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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.)

Mortality and Prognosis

Progressive interstitial lung disease in patients with systemic sclerosis–associated interstitial lung disease (SSc–ILD) in the EUSTAR database. SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. PubMed, Ann Rheum Dis, 2021 Feb;80(2):219-227.

Serum markers of pulmonary epithelial damage in systemic sclerosis–associated interstitial lung disease (SSc-ILD) and disease progression. Our results suggest serum KL-6 predicts decline in lung function in SSc, suggesting its clinical utility in risk stratification for progressive SSc-ILD. PubMed, Respirology, 12/17/2020.

Increased expression of interferon regulated and antiviral response genes in CD31+/CD102+ lung microvascular endothelial cells from systemic sclerosis patients with end-stage interstitial lung disease. These observations suggest that interferon-induced and antiviral response proteins may participate in the pathogenesis of SSc vasculopathy and SSc-ILD. PubMed, Clin Exp Rheumatol, 11/17/2020.

Bioactive Plasma Mitochondrial DNA (mtDNA) Is Associated With Disease Progression in Scleroderma Associated Interstitial Lung Disease (SSc-ILD). These findings demonstrate an unrecognized connection between extracellular vesicles-encapsulated mtDNA, clinical outcomes, and intracellular DNA-sensing pattern recognition receptors activation in SSc-ILD. PubMed, Arthritis Rheumatol, 06/30/2020.

Systemic sclerosis (SSc) and the COVID-19 pandemic: World Scleroderma Foundation preliminary advice for patient management. SSc patients are a great challenge for the physician to achieve an effective protective strategy or, when infected, to optimise a real-time treatment as suggested by the rapidly evolving guidelines. BMJ Journals, Ann Rheum Dis, 04/29/2020.

Pneumoproteins KL-6 and CCL-18 Predict Progression of Interstitial Lung Disease in Systemic Sclerosis (SSc-ILD). In a rigorously-conducted clinical trial for SSc-ILD, KL-6 and CCL-18 levels correlated with ILD severity and declined with immunosuppression. PubMed, Arthritis Rheumatol, 06/24/2019.

Relationship Between Interstitial Lung Disease (ILD) and Oesophageal Dilatation on Chest High-Resolution Computed Tomography (HRCT) in Patients With Systemic Sclerosis: A Cross-Sectional Study. An increased oesophageal diameter ( ≥ 11 mm) on chest HRCT is associated with pulmonary and oesophageal symptoms, more severe ILD, and lower DLco. PubMed, Radiol Med, 2018 Sep;123(9):655-663. (Also see Esophageal (Throat) Involvement)

Short–term progression of interstitial lung disease (ILD) in systemic sclerosis (SSc) predicts long–term survival in two independent clinical trial cohorts. These findings suggest that short–term changes in surrogate measures of SSc–ILD progression may have important effects on long–term outcomes. PubMed, Ann Rheum Dis, 11/08/2018.

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. (Also see Antibodies in Systemic Scleroderma)


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)
Therapeutic Plasma Exchange
Velcade® (Bortezomib)
PF Clinical Trials
PF Research

Pulmonary Fibrosis Research

TL1A Promotes Lung Tissue Fibrosis and Airway Remodeling. Reagents that disrupt the interaction of TL1A with DR3 then have the potential to prevent deregulated tissue cell activity in lung diseases that involve fibrosis and remodeling. PubMed, J Immunol, 2020 Sep 21;ji2000665.

Short or long sleep associated with Idiopathic Pulmonary Fibrosis. The study reveals that targeting the body clock reduces fibrosis in vitro, revealing a potential target for this incurable disease. University of Manchester, 12/30/2019.

Interferon Lambda (IFNL3) genotype is associated with pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc). IFNL3 serum levels and the genetic variant known to be associated with liver fibrosis are similarly linked to PF, but not to worsening of skin fibrosis in SSc. PubMed, Sci Rep, 2019 Oct 16;9(1):14834.

Blockade of CCL24 with a monoclonal antibody ameliorates experimental dermal and pulmonary fibrosis. CCL24 plays an important role in pathological processes of skin and lung inflammation and fibrosis. PubMed, Ann Rheum Dis, 05/25/2019.

Increased monocyte count as a cellular biomarker for poor outcomes in fibrotic diseases: a retrospective, multicentre cohort study. Further investigation into the mechanistic role of monocytes in fibrosis might lead to insights that assist the development of new therapies. PubMed, Lancet Respir Med, 03/29/2019.

Anti-C1q autoantibodies (autoAbs) are frequently detected in patients with systemic sclerosis (SSc) associated with pulmonary fibrosis. Anti-C1q autoAbs were frequently detected in patients with SSc, and their high levels predict the co–occurrence of pulmonary fibrosis or pulmonary arterial hypertension. PubMed, Br J Dermatol, 03/15/2019.

Recent progress in systemic sclerosis–interstitial lung disease (SSc–ILD). The implications of much of this ongoing work is our ability to identify those patients at risk for progression, and to offer novel therapies that can limit the progression of inflammatory and fibrotic lung disease. PubMed, Curr Opin Rheumatol, 2018 Nov;30(6):570-575.

TLR4-dependent fibroblast activation drives persistent organ fibrosis in skin and lung. The results suggest that systemic scleroderma patients with high TLR4 activity might show optimal therapeutic response to selective inhibitors of MD2/TLR4 complex formation. PubMed, JCI Insight, 2018 Jul 12;3(13). (Also see Fibroblasts and Skin Fibrosis)


(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.

Go to Pulmonary Fibrosis: Correlations

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