Pulmonary Fibrosis

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Disease Correlations
Living with PF
Patient Stories


Pulmonary (lung) fibrosis, is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). It is often also referred to as interstitial lung disease.

Pulmonary fibrosis (PF) can occur by itself or as secondary to some autoimmune diseases, including systemic sclerosis (SSc, scleroderma).

What is pulmonary fibrosis? Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions such as sarcoidosis, hypersensitivity pneumonitis, asbestosis, certain medications, etc. MedicineNet

About 70% of patients with diffuse scleroderma develop some degree of pulmonary fibrosis, which is the most common cause of death directly related to scleroderma. Therefore, prompt diagnosis and aggressive treatment of pulmonary fibrosis is very important.

Pulmonary fibrosis is also one of the three minor criteria (1) for the classification of limited systemic scleroderma. (See Types of Scleroderma.)

Key roles for interferon- and TGF-beta-regulated genes, and macrophage activation in progressive lung fibrosis associated with Systemic Sclerosis. This highlights major pathogenic pathways relevant to progressive pulmonary fibrosis in SSc-ILD: macrophage activation, and upregulation of TGF-beta- and IFN-regulated genes. PubMed, Arthritis Rheum, 2013 Nov 27.

Mortality and Prognosis

Esophageal dilatation and interstitial lung disease in systemic sclerosis (SSc): A cross-sectional study. Increasing esophageal diameter on high–resolution computed tomography in patients with SSc is associated with more severe radiographic interstitial lung disease, lower lung volumes, and lower DLCO % predicted. PubMed, Semin Arthritis Rheum, 2016 Aug;46(1):109-14. (Also see Esophageal (Throat) Involvement)

Clinical characteristics and survival in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated usual interstitial pneumonia (CTD-UIP). Although the survival of CTD-UIP patients was similar compared with that of IPF/UIP patients, it appears that Undifferentiated Connective Tissue Disease influences the survival rate of CTD-UIP patients. Journal of Thoracic Disease, 04/03/2015. (Also see Connective Tissue Disease)

Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody. Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile. Seminars in Arthritis and Rheumatism, 07/15/2014. (Also see Antibodies in Systemic Scleroderma)

Proteome-wide Analysis and CXCL4 as a Biomarker in Systemic Sclerosis. Levels of CXCL4 were elevated in patients with systemic sclerosis and correlated with the presence and progression of complications, such as lung fibrosis and pulmonary arterial hypertension. The New England Journal of Medicine, 12/18/2013. (Also see Biomarkers and Pulmonary Hypertension)

Skin gene expression correlates of severity of interstitial lung disease (ILD) in systemic sclerosis. A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD. American College of Rheumatology, 07/30/2013.


Symptoms of pulmonary fibrosis include shortness of breath (dyspnea) on exertion. In treating scleroderma, its best to identify pulmonary fibrosis before it is so advanced that it shows up on x-ray. That is why your doctor may order an echocardiogram, or other tests. ISN.

Correlation of Pulmonary Fibrosis with Other Complications

Pulmonary fibrosis complications iconPulmonary Fibrosis Correlations and Complications. Pulmonary fibrosis can be correlated or complicated with the following conditions, such as esophageal issues, cancer, connective tissue diseases, heart problems, infections, pulmonary hypertension and thyroid diseases. ISN.

Lung and Esophageal
Pulmonary Fibrosis and:
—Connective Tissue Disease
—Pulmonary Hypertension
—Thyroid Disease
Patient Stories


Diagnosis of Pulmonary Fibrosis. All systemic sclerosis patients should have regular screening for both pulmonary fibrosis and pulmonary hypertension. ISN.

High Resolution Computed Tomography
Induced Sputum and Bronchoalveolar Lavage
Lung Biopsy
Pulmonary Function Tests
Walking and Stress Tests
Pulmonary Fibrosis Antibodies

Living with Pulmonary Fibrosis

Living with Pulmonary Fibrosis requires a constant awareness of your symptoms, your breathing, your surroundings, and your overall well-being. Work closely with your doctors on treatments, diet and exercise. If you require oxygen therapy, use it. It protects your heart as well as improves the quality of your life. ISN.

Air Quality
Oxygen Therapy
Dangers of Oxygen
Support Groups


Treatments for Pulmonary Fibrosis include oxygen therapy, oral and IV cyclophosphamide, biologic agents, Mycophenolate Mofetil (cellcept), and lung transplants. There are current clinical trials that are studying the effectiveness of various treatments for scleroderma. Some of these trials are using the treatment's effect on the patient's pulmonary fibrosis as a measurement criterion. ISN.

