
Nicholas S. Hill ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease

scholarly journals Association between lung ultrasound B‐lines and exercise‐induced pulmonary hypertension in patients with connective tissue disease

2021 ◽  
Author(s):  
Kazuki Kagami ◽  
Tomonari Harada ◽  
Koichi Yamaguchi ◽  
Shunichi Kouno ◽  
Takahiro Ikoma ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Ultrasound ◽  
Exercise Induced ◽  
B Lines

Classification tree criteria of pulmonary hypertension in mixed connective tissue disease

1997 ◽  
Vol 7 (4) ◽  
pp. 293-303
Author(s):  
Lisa Kotajima ◽  
Shinichi Aotsuka ◽  
Tomoe Nishimaki ◽  
Heihachiro Kashiwagi ◽  
Takeyoshi Kunieda ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Classification Tree

scholarly journals Fatal Pulmonary Hypertension in a Patient with Mixed Connective Tissue Disease: Report of an Autopsy Case.

1992 ◽  
Vol 31 (1) ◽  
pp. 74-77 ◽  
Author(s):  
Makoto SUZUKI ◽  
Mareomi HAMADA ◽  
Michihito SEKIYA ◽  
Yuji SHIGEMATSU ◽  
Soei GO ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Autopsy Case

scholarly journals Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

2010 ◽  
Vol 17 (6) ◽  
pp. 282-286 ◽  
Author(s):  
Shikha Mittoo ◽  
Thomas Jacob ◽  
Andrea Craig ◽  
Zoheir Bshouty
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Term Treatment ◽  
Kaplan Meier ◽  
Long Term Treatment ◽  
Survival Differences

BACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

Pulmonary hypertension in connective tissue disease. Clinical analysis of sixty patients in multi-institutional study

1990 ◽  
Vol 9 (1) ◽  
pp. 56-62 ◽  
Author(s):  
R. Kasukawa ◽  
T. Nishimaki ◽  
T. Takagi ◽  
S. Miyawaki ◽  
R. Yokohari ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Analysis

scholarly journals Social deprivation in Scottish populations with pulmonary hypertension secondary to connective tissue disease and chronic thromboembolic disease

2020 ◽  
Vol 6 (4) ◽  
pp. 00297-2019 ◽  
Author(s):  
Michael McGettrick ◽  
Paul McCaughey ◽  
Alexander MacLellan ◽  
Melanie Brewis ◽  
A. Colin Church ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Proportional Hazards ◽  
Social Deprivation ◽  
Population Distribution ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Cox Proportional Hazards ◽  
Functional Class ◽  
The Usa

Socioeconomic factors have been shown to have an adverse impact on survival in some respiratory diseases. Studies from the USA and China have suggested worse survival in idiopathic pulmonary arterial hypertension in low socioeconomic groups. We looked at the effect of deprivation on the outcomes in patients with connective tissue disease-associated pulmonary hypertension (CTDPH) and chronic thromboembolic pulmonary hypertension (CTEPH) in a retrospective observational study.Data were obtained from 232 patients with CTDPH and 263 with CTEPH who were under the care of the Scottish Pulmonary Vascular Unit, Glasgow, UK. We used Cox proportional hazards regression to assess for a relationship between deprivation and survival.We found no difference in survival across deprivation quintiles in the CTDPH (p=0.26) or CTEPH cohorts (p=0.18). We constructed multivariate models using enrolment time, age, sex and body mass index, with no significant change in findings. There was no difference between expected and observed population distribution of CTDPH (p=0.98) and CTEPH (p=0.36). Whilst there was no difference in presenting functional class in the CTDPH group, the CTEPH patients in more deprived quintiles presented in a worse functional class (p=0.032). There was no difference between quintiles of CTEPH patients who had distal or proximal disease (p=0.75), or who underwent surgery (p=0.5).Increased social deprivation is not associated with worse survival in patients with CTDPH and CTEPH managed in the Scottish National Health Service. Whilst there is no evidence of referral barriers in CTDPH, this may not be the case in CTEPH, as lower deprivation was associated with worse functional class at presentation.

Sign in / Sign up

Export Citation Format

Share Document