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?)

Scleroderma-related lung disease: are adipokines involved pathogenically? The systemic nature of scleroderma-related lung disease has led many to implicate circulating factors in the pathogenesis of some of its organ impairment. PubMed, Curr Rheumatol Rep, 2013 Dec;15(12):381. (Also see Causes of Scleroderma: Cytokines)

Scientists identify agent that can block fibrosis of skin, lungs. Researchers at the University of Pittsburgh School of Medicine have identified an agent, E4 (which is a piece of protein or peptide derived from endostatin) that in lab tests protected the skin and lungs from fibrosis. MedicalXpress. 05/30/12. (Also see Skin Fibrosis)

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)

Biomarkers in connective tissue disease-associated interstitial lung disease. Besides autoantibodies, an increase in serum or BALF of a biomarker of pulmonary origin may be able to predict or reflect the development of fibrosis, the impairment of lung function, and ideally also the prognosis. PubMed, Semin Respir Crit Care Med, 2014 Apr;35(2):181-200. (Also see Scleroderma Biomarkers)

Increased circulating soluble vascular adhesion protein-1(sVAP-1) levels in systemic sclerosis (SSc): association with lower frequency and severity of interstitial lung disease. Study results suggest that sVAP-1 plays a role in the pathogenesis of interstitial lung disease in SSc. International Journal of Rheumatic Diseases, 28 May 2013.

Biomarkers from bronchoalveolar lavage fluid (BALF) in systemic sclerosis (SSc) patients with interstitial lung disease (ILD) relate to severity of lung fibrosis. In SSc related ILD, biomarkers from BALF and serum correlate to findings on HRCT suggesting usefulness as markers of presence and extent of lung fibrosis. PubMed, Respir Med, 2013 May 6. (Also see Induced Sputum and Bronchoalveolar Lavage Tests)

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
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
Most Recent Donors

Winn Schillberg
Reading Voices of Scleroderma Books: Diana Kramer.
Sharing Scleroderma Awareness Bracelets: Deb Martin, Brenda Miller, Vickie Risner.
Thanks to UNITED WAY donors of Central New Mexico and Snohomish County!

In Loving Memory

Patricia Ann Black: Marilyn Currier, Shelley Ensz, Richard Howitt, Gerald and Pat Ivanejko, Juno Beach Condo Association, Keith and Rosalyn Miller, and Elaine Wible.
Gayle Hedlin: Daniel and Joann Pepper and Nancy Smithberg.
Janet Paulmenn: Anonymous, Mary Jo Austin, Shelley Blaser, Susan Book, Dennis and Pat Clayton, Grace Cunha, Cindy Dorio, Michael and Patricia Donahue, Shelley Ensz, Nancy Falkenhagen, Jo Frowde, Alice Gigl, Margaret Hollywood, Karen Khalaf and Family, Susan Kvarantan, Bradley Lawrence, Jillyan Little, Donna Madge, Michele Maxson, Barry and Judith McCabe, John Moffett, My Tribute Foundation, Joan-Marie Permison, John Roberts, Margaret Roof, Maryellen Ryan, Mayalin and Kiralee Murphy, Nancy Settle-Murphy, and Bruce and Elizabeth Winter.


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