Retrospective Analysis of Patients with Systemic Sclerosis Using Bosentan for Digital Ulcer: A Ten-Year Single Center Experience

Year 2023, Volume: 49 Issue: 1, 37 - 41, 09.06.2023

Ali Ekin , Belkıs Nihan Coşkun , Burcu Yağız , Hüseyin Ediz Dalkılıç , Yavuz Pehlivan

https://doi.org/10.32708/uutfd.1213513

Abstract

The risk of ischemic digital ulcer (DU) development is high in systemic sclerosis (SS). The use of bosentan, an endothelin receptor antagonist, is recommended in the treatment of DU secondary to SS. In our study, we aimed to raise awareness of clinicians about the side effects that clinicians may encounter as a result of bosentan use and its management by presenting long-term data of patients who used bosentan in the treatment of DU, which is difficult to manage in patients with SS. The most common side effects that develop during bosentan treatment are headache, dizziness, cough, dyspnea, syncope, flushing, elevated liver enzymes and palpitations. In our study, safety data of bosentan were analyzed by evaluating side effects. Of the 25 patients included in the study, 24 (96%) were female, the median age was 54 years and the mean follow-up period was 15.16± 9.01 years. All patients had used at least one vasodilator drug for the treatment of DU. All patients used calcium channel blockers and pentoxifylline, 23 (92%) patients used iloprost and 11 (44%) patients used sildenafil and/or tadalafil. There were five (20%) patients who died during follow-up while on bosentan for reasons not related to drug use. No side effects were observed in these five patients during follow-up. In five patients, the drug was discontinued for different reasons. One of them was discontinued because of pregnancy and the other one was discontinued voluntarily because she said she felt unwell. In three patients, the drug was discontinued because of palpitations in one, headache in one and elevated aminotransferase in the other. The use of bosentan may reduce the development of DU when used alone or in combination with other drugs. The most important point for a clinician in terms of safety while using bosentan is the side effects that may develop and their management. In our study, there was one patient with headache, aminotransferase elevation and palpitations. This study emphasizes that clinicians should be more careful in terms of side effects when using bosentan for digital ulcers that require difficult and laborious treatment management.

Keywords

Sistemik sklerozis, bosentan, dijital ülser, romatoloji

Sistemik Sklerozis Tanılı ve Dijital Ülser Nedeniyle Bosentan Kullanan Hastaların Retrospektif İncelenmesi: On Yıllık Tek Merkez Deneyimi

Abstract

Sistemik sklerozis (SS)’te iskemik dijital ülser (DÜ) gelişimi riski yüksektir. SS’a ikincil gelişen DÜ tedavisinde endotelin reseptör antagonisti olan bosentan kullanımı önerilmektedir. Çalışmamızda SS tanılı hastalarda görülen, tedavi yönetimi zor olan DÜ tedavisinde bosentan kullanan hastaların uzun dönem verilerini sunarak, klinisyenlerin bosentan kullanımı sonucu karşılaşabilecekleri yan etkiler ve yönetimiyle ilgili farkındalık oluşturmayı amaçladık. Bosentan tedavisi sırasında gelişen en sık yan etkiler baş ağrısı, baş dönmesi, öksürük, nefes darlığı, senkop, flushing, karaciğer enzim yüksekliği ve çarpıntıdır. Çalışmamızda bosentanın güvenlik verileri yan etkiler üzerinden değerlendirilerek incelendi. Çalışmaya alınan 25 hastanın 24’ü (%96) kadın olup, medyan yaş 54 yıl, hastalık ortalama takip süresi 15.16± 9.01 yıldı. Hastaların tamamı DÜ tedavisi için en az bir vazodilatatör ilaç kullanmıştı. Tüm hastaların kalsiyum kanal blokeri ve pentoxifilin kullanımı, 23 (%92) hastanın iloprost, 11 (%44) hastanın ise sildenafil ve/veya tadalafil kullanımı vardı. Bosentan kullanırken takip süresince ilaç kullanımına bağlı olmayan sebeplerden exitus olan beş (%20) hasta vardı. Bu beş hastanın takipleri süresince yan etki gözlenmedi. Beş hastada ise farklı nedenlerle ilaç kesildi. Biri gebelik nedeniyle, diğeri ise kendini kötü hissettiğini söylediğinden kendi isteğiyle ilaç kesildi. Üç hastanın birinde çarpıntı, birinde baş ağrısı ve diğerinde ise aminotransferaz yüksekliği nedeniyle ilaç kesildi. Bosentan kullanımı DÜ gelişimini tek başına ya da diğer ilaçlarla kombine kullanıldığında azaltabilmektedir. Bosentanı kullanırken bir klinisyen için güvenlik açısından en önemli nokta gelişebilecek yan etkiler ve bunların yönetimidir. Çalışmamızda baş ağrısı, aminotransferaz yüksekliği ve çarpıntı görülen birer hasta vardı. Bu çalışmayla klinisyenlerin zor ve zahmetli bir tedavi yönetimi gerektiren dijital ülserler için bosentan kullanımında yan etkiler açısından daha dikkatli davranmaları gerektiği vurgulanmıştır.

Keywords

Sistemik sklerozis, bosentan, dijital ülser, romatoloji

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