Treatments for Pulmonary Fibrosis

Author: Shelley Ensz. See Pulmonary Fibrosis. Read Disclaimer
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

Overview of Treatments

Medications may be used to attempt to slow progression of pulmonary fibrosis. Disease management includes regular flu and pneumonia vaccinations. (Also see Pulmonary Fibrosis, Pulmonary Involvement and What is Scleroderma?)

Some people eventually need supplemental oxygen. Lung transplantation may be considered if medication doesn't work, however many scleroderma patients are not good candidates for transplantation because of other disease complications.

Pulmonary fibrosis medications include oral and IV cyclophosphamide, biologic agents, Esbriet, and mycophenolate mofetil (Cellcept). 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.

Changes in plasma CXCL4 levels are associated with improvements in lung function in patients receiving immunosuppressive therapy for systemic sclerosis–related interstitial lung disease. Findings suggest that intermediate–term changes in CXCL4 may have predictive significance for long–term progression of SSc–ILD in patients receiving immunosuppressive therapy. PubMed, Arthritis Res Ther, 2016 Dec 30;18(1):305. (Also see Immunosuppressants)

A decision model for the watch-and-wait strategy in systemic sclerosis-associated interstitial lung disease (SSc-ILD). Watchful waiting may be effective for SSc-ILD patients who have minimal pulmonary involvement on CT and lack pulmonary arterial hypertension on echocardiography at baseline. PubMed, Rheumatology (Oxford), 05/13/2015.

Interstitial lung disease (ILD) in connective tissue disease (CTD) - mechanisms and management. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease. PubMed, Nat Rev Rheumatol, 2014 Sep 30. (Also see Connective Tissue Disease)

Lung Treatments Slow Damage From Fatal Lung Disease in Studies. Drugs made by Intermune Inc. and Boehringer Ingelheim GmbH slowed progression of a lung disease with no cure that often kills people three to five years after diagnosis, according to findings reported by two studies. Bloomberg, 05/19/2014.

Antihistamine Warning

A warning about antihistamines and Lung Disorders. Antihistamines, which dry the respiratory tract, have little or no value in treating a cough, except when it is caused by an upper airway allergy. With coughs from other causes, such as bronchitis, the drying action of antihistamines can be harmful, thickening respiratory secretions and making them difficult to cough up.


Dabigatran, a direct thrombin inhibitor, demonstrates antifibrotic effects on lung fibroblasts. Dabigatran restrains important profibrotic events in lung fibroblasts and warrants study as a potential antifibrotic drug for the treatment of fibrosing lung diseases such as scleroderma lung disease and idiopathic pulmonary fibrosis. GS Bogatkevich. Arthritis Rheum 2009 Nov.

Biologic Agents

Biologic agents are biologic response modifying agents that block specific pathways and signals of inflammation. Because of their success with other rheumatic diseases and symptoms, biologics are now be tested for their effectiveness on pulmonary fibrosis. ISN.

CellCept® (Mycophenolate Mofetil) by Genentech

CellCept®(mycophenolate mofetil) is relatively new in the treatment of pulmonary fibrosis. However, a few small studies have proven it to be effective in slowing the progression of pulmonary fibrosis. It has also proven to be well-tolerated and safe when compared to cyclophosphamide. ISN.


Cyclophosphamide was also formerly known by the brand name Cytoxan.

Oral and intraveneous Cyclophosphamide have proven to be effective in the slowing the progress of pulmonary fibrosis related to scleroderma. ISN.

Cyclosporine (CYC)

Cyclosporine (CYC) in Anti-Jo1-positive Patients with Corticosteroid-refractory Interstitial Lung Disease (ILD). CYC is effective and substantially safe in patients with anti-Jo1 antisynthetase syndrome with corticosteroid-refractory ILD. CYC withdrawal may be associated with ILD relapse, and low-dose CYC was effective in ILD control. Journal of Rheumatology.

Esbriet® (Pirfenidone) by Genentech

Esbriet® (pirfenidone) Prescribing Information. This is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF). Genentech.

Learn about Esbriet® (pirfenidone). Esbriet has been approved outside of the United States since 2011. More than 27,000 patients have taken Esbriet worldwide. The effectiveness and safety of Esbriet were studied in three clinical trials of patients with IPF. Genentech.

