Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis-related pulmonary artery hypertension and cutaneous vascular complications

Rheumatol Int. 2013 Apr;33(4):1047-52. doi: 10.1007/s00296-012-2466-5. Epub 2012 Jul 26.

Abstract

Pulmonary artery hypertension (PAH) remains the leading cause of morbidity and mortality in systemic sclerosis, while Raynaud's phenomenon and digital ulcers significantly add to the morbidity in systemic sclerosis (SSc). This study was undertaken to evaluate the role of sildenafil in PAH, Raynaud's phenomenon, and digital ulcers in systemic sclerosis patients. A prospective, open-label, uncontrolled pilot study was done at a tertiary care centre in India to study the safety and efficacy of oral sildenafil in PAH, Raynaud's phenomenon, digital infarcts, and ulcers in SSc. Seventeen patients fulfilling ACR classification criteria for scleroderma and having PAH were recruited. Six-minute walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and 2D ECHO were performed in all the study subjects at baseline and at 3 months post-treatment. All patients were treated with oral sildenafil 25 mg three times a day for a period of 3 months. The pre- and post-treatment values of mean pulmonary artery pressure (PAP), 6-min walk test, WHO class of dyspnoea, and severity of Raynaud's phenomenon were compared to look for any significant change. Sixteen patients who completed 3-month follow-up had shown statistically significant improvement in 6-min walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and mPAP. Also, there was no occurrence of new digital infarcts or ulcers, and existing ulcers showed signs of healing. Sildenafil is highly efficacious cheaper and safe alternative to other available therapies for SSc-associated PAH, Raynaud's phenomenon, and digital infarcts/ulcers.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Arterial Pressure / drug effects
  • Arterial Pressure / physiology
  • Female
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Middle Aged
  • Pilot Projects
  • Piperazines / administration & dosage
  • Piperazines / adverse effects
  • Piperazines / therapeutic use*
  • Prospective Studies
  • Pulmonary Artery / physiopathology
  • Purines / administration & dosage
  • Purines / adverse effects
  • Purines / therapeutic use
  • Raynaud Disease / drug therapy*
  • Raynaud Disease / etiology
  • Raynaud Disease / physiopathology
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / physiopathology
  • Sildenafil Citrate
  • Skin / blood supply
  • Skin / physiopathology
  • Skin Ulcer / drug therapy*
  • Skin Ulcer / etiology
  • Skin Ulcer / physiopathology
  • Sulfones / administration & dosage
  • Sulfones / adverse effects
  • Sulfones / therapeutic use*
  • Treatment Outcome
  • Vasodilator Agents / administration & dosage
  • Vasodilator Agents / adverse effects
  • Vasodilator Agents / therapeutic use*

Substances

  • Piperazines
  • Purines
  • Sulfones
  • Vasodilator Agents
  • Sildenafil Citrate