CellCept® (Mycophenolate Mofetil)
Lung Transplant Media Stories
Therapeutic Plasma Exchange
PF Clinical Trials
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.
Managing systemic sclerosis–related interstitial lung disease (SSC–ILD) in the modern treatment era. This review describes the current and experimental therapies available and also explores unanswered questions directly relevant to patient care and future research efforts for SSc–ILD. Journal of Scleroderma and Related Disorders, JSRD 2017; 2(2): 72 - 83.
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.
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.
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 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) 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) 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) 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 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.
Idiopathic means of unknown cause.
Ofev® (Nintedanib) Prescribing Information. Ofev is a prescription medication used to treat people with idiopathic pulmonary fibrosis. Boehringer Ingelhaim.
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, 2016 Aug;78(3):329-34. (Also see Kidney (Renal) Involvement and Therapeutic Plasma Exchange)
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. Clinicaltrials.gov.
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)
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. ClinicalTrials.gov
The Antiretroviral nelfinavir mesylate (NFV), a potential therapy for systemic scleroderma. NFV abrogates TGF–ß1–mediated myofibroblast differentiation and pulmonary fibrosis through off–target protein binding and supports consideration of this FDA–approved medication as an anti–fibrotic agent. PubMed, Arthritis Rheumatol, 09/21/2017. (Also see Fibroblasts)
Safety, Tolerability, and Efficacy Study of Idiopathic Pulmonary Fibrosis (FGCL-3019-049) To evaluate the safety and tolerability of FG-3019 in subjects with Idiopathic Pulmonary Fibrosis (IPF) and the efficacy of FG-3019 for attenuating fibrosis in these subjects. ClinicalTrials.gov, 08/04/2017.
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.
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