Effect of treatment with iloprost with or without bosentan on nailfold videocapillaroscopic alterations in patients with systemic sclerosis

Mod Rheumatol. 2017 Jan;27(1):110-114. doi: 10.1080/14397595.2016.1192761. Epub 2016 Jun 16.


Introduction: Vascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis; it is responsible for some important clinical manifestations of the disease such as Raynaud's phenomenon and digital ulcers (DU). Bosentan, a dual receptor endothelin antagonist, and iloprost, often in combination therapy, seems to be able to interfere with the scleroderma microangiopathy.

Objectives: Aim of the study was to evaluate the effect of bosentan and iloprost on scleroderma microangiopathy, analyzed by means of capillaroscopic skin ulcer risk index (CSURI), in SSc patients treated for the prevention of DU.

Methods: Nailfold videocapillaroscopy (NVC) was performed in 95 SSc patients, treated with iloprost alone (group 1) or combination therapy with iloprost and bosentan (group 2), at baseline and after one year. In all patients CSURI was calculated according to the formula "diameter × number of megacapillaries/(total number of capillaries)2": in addition, total number of capillaries, giant capillaries, micro-hemorrhages, disorganization of the vascular array, and ramified capillaries were evaluated by means of a semiquantitative score.

Results: After 12 months, we observed a reduction of the number of giant capillaries in both groups, while an increase of ramified capillaries was recorded only in group 2. CSURI improved slightly in group 2 without statistical significance; on the contrary, in group 1 a significant worsening was recorded (p ≤ 0.001).

Conclusions: Our study confirms the effectiveness of bosentan, in combination with iloprost, in SSc microangiopathy observed to NVC. Moreover, the observed findings further support the role of CSURI in the evaluation and monitoring of SSc microangiopathy.

Keywords: Bosentan; CSURI; Iloprost; Microangiopathy; Systemic sclerosis; Videocapillaroscopy.

MeSH terms

  • Adult
  • Aged
  • Antihypertensive Agents / pharmacology
  • Antihypertensive Agents / therapeutic use*
  • Bosentan
  • Capillaries / diagnostic imaging
  • Capillaries / drug effects*
  • Drug Therapy, Combination
  • Female
  • Humans
  • Iloprost / pharmacology
  • Iloprost / therapeutic use*
  • Male
  • Microscopic Angioscopy
  • Middle Aged
  • Raynaud Disease / diagnostic imaging
  • Raynaud Disease / drug therapy*
  • Raynaud Disease / etiology
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / diagnostic imaging
  • Scleroderma, Systemic / drug therapy*
  • Sulfonamides / pharmacology
  • Sulfonamides / therapeutic use*
  • Treatment Outcome


  • Antihypertensive Agents
  • Sulfonamides
  • Iloprost
  • Bosentan