PROVAL: A Comprehensive Protocol for Developing Validation Studies.

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.

LEARNING POINTS: This case report shows the efficacy of inhaled prostacyclin in the treatment of digital ulcers in systemic sclerosis as an equally effective, better tolerated, and safer alternative to the usual intravenous route.