ABSTRACT

Conclusion:

Bosentan, used for the treatment of digital ulcers in SS patients, has been shown to have an acceptable side-effect profile.

Results:

A total of 30 patients were included in the study. The mean age of SS diagnosis was 37.5±14.7 years and 25 of these patients were female. The distribution of disease patterns was limited cutaneous for 25 patients and diffuse cutaneous for 5 patients. The median length of bosentan treatment was 31.0±23.9 months. Five patients required the discontinuation of bosentan due to adverse events including toxic hepatitis (2 patients) and headache (3 patients). Also, 3 patients discontinued the treatment on their own, despite the absence of adverse effects or toxicity.

Methods:

SS patients with digital ulcers, who had a follow-up period in outpatient and inpatient clinics of Rheumatology Department, were included in our study. Duration of treatment and drug-related side effects were retrieved from the patient’s files. For the patients who did not come to the clinic in the last 6 months, visits were made over the phone.

Objective:

Digital ulcers related to systemic sclerosis (SS) are associated with disability and mortality in the progression of the disease. In our study, we investigated the duration of treatment and drug-related side effects on patients, who were started bosentan for the treatment of digital ulcers.

References

1Guiducci S, Giacomelli R, Cerinic MM. Vascular complications of scleroderma. Autoimmun Rev 2007;6:520-3.

2Chung L, Fiorentino D. Digital ulcers in patients with systemic sclerosis. Autoimmun Rev 2006;5:125-8.

3Ingraham KM, Steen VD. Morbidity of digital tip ulcerations in scleroderma [abstract]. Arthritis Rheum 2006;54(Suppl 9):P578.

4Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford) 2009;48(Suppl 3):iii19-iii24.

5Mihai C, Landewé R, van der Heijde D, et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 2016;75:681-6.

6Tingey T, Shu J, Smuczek J, Pope J. Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken) 2013;65:1460-71.

7Hachulla E, Coghlan JG. A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann Rheum Dis 2004;63:1009-14.

8Korn JH, Mayes M, Matucci Cerinic M, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004;50:3985-93.

9Matucci-Cerinic M, Denton CP, Furst DE, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 2011;70:32-8.

10Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017;76:1327-39.

11Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346:896-903.

12Humbert M, Segal ES, Kiely DG, Carlsen J, Schwierin B, Hoeper MM. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J 2007;30:338-44.

13Agard C, Carpentier PH, Mouthon L, et al. Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval. Scand J Rheumatol 2014;43:398-402.

14Roman Ivorra JA, Simeon CP, Alegre Sancho JJ, et al. Bosentan in clinical practice for treating digital and other ischemic ulcers in Spanish patients with systemic sclerosis: IBER-DU cohort study. J Rheumatol 2011;38:1631-5.

15van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013;72:1747-55.

16LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988;15:202-5.

Evaluation of the follow-up and drug-related side effects of bosentan treatment for the treatment of systemic sclerosis related digital ulcer: a real life experience

ABSTRACT

References