Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients
Yıl: 2020 Cilt: 35 Sayı: 2 Sayfa Aralığı: 180 - 188 Metin Dili: İngilizce DOI: 10.46497/ArchRheumatol.2020.7381 İndeks Tarihi: 13-10-2020
Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients
Öz: Objectives: This study reports a low dose combination therapy of cyclophosphamide (CYC) and pirfenidone (PFD) and the efficiency and safety ofthe therapy in refractory connective tissue disease associated interstitial lung disease (CTD-ILD) patients.Patients and methods: The study included seven CTD-ILD patients (2 males, 5 females; mean age 48.8 years; range, 32 to 63 years) treated betweenJanuary 2016 and December 2017 in our clinic. At enrolment, all patients had shown no improvement in their symptoms (dyspnea or cough) after atleast one month of high dose steroids treatment. Patients who had received adjusted immunosuppressive agents other than steroids or anti-fibroticmedications within the three months before enrolment were excluded. We changed the treatment to a low dose combination of CYC 0.4 g/m2monthly and PFD 300 mg twice per day and quickly reduced the steroids. All the patients were followed-up for 12 months.Results: Two patients had anti-synthetase syndrome, two had Sjögren syndrome, two had scleroderma and one had mixed connective tissuedisease. The baseline forced vital capacity (FVC) was 39-81% and the six-minute walk distance (6MWD) was 202 m-324 m. Within 12 months followup,the median improvement in the FVC was 13.4% (range, 0-35.9%), the median improvement of carbon monoxide diffusing capacity was 6.3%(range, 1.7-16%) and the median improvement of 6MWD was 52.7% (range, 34.4-86.3%). All the patients were self-sufficient, and their dyspnea,chest high-resolution computed tomography scores, and quality of life improved simultaneously. Exceeding our expectations, no adverse eventsassociated with CYC or PFD were observed during the follow-up period.Conclusion: Our study provided preliminary while promising clinical evidence for combination therapy of CYC-PFD for CTD-ILD. A low dosecombination of CYC and PFD was unexpectedly well tolerated, with satisfactory effects in refractory CTD-ILD patients. Well-designed controlledstudies are needed to further establish the safety and efficacy of this approach.
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| APA | Shen L, Yan Q, Chen X (2020). Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. , 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| Chicago | Shen Lichong,Yan Qingran,Chen Xiaoxiang Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. (2020): 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| MLA | Shen Lichong,Yan Qingran,Chen Xiaoxiang Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. , 2020, ss.180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| AMA | Shen L,Yan Q,Chen X Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. . 2020; 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| Vancouver | Shen L,Yan Q,Chen X Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. . 2020; 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| IEEE | Shen L,Yan Q,Chen X "Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients." , ss.180 - 188, 2020. 10.46497/ArchRheumatol.2020.7381 |
| ISNAD | Shen, Lichong vd. "Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients". (2020), 180-188. https://doi.org/10.46497/ArchRheumatol.2020.7381 |
| APA | Shen L, Yan Q, Chen X (2020). Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. Archives of Rheumatology, 35(2), 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| Chicago | Shen Lichong,Yan Qingran,Chen Xiaoxiang Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. Archives of Rheumatology 35, no.2 (2020): 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| MLA | Shen Lichong,Yan Qingran,Chen Xiaoxiang Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. Archives of Rheumatology, vol.35, no.2, 2020, ss.180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| AMA | Shen L,Yan Q,Chen X Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. Archives of Rheumatology. 2020; 35(2): 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| Vancouver | Shen L,Yan Q,Chen X Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients. Archives of Rheumatology. 2020; 35(2): 180 - 188. 10.46497/ArchRheumatol.2020.7381 |
| IEEE | Shen L,Yan Q,Chen X "Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients." Archives of Rheumatology, 35, ss.180 - 188, 2020. 10.46497/ArchRheumatol.2020.7381 |
| ISNAD | Shen, Lichong vd. "Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients". Archives of Rheumatology 35/2 (2020), 180-188. https://doi.org/10.46497/ArchRheumatol.2020.7381 |