Genentech’s ESBRIET Recommended in ATS Guidelines for IPF Treatment. FDA approval of Esbriet in the Fall of 2014 brought much-needed therapeutic options to those who suffer from IPF. Esbriet has been shown in different Phase III clinical trials to slow the decline of lung function in IPF patients and consequently disease progression. Pulmonary Hypertension News, 08/12/2015.

Lung Transplant

Lung Transplants, in most cases, is the last resort. This is due to it being a very invasive procedure and requires a lung donor. This procedure is also extremely expensive compared to other treatments. However, lung transplants have been proven to be effective for patient with scleroderma related pulmonary fibrosis. ISN.

Ofev® (Nintedanib) by Boehringer Ingelheim

Idiopathic means of unknown cause.

Ofev® (Nintedanib) Prescribing Information. Ofev is a prescription medication used to treat people with idiopathic pulmonary fibrosis. Boehringer Ingelhaim.

Velcade® (Bortezomib) by Millenium Pharmaceuticals

Bortezomib is being studied in clinical trials for scleroderma pulmonary fibrosis.

Comparing and Combining Bortezomib and Mycophenolate in SSc Pulmonary Fibrosis. This is a Phase II clinical trial at Northwestern. It is still recruiting as of March 2016.

Cancer drug may also work for scleroderma. In mouse models and human fibroblast cells, Bortezomib (brand name Velcade), which is used to treat multiple myeloma, appears to put the brakes on abnormal development of scar tissue in the lungs and skin and may also work in other organs. Northwestern University, Eurekalert, 09-22-11. (Also see: Scleroderma Treatments and Clinical Trials: Bortezomib)

Pulmonary Fibrosis Clinical Trials

OFEV (nintedanib) to Be Tested in Systemic Sclerosis Patients (SSc) with Interstitial Lung Disease (ILD). The potential use of nintedanib in conditions that commonly occur with fibrotic lung conditions, such as systemic sclerosis are being investigated. Scleroderma News, 12/22/2015. (Also see Scleroderma Clinical Trials)

Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Although many outcome variables were stable, bosentan did not reduce the frequency of clinically important worsening. These data do not support the use of endothelin receptor antagonists as therapy for ILD secondary to SSc. Arthritis Rheum, 2010 Mar 26;62(7):2101-2108. (Also see Bosentan)

Scleroderma Lung Study II. This study compares 2 different medications—daily oral cyclophosphamide (CYC) with daily oral mycophenolate mofetil (MMF, also called CellceptTM) in the treatment of scleroderma-related pulmonary fibrosis. There are twelve study centers across the U.S. This study is currently recruiting. University of California, Los Angeles. November 2009. (Also see Cellcept, Cyclophosphamide, and Clinical Trials)

Clinical Trials for Pulmonary Fibrosis.

Pulmonary Fibrosis Research on New Treatments

US FDA Grants Orphan Drug Status to FibroGen's FG-3019 to Treat Idiopathic Pulmonary Fibrosis. The US Food and Drug Administration (FDA) has granted Orphan Drug Designation to FibroGen's FG-3019, the human monoclonal antibody against connective tissue growth factor (CTGF), for the treatment of idiopathic pulmonary fibrosis (IF). CheckOrphan. 08/02/12.

Elevated Circulating TWEAK Levels in Systemic Sclerosis (SSc): Association with Lower Frequency of Pulmonary Fibrosis. TWEAK (tumor necrosis factor-related weak inducer of apoptosis ) levels were increased in patients with SSc, and associated with a lower frequency of pulmonary fibrosis in patients with SSc. TWEAK could be a protective factor against the development of pulmonary fibrosis in this disease and as such would be a possible therapeutic target. Koichi Yanaba. J Rheumatol August 2009 36(8):1657-1662.

Go to Pulmonary Fibrosis Treatments: Biologics
Recent Donors
See ISN News for recent donors, including memorials for
Christine Kane, Joleen M. Cascaden.

SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:

International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA

Email [email protected] to request our Welcome email, or to report bad links or to update this page content.

TOLL FREE HOTLINE 800-564-7099
Free to U.S. and Canadian Callers. Ask for our Free Info Packet by mail or email!
Scleroderma, Pulmonary Arterial Hypertension, and related illnesses.
Privacy Policy.

The most important thing in the world to know about scleroderma is!
Donate Now
Copyright 1998-2017, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.