DOI: 10.12890/2025_004913

Inhaled iloprost for digital necrosis in systemic sclerosis – an effective alternative

Authors

  • Jaouad Yousfi Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Soukaina Alami Idrissi Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Fatima Dakir Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Zineb Bott Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Chadyne Taouil Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Mouna Zahlane Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco
  • Lamiaa Essaadouni Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco

soukalid@gmail.com

Keywords

Systemic sclerosis, digital ulcers, necrosis, inhaled prostacyclin

Abstract

Introduction: Vascular issues in systemic sclerosis (SSc), notably Raynaud’s phenomenon, can lead to digital ulcers (DU) and necrosis, causing pain and infections. Intravenous prostacyclin (iloprost) is used for established DUs. We report a case where severe Raynaud’s phenomenon revealed SSc sine scleroderma, which improved with inhaled iloprost.
Case description: A 52-year-old former smoker with protein C deficiency, on anticoagulation since 2016 for a previous deep vein thrombosis, presented with severe Raynaud’s phenomenon and pulp necrosis of the third right finger. The capillaroscopy revealed megacapillaries suggestive of a connective tissue disease, and antinuclear antibodies were positive for centromere fluorescence. Doppler ultrasound and cardiopulmonary assessments were normal. The patient underwent necrosectomy but had ischemic pain and cyanosis post-surgery. Inhaled iloprost (2 ampoules of 10 mg/10 ml daily in 3 cycles of 5 days) led to significant clinical improvement.
Conclusion: Inhaled iloprost could be an effective and better-tolerated alternative to intravenous iloprost for treating SSc DUs. Further studies are needed to confirm this potential.

View the entire article

Downloads

References

  1. Lok C, Mouthon L, Ségard M, Richard MA, Guillevin L. Les ulcères digitaux de la sclérodermie systémique. Ann Dermatol Venereol 2011;138:762-768.
  2. Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR scleroderma trials and research group (EUSTAR). Ann Rheum Dis 2009;68:620-628.
  3. Pakozdi A, Howell K, Wilson H, Fox S, Gonzalez L, Black CM, et al. Inhaled iloprost for the treatment of Raynaud’s phenomenon. Clin Exp Rheumatol 2008;26:709.
  4. Chen KC, Kan P, Liang HF. AB1166 EXTENDED RELEASE OF INHALED ILOPROST LIPOSOME (L608) FOR TREATMENT OF SYSTEMIC SCLEROSIS-RELATED RAYNAUD PHENOMENA AND DIGITAL ULCER. Annals of the Rheumatic Diseases 2024;83:1916.
  5. Fries R, Shariat K, von Wilmowsky H, Böhm M. Sildenafil in the treatment of Raynaud’s phenomenon resistant to vasodilatory therapy. Circulation 2005;112:2980-2985.
  6. Korn JH, Mayes M, Matucci-Cerinic M, Rainisio M, Pope J, Hachulla E, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004;50:3985-3993.
  7. Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P, 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;70:32-38.

Published

2025-03-19

Issue

Vol. 12 No. 4 (2025)

Categories

How to Cite

1.
Yousfi J, Alami Idrissi S, Dakir F, Bott Z, Taouil C, Zahlane M, et al. Inhaled iloprost for digital necrosis in systemic sclerosis – an effective alternative. EJCRIM [Internet]. 2025 Mar. 19 [cited 2026 Nov. 4];12(4). Available from: https://ejcrim.com/index.php/ejcrim/article/view/4913

Most read articles by the same author(s)