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)
Therapeutic Plasma Exchange
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.

The Treatment of Lung Involvement in Systemic Sclerosis (SSc). Treatment planning and intensity are guided by the disease severity and risk of progression and herein, recent advances in SSc-interstitial lung disease treatment will be explored. PubMed, Pharmaceuticals (Basel), 2021 Feb 13;14(2):154.

Disease staging and sub setting of interstitial lung disease associated with systemic sclerosis (SSc–ILD): impact on therapy. In the management of SSc–ILD, a multidisciplinary team approach which integrates physiology and radiology with the patient at the centre of the process is crucial for effective management and provision of the best outcomes. PubMed, Expert Rev Clin Immunol, 01/10/2018.

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.


NOX1 Promotes Mesothelial-Mesenchymal Transition (MesoMT) Through Modulation of ROS-mediated Signaling. NOX4 down-regulation attenuated TGF-ß- and thrombin-mediated MesoMT. PubMed, Am J Respir Cell Mol Biol, 01/29/2021.

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.

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.

Safety and tolerability of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SSC-ILD)): data from the SENSCIS trial. The adverse event profile of nintedanib in patients with SSc-ILD is consistent with its established safety and tolerability profile in patients with idiopathic pulmonary fibrosis. PubMed, Ann Rheum Dis, 2020 Nov;79(11):1478-1484.

Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the forced vital capacity was significantly lower among patients who received nintedanib than among those who received placebo. PubMed, N Engl J Med, 2019 Oct 31;381(18):1718-1727. (Also see Clinical Trials)

FDA approves first treatment for patients with rare type of lung disease. The U.S. Food and Drug Administration today approved Ofev (nintedanib) capsules to slow the rate of decline in pulmonary function in adults with interstitial lung disease associated with systemic sclerosis or scleroderma, called SSc-ILD. FDA News Release, 09/06/2019. (Also see Clinical Trials)

Phase III study showed nintedanib slows the loss of pulmonary function in people living with systemic sclerosis associated interstitial lung disease (ILD). Results show that nintedanib slows the loss of pulmonary function in patients with SSc-ILD compared to placebo. Business Wire, 05/20/2019. (Also see Clinical Trials)

Exploring Novel Treatment for Scleroderma–Associated Interstitial Lung Disease. This study is one of three major multicenter trials in which Cleveland Clinic’s Rheumatic Lung Disease Program is participating. Consult QD, 12/13/2018. (Also see Clinical Trials)

Therapeutic Plasma Exchange

Scleroderma renal crisis (SRC) during intravenous cyclophosphamide pulse therapy for complicated interstitial lung disease (ILD) was successfully treated with angiotensin converting enzyme inhibitor and plasma exchange (PE). This study presents the possibility of favorable effects of PE for SSc–associated ILD and SRC. PubMed, Nagoya J Med Sci. (Also see Kidney (Renal) Involvement and Therapeutic Plasma Exchange)

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.

Pulmonary Fibrosis Clinical Trials

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. (Also see Cellcept, Cyclophosphamide, and Clinical Trials)

Clinical Trials for Pulmonary Fibrosis.

Pulmonary Fibrosis Research on New Treatments

MicroRNA-320a: an important regulator in the fibrotic process in interstitial lung disease (ILD) of systemic sclerosis (SSc). Investigation of more detailed mechanisms of miR-320a-mediated regulation of collagen expression may provide new therapeutic strategies for SSc-ILD. PubMed, Arthritis Res Ther, 2021 Jan 11;23(1):21.

B cell activating factor (BAFF) inhibition attenuates fibrosis in scleroderma (SSc) by modulating the regulatory and effector B cell balance. BAFF inhibition is a potential therapeutic strategy for SSc via alteration of B cell balance. Science Advances, 07/11/2018.

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. PubMed, J Rheumatol.

Go to Pulmonary Fibrosis Treatments: Biologics

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