An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial
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Objective.: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) shares a number of clinical features and pathogenic mechanisms with idiopathic pulmonary fibrosis (IPF). This study was designed to evaluate the tolerability of the IPF treatment pirfenidone in SSc-ILD. The known gastrointestinal, skin, and liver adverse events (AE) of pirfenidone are of importance given the involvement of these organs in SSc. Methods.: All patients received pirfenidone and were randomized 1:1 to either a 2- or 4-week titration starting at 801 mg/day and finishing at a maintenance dose of 2403 mg/day. Patients received pirfenidone for 16 weeks in total. Assessments included treatment-emergent AE (TEAE) and exploratory disease outcomes. Results.: Sixty-three patients were randomized; 96.8% experienced a TEAE and more patients reported TEAE during the titration versus the maintenance period. The most commonly reported TEAE were consistent with those observed for pirfenidone in IPF (nausea, headache, fatigue) and were similar regardless of titration schedule. More patients discontinued treatment because of TEAE in the 2- versus 4-week titration group (5 vs 1, respectively); all discontinuation events occurred > 3 weeks after reaching the full dose of pirfenidone. Mycophenolate mofetil (MMF), taken by 63.5% of patients in addition to pirfenidone, did not appear to affect tolerability. Exploratory disease outcomes remained largely unchanged. Conclusion.: Pirfenidone showed an acceptable tolerability profile in SSc-ILD, although a longer titration may be associated with better tolerability. Tolerability was not affected by concomitant MMF. The present findings support further investigation of pirfenidone in future clinical trials in patients with SSc-ILD. Trial registration: ClinicalTrials.gov; www.clinicaltrials.govNCT01933334.
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In an open label phase 2 study of 63 SSc patients with ILD, pirfenidone had no beneficial effects on disease outcomes.
“However, in an open label phase 2 study with 63 SSc patients with ILD, no beneficial effects of pirfenidone were observed on disease outcomes (187).”
From Unraveling SSc Pathophysiology; The Myofibroblast (2018) · cites this paper as [187]
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- Authors
- Khanna, D., Albera, C., Fischer, A., Khalidi, N., Raghu, G., Chung, L., Chen, D., Schiopu, E., Tagliaferri, M., Seibold, J. R., & Gorina, E.
- Affiliations
- University of Washington (US)
- ORCID
- Venue
- The Journal of Rheumatology
- Publisher
- The Journal of Rheumatology
- Published
- 2016-07-01
- Volume
- 43
- Issue
- 9
- Pages
- 1672-1679
- ISSN
- 0315-162X (ISSN-L)
- 1499-2752
- OpenAlex
- W2472891063
- Cited by
- 268
- References
- 31
- Citations by year
- 2026: 8 · 2025: 21 · 2024: 17 · 2023: 24 · 2022: 28 · 2021: 43 · 2020: 43 · 2019: 32 · 2018: 22 · 2017: 23 · 2016: 7
- FWCI
- 25.41
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Journal of Rheumatology (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- pirfenidone, systemic sclerosis-associated interstitial lung disease, drug tolerability, adverse events, dose titration, mycophenolate mofetil, treatment-emergent adverse events, idiopathic pulmonary fibrosis
- MeSH terms
- Adult, Aged, Anti-Inflammatory Agents, Non-Steroidal, Dose-Response Relationship, Drug, Drug Therapy, Combination, Female, Humans, Male, Middle Aged, Mycophenolic Acid, Pyridones, Scleroderma, Systemic, Treatment Outcome, Lung Diseases, Interstitial
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