| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Fibrosis: MAIN MENU |
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| 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 |
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| 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. |
| Advances in immunosuppressive therapy. This represents the first evidence that immunosuppressive drugs are efficacious in rheumatic disease-associated interstitial fibrosis and provides a rationale for developing therapeutic regimens that optimize efficacy and safety. PubMed. Semin Respir Crit Care Med. 2007 Aug;28(4):398-417. (Also see: Medications ) |
| Against Pulmonary Fibrosis. The biotech companies Digna Biotech and Biotherapix have signed an agreement to jointly apply their patented products towards the development of a treatment for pulmonary fibrosis. Medical News Today. 03/09/06. |
| Bosentan for severe pulmonary arterial hypertension related to systemic sclerosis with interstitial lung disease. Bosentan treatment was well tolerated in this cohort of SSc patients with interstitial lung disease and was effective for treatment of severe PAH in the majority of patients. PubMed. Eur J Clin Invest. 2006 Sep;36 Suppl 3:44-8. (Also see: Pulmonary Hypertension ) |
 Scleroderma Care and Research Journal (PDF) This inaugural issue for physicians focuses on elevating the standards of care for scleroderma lung involvement. Articles include Interstitial Lung Disease in Systemic Sclerosis: Optimizing Evaluation and Management, as well as Pulmonary Hypertension Related to Systemic Sclerosis: A Primer for the Rheumatologist. Journal of the Scleroderma Clinical Trials Consortium (SCTC) Vol 1, No. 1, Autumn 2003 (Also see: Pulmonary Hypertension) |
| Scleroderma Treatment Differs Among Experts vs. General Rheumatologists. Scleroderma experts see more systemic sclerosis (SSc) patients and use more immunosuppressives including unproven treatments. They also use less drugs where a negative trial exists (such as D-penicillamine) than other rheumatologists. There are some differences in the treatment of SSc between Canada, the US and the Scleroderma Clinical Trials Consortium (SCTC), perhaps due to the lack of guidelines and proven interventions. SCTC may treat more severe cases, but it also may be that ILD (interstitial lung disease) and PAH (pulmonary arterial hypertension) are under-recognized by non-experts. J. E. Pope. Pres. No. 473. ACR Conference Oct. 2003. (Also see: Scleroderma Specialists, Pulmonary Hypertension, Clinical Trials and Ineffective Treatments: D-penicillamine, Scleroderma Experts: SCTC) |
| Interstitial lung disease in connective tissue disorders. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression. PubMed. Rev Pneumol Clin. 2005 Jun;61(3):211-9. |
| Fibrosis regression induced by intravenous gammaglobulin treatment.I IVIg may enhance resorption of fibrosis and promote healing in patients with fibrotic associated disorders. PubMed. Ann Rheum Dis 2003 Feb;62(2):175-177 (Also see: Skin Fibrosis) |
| 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 |
| 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 |
| Mycophenolate Mofetil (Cellcept) as First-Line Treatment Improves Clinically-Evident Early Scleroderma Lung Disease. Our preliminary data suggest that in patients with recent, clinically apparent dSSc-related alveolitis, early treatment with Cellcept and small doses of corticosteroids may represent an effective, well-tolerated and safe alternative therapy. S. C. Liossis. SAT0210 EULAR 2006. (Also see: Medications ) |
| The Efficacy of Cyclophosphamide Pulse Therapy in Patients with Systemic Sclerosis and Lung Involvement. In patients with systemic sclerosis and lung involvement, an improvement of lung diffusing capacity was noticed six months after beginning of cyclophosphamide pulse therapy, with only trivial side-effects. P. S. Ostojic. FRI0121 EULAR 2005. |
| Clinical aspects of lung involvement: lessons from idiopathic pulmonary fibrosis and the scleroderma lung study. Optimal treatment of SSc-ILD remains to be determined, but cyclophosphamide has been reported to be effective in a number of case series. A randomized controlled trial, the Scleroderma Lung Study (SLS), will be completed in 2005; the outcome of the SLS should define the efficacy of daily oral cyclophosphamide for SSc-ILD patients with alveolitis. PubMed. Curr Rheumatol Rep. 2005 Apr;7(2):135-41. |
| Pulmonary Fibrosis Research on New Treatments |
| Mechanisms of Disease: leukotrienes and lipoxins in scleroderma lung disease--insights and potential therapeutic implications. Pharmacologic correction of a leukotriene-lipoxin imbalance using leukotriene inhibitors or lipoxin analogs might be a new approach to the treatment of SLD. PubMed. Nat Clin Pract Rheumatol. 2007 Jan;3(1):43-51. |
| New Developments in Scleroderma Interstitial Lung Disease. Basic and clinical studies of systemic sclerosis patients with interstitial lung disease are yielding promising data that ultimately will be translated in to more effective diagnostic and therapeutic strategies. PubMed. Curr Opin Rheumatol. 2005 Nov;17(6):737-745. |
| Curcumin-Induced Apoptosis in Scleroderma Lung Fibroblasts: Role of Protein Kinase C {epsilon}. These observations suggest that curcumin may have therapeutic value in treating scleroderma, just as it has already been shown to protect rats from lung fibrosis induced by a variety of agents. PubMed. Am J Respir Cell Mol Biol. 2004 Jan 23. (Also see: Pulmonary Fibrosis) |
