AB0690 USE OF MYCOPHENOLATE IN SYSTEMIC SCLEROSIS. CASE SERIES. (June 2019)

• Record Type: Journal Article
• Authors: TANDAIPAN JAIME, Jose Luis; Alonso, Elena Riera; Alvarez-Abella, Alba; Vilella, Mireia Castillo; Alarcon, Georgina Salvador; Pujol, Manel; Pardo, Silvia Martinez
• Is Part Of: Annals of the rheumatic diseases. Volume 78(2019)Supplement 2
• Page Start: 1805
• Page End: 1806
• Publication Date: 2019-06
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2019-eular.5372
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: At present, there are not specific targets to treat or prevent Systemic Sclerosis (SSc), but there are some to reduce the impact of its associated complications. Although mycophenolate (MP) is not recommended in latest EULAR SSc guidelines to treat cutaneous and pulmonary manifestations, its efficacy is known, based on observational studies and the Sclerodermalungstudy II. Objectives: To describe our center's experience in treating SSc patients with MP. Methods: Descriptive series of SSc patients treated with MP from November 2017 to December 2018. Demographic, clinical, analytical, spirometric, radiological (HR-CT) and modified Rodnan skin score (mRSS) data were collected. Results: Case 1 : woman diagnosed at 42 years old with diffuse SSc, having positive anti-toposiomerase-I antibody (ATA), telangiectasias, pitting scars, scleroderma and Non-specific interstitial Pneumonia (NSIP) without significant alterations in spirometry. One year after diagnosis, as a result of skin worsening (mRSS 21), we started MP in association with prednisone (mean dose 5 mg. A day). After treatment for 14 months, she showed significant improvement (mRSS 9) without spirometric changes. Case 2 : woman diagnosed at 30 years old with diffuse SSc, having positive aTA, pitting scars, gastroesophageal reflux disease (GERD) and scleroderma. This same year, we started MP due to skin involvement (mRSS 14), improving significantly (mRSS 9) after 14 months of treatment. Case 3 : woman diagnosed at 35 years old with diffuse SSc, having positive anti-RNA polymerase-III (ARA), pitting scars, telangiectasias, calcinosis, myopathy and scleroderma. 40 months after diagnosis, MP and prednisone (mean dose 5mg. A day) were started to treat skin (mRSS 19) and muscle (confirmed by biopsy) involvement. Both showed significant improvement after 8 months of treatment, with mRSS 13, myopathy symptoms resolution and creatine kinase (CK) normalization. Case 4 : a woman diagnosed at 35 years of age with limited SSc, having positive anti-Ro52, telangiectasias, skin involvement (mRSS 14) and NSIP with non-assessable spirometry. Clinical respiratory improvement was shown after just 1 month of treatment, without mRSS changes. Case 5 : woman diagnosed at 54 years old with diffuse SSc, having positive aTA, hands edema, scleroderma (mRss 11), GERD and NSIP (FVC 56%, FEV1 64%, non-assessable DLCO). After 25 months of disease progression and skin involvement, we started MP with prednisone (mean dose 8mg. A day), observing no significant changes after treating for 8 months on skin or lung disease, only improving the hands edema. Case 6 : woman diagnosed at 47 years old with diffuse SSc, having telangiectasias, scleroderma, positive aTA and NSIP. At 100 months from diagnosis, after 6 cyclophosphamide cycles followed by azathioprine (both ineffective) due to lung function (FVC 76%, FEV 83%, DLCO 26%) and skin (mRSS 30) worsening, MP and prednisone (mean dose 15mg. A day) were started. Currently, we are still waiting to assess clinical response. Conclusion: Despite it is not included in EULAR current recommendations for SSc complications, our patients have remarkably improved with MP, especially skin involvement, with a good safety profile. These data reaffirm those obtained in previous studies and encourages us to continue considering its use. Disclosure of interests: None declared