Treatments for Pulmonary Fibrosis

This page was written by Shelley Ensz, and has not been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer

Overview of Treatments
Antihistamine Warning
Anticoagulant

Cyclophosphamide (Cytoxan)
Biologic Agents
Mycophenolate Mofetil (Cellcept)

Lung Transplant
Clinical Trials

Oxygen Therapy
Research on New Treatments

Overview of Treatments

Treatments for Pulmonary Fibrosis include oxygen therapy, oral and IV cyclophosphamide (cytoxan), 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.

Current concepts in disease-modifying therapy for systemic sclerosis (SSc)-associated interstitial lung disease (ILD): Lessons from clinical trials. Newer therapies for SSc patients with ILD include mycophenolate mofetil, cyclophosphamide, tyrosine kinase inhibitors (imatinib, dasatinib), and anti-interleukin-13 monoclonal antibody. Several uncontrolled trials have reported favorable results of mycophenolate mofetil in SSc-related ILD. Karen Au. (Metapress) Current Rheumatology Reports. Volume 11, Number 2. April, 2009.

Scleroderma lung disease: evolving understanding in light of newer studies. Treatment benefits consist of the prevention of progression and are largely confined to patients with extensive pulmonary fibrosis. Accurate prognostic evaluation by staging the severity of lung disease remains central to management and will be a major focus of future studies. Antoniou KM. (PudMed) Curr Opin Rheumatol. 2008 Nov;20(6):686-91.

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. Merck.com.

Anticoagulant

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.

Cyclophosphamide (Cytoxan)

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

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.

Mycophenolate Mofetil (Cellcept)

Mycophenolate Mofetil (Cellcept) 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 (cytoxan). ISN.

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.

Clinical Trials for Pulmonary Fibrosis

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. J. Seibold, Arthritis Rheum. 2010 Mar 26;62(7):2101-2108. (Also see: Bosentan and Dr. JR Seibold)

Scleroderma Lung Study II. This study compares 2 different medications—daily oral cyclophosphamide (CYC, also called CytoxanTM) 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, Cytoxan, and Clinical Trials)

Safety Evaluation of Dasatinib in Subjects With Scleroderma Pulmonary Fibrosis. The purpose of this study is to see if dasatinib, with its known side effects, is safe to use in patients with scleroderma pulmonary fibrosis. This study is currently recruiting participants. Clinicaltrials.gov. August 2009. (Also see: Clinical Trials)

Pulmonary Fibrosis with Systemic Sclerosis. 2008 recruiting is taking place for a multi-center placebo controlled research study for pulmonary fibrosis with systemic sclerosis sponsored by Novartis Pharmaceuticals. Male and female patients, 18 to 65 years of age may be eligible for participation. (Also see: Clinical Trial Open Enrollments)

Clinical Trials for Pulmonary Fibrosis. ClinicalTrials.gov

Pulmonary Fibrosis Research on New Treatments

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.

Positive Data From Lung Study May Lead to First FDA Approved Treatment for Pulmonary Fibrosis PF). A pivotal Phase III clinical trial is completed for Pirfenidone and data released. The positive data could pave the way for the first FDA-approved therapy to treat PF. Breakthrough Digest. 02/04/09. (Also see: Clinical Trials)