scholarly journals Diagnosis and management of connective tissue disease‐associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand*

Respirology ◽  
2020 ◽  
Vol 26 (1) ◽  
pp. 23-51
Author(s):  
Adelle S. Jee ◽  
Robert Sheehy ◽  
Peter Hopkins ◽  
Tamera J. Corte ◽  
Christopher Grainge ◽  
...  
Keyword(s):  
New Zealand ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Position Statement ◽  
Diagnosis And Management

scholarly journals Lung cancer development in patients with connective tissue disease–related interstitial lung disease

Medicine ◽  
2016 ◽  
Vol 95 (50) ◽  
pp. e5716 ◽  
Author(s):  
Yasunori Enomoto ◽  
Naoki Inui ◽  
Katsuhiro Yoshimura ◽  
Koji Nishimoto ◽  
Kazutaka Mori ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Cancer Development

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.

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