Pulmonary Fibrosis Research

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Anti-topoisomerage I Antibodies
Aspiration and Lung Disease
Biomarkers for Pulmonary Fibrosis
Sleep and Pulmonary Fibrosis
Thrombin'sRole in SSc
Vitamin D Deficiency
Treatments and Clinical Trials

Overview of Pulmonary Fibrosis Research

Research on pulmonary fibrosis shows that it is important for all systemic sclerosis (scleroderma) patients to have their Vitamin D levels tested an optimized. Equally important is to make sure that heartburn is well controlled because it may play a role in causing interstitial lung disease when it is aspirated into the lungs. Some of the fatigue with ILD/PF might be due to related sleep disorders. (Also see What is Scleroderma?)

Anti-topoisomerase I Antibodies Predict Worse Outcome in SSc

The presence of anti-topoisomerase I antibodies is associated with a faster decline of lung function in scleroderma patients.

Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. Presence of anti-topoisomerase I antibodies (ATA) was the only baseline variable, associated with differential FVC (Forced Vital Capacity) levels, predicting the rate of decline in FVC within the first three years of follow up. Shervin Assassi. Arthritis Research & Therapy. Vol 12. Issue 5. Sept, 2010.

Aspiration and Interstitial Lung Disease

It is important to properly control heartburn in systemic scleroderma patients, because aspiration of acid reflux into the lungs may be a causative factor for pulmonary fibrosis.

Does Chronic Microaspiration Cause Idiopathic Pulmonary Fibrosis? Emerging data support a role for chronic microaspiration (i.e., subclinical aspiration of small droplets) in the pathogenesis and natural history of idiopathic pulmonary fibrosis. Joyce S. Lee, MD Volume 123, Issue 4, Pages 304-311 (April 2010). (Also see Aspiration Pneumonia)

Biomarkers for Pulmonary Fibrosis

Decrease of LL-37 in systemic sclerosis (SSc): a new marker for interstitial lung disease (ILD)? The circulating levels of LL-37 in SSc patients were investigated as lower LL-37 levels may be associated with the development of ILD. PubMed, Clin Rheumatol, 01/20/2015. (Also see: What is Scleroderma? and Scleroderma Biomarkers)

Sleep and Interstitial Lung Disease (ILD)

Interstitial lung disease seems to cause some sleeping problems, including decreased REM sleep, fragmented sleep, and possible obstructive sleep apnea. Addressing sleep disorders might help relieve some of the fatigue associated with pulmonary fibrosis.

Inflammatory Response Mechanisms Exacerbating Hypoxemia in Coexistent Pulmonary Fibrosis and Sleep Apnea. Increased understanding of the inflammatory mechanisms driving hypoxemia in pulmonary fibrosis and obstructive sleep apnea may potentiate the identification of appropriate therapeutic targets for developing effective therapies. Hindawi, 03/17/2015. (Also see Sleep Apnea)

Thrombin's Role in Causes of Scleroderma Lung Disease

Thombin triggers coagulation response, and it may play a role in the development of pulmonary fibrosis in systemic scleroderma.

Scleroderma Bronchoalveolar Lavage Fluid Thrombin Activity: Correlation with Pulmonary Function. Thrombin activity may not be a useful biomarker for decline in pulmonary function, but is increased in SSc cases when compared to controls. 2015 ACR/ARHP Annual Meeting. (Also see Causes of Scleroderma)

Vitamin D Deficiencies

Vitamin D deficiency common in patients with lung disease. in this study there was a high prevalence of vitamin D deficiency in individuals with chronic obstructive pulmonary disease (COPD), as well as a significant relationship between vitamin D deficiency and airflow limitations. Eureka, 04/20/2015. (Also see Vitamin D deficiency)

Treatments and Clinical Trials for Pulmonary Fibrosis

Scleroderma Treatments and Clinical Trials: Open Enrollments. Find clinical trials that are now recruiting for scleroderma and related illnesses, such as pulmonary fibrosis and pulmonary hypertension. ISN.

Symptom Treatments
Actemra (Tocilizumab)
ADRC Injections/STAR Trial
Allogenic Stem Cell Transplant
AM152 (Lysophosphatidic Acid 1)
Ambrisentan for Scleroderma
Bardoxolone by Reata
BEAT Clinical Trial
Cyclophosphamide and rATG With Hematopoietic Stem Cell Support
Fludarabine/Donor Peripheral SCT
GS-4997 (Selonsertib)
Hydrogen Sulphide
Nilotnib (Tasigna)
Nintedanib (OFEV)
Opsumit® (Macitentan)
Rituximab for PAH
SAR156597 for IPF (Sanofi)
Scleroderma Lung Study II
SPIN: Patient-centered Network
Stem Cell Transplants for Scleroderma
Tracleer® (Bosentan)
Twins and Siblings Study
Velcade® (Bortezomib)
Go to Pulmonary Fibrosis: Stories
Recent Donors
See ISN News for recent donors, including memorials for
Christine Kane, Joleen M. Cascaden.

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