Antihistamine Warning
Biologic Agents
CellCept® (Mycophenolate Mofetil)
Cyclosporine (CYC)
Esbriet® (Pirfenidone)
Lung Transplant
Lung Transplant Media Stories
Ofev® (Nintedanib)
Velcade® (Bortezomib)
PF Clinical Trials
PF Research

Pulmonary Fibrosis Research

Netrin–1 Regulates Fibrocyte Accumulation in the Decellularized Fibrotic Sclerodermatous Lung Microenvironment and in Bleomycin–Induced Pulmonary Fibrosis. Netrin–1 regulates bleomycin–induced pulmonary fibrosis in mice and it might be a novel therapeutic target in scleroderma–related interstitial lung disease. PubMed, Arthritis Rheumatol, 2016 May;68(5):1251-61.

Frequency of circulating topoisomerase–I–specific CD4 T cells predicts presence and progression of interstitial lung disease (ILD) in scleroderma. Topo–I–specific T cells can be reliably quantified in the peripheral blood of patients with scleroderma, exhibit a pro–inflammatory Th17 phenotype, and predict progression of ILD. BioMed Central, Arthritis Research & Therapy, 05/04/2016. (Also see Causes of Scleroderma: B Cells and T Cells)

M10, a caspase cleavage product of the hepatocyte growth factor receptor, interacts with Smad2 and demonstrates antifibrotic properties in vitro and in vivo. M10 peptide interacts with Smad2 and demonstrates strong antifibrotic effects in vitro and in vivo in an animal model of lung fibrosis and should be considered as a potential therapeutic agent for systemic sclerosis and other fibrosing diseases. PubMed, Transl Res, 2016 Apr;170:99-111. (Also see Skin Fibrosis)

Research of Lung Fibrosis in Scleroderma (SSc) Reveals High Levels of Antibodies That Correlate with Disease Progression. Circulating antibodies against specific chemokine receptors exist in higher levels in patients with SSc and these antibodies correlate with disease progression and clinical manifestations of the disease. Scleroderma News, 02/25/2016.

Scleroderma–related Interstitial Lung Disease (SSc–ILD) Not Linked to Idiopathic Interstitial Pneumonia. The study confirmed that SSc–ILD and idiopathic interstitial pneumonia are different diseases, not sharing a genetic basis. Scleroderma News, 02/02/2016. (Also see Lung Involvement)

Fewer Mycophenolate Adverse Events in Scleroderma Lung Study. Mycophenolate mofetil can be substituted for cyclophosphamide for the immunosuppressive treatment of scleroderma-related interstitial lung disease and might even be safer. Medscape, 11/02/2015. (Also see Immunosuppressants and Clinical Trials)

Pleural irregularity (PI), a new ultrasound sign for the study of interstitial lung disease(ILD) in systemic sclerosis (SSC) and antisynthetase syndrome (ASS). PI is useful for evaluation of ILD in SSc and ASS patients and can be incorporated into a diagnostic algorithm in SSc patients to reducing the need for exposure to ionising radiation. PubMed, Clin Exp Rheumatol, 08/27/2015.

Measures of Lung Function Sensitive to Comorbidity of Scleroderma. Accurately determining functional impairment of the respiratory system of scleroderma patients is essential to helping their recovery from symptoms. Scleroderma News, 04/24/2015.

Reconciling Healthcare Professional and Patient Perspectives in the Development of Disease Activity and Response Criteria in Connective Tissue Disease-related Interstitial Lung Diseases (CTD-ILD). Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. PubMed, J Rheumatol, 2014 Feb 1. (Also see Connective Tissue Disease)

Combined Pulmonary Fibrosis and Emphysema (CPFE) Among Patients With Scleroderma (SSc)-Associated Pulmonary Hypertension (PH). There appears to be no difference in the severity of PH or in pulmonary function between SSc-PH patients with and without CPFE. Chest, 2013;144. (Also see Pulmonary Hypertension)

Pulmonary Fibrosis Preceding Skin Involvement in Scleroderma. Pulmonary fibrosis can be the initial manifestation of scleroderma, preceding skin involvement. Chest, 2013;14.

Targeting Focal Adhesion Kinase (FAK) in Fibrotic Diseases. Recent findings suggest that FAK plays a key role in development of fibrotic disorders, and it appears to be an attractive target for antifibrotic therapy and has potential as a future therapeutic target to counteract fibrosis. PubMed, BioDrugs, 2012 Nov 27. (Also see Pulmonary Fibrosis Research and Clinical Trials)


(1) Reference: Subcommittee for Scleroderma criteria of the American Rheumatism Association diagnostic and therapeutic criteria committee. 1980. Preliminary criteria for the classification of systemic sclerosis (Scleroderma). Arthritis Rheum. 23,581:590.

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