Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
High-Dose I.v. N-Acetylcysteine Increases Peripheral Tissue Perfusion and Improves Clinical Symptoms of Systemic Sclerosis Patients. A Pilot Study.
Sulli, Alberto, Ruaro, Barbara, Zampogna, Giuseppe, Ferrone, Carmela, Ravera, Francesca, Seriolo, Bruno, Cutolo, Maurizio
Systemic sclerosis (SSc) is characterised by progressive tissue hypoperfusion and morphological anomalies of the microcirculation, inducing functional and organic clinical manifestations. This is a pilot study to evaluate clinical efficacy of a single i.v. cycle of N-acetylcisteine (NAC) treatment at a high dosage in patients affected by severe Raynaud's phenomenon (RP) secondary to SSc with digital ulcers and non-responders to either calcium channel blockers or prostacyclin.
Ten SSc patients (mean age 67±11SD years, mean disease duration 9±4SD years) with the aforementioned characteristics were enrolled into the study after having obtained written informed consent. At baseline and at 5 days after continuous i.v. NAC infusion (20 g/24 hrs/day for 5 days) the following evaluations were carried out: laser Doppler flowmetry (LDF), nailfold videocapillaroscopy (NVC), biochemical assessments (routine haematochemicals, homocysteine, osteocalcin, pyridinoline, deoxypyridinoline, telopeptide, factor VIII), along with clinical parameter assessment. Peripheral digital perfusion (PDP) was assessed by LDF at basal temperature and after heating the probe at 36°C analysing the central area of the fingertips from 2nd to 5th finger bilaterally (12). NVC was performed at the level of the same fingers to establish the pattern of microangiopathy and quantify the morphological anomalies (3). Both frequency and severity of the RP attacks, pain intensity tied to digital ulcers, and the subjective elasticity of the skin were evaluated by visual analogical scales (VASs). Statistical analysis was carried out by non-parametric Wilcoxon signed rank test.
Eight patients showed a "Late pattern" of microangiopathy at NVC, whilst two had an "Active pattern". A statistically significant increase of PDP was observed after NAC treatment in 8/10 patients, both at basal temperature (median from 72 to 95 PU) and at 36°C (median from 106 to 134 PU) (p=0.05). NAC administration did not interfere with capillary dilation capacity, as the magnitude of PDP rise was about 30% as at basal temperature assessment, as at 36°C. No change was observed in the score for the NVC parameters during the brief observation period. The blood concentrations of telopeptide and factor VIII showed statistically significant increase (p<0.05), whilst there was a statistically significant decrease in concentrations of homocysteine, osteocalcin, and phosphorus (p<0.05). When VASs were analysed, there was a notable reduction in both the frequency and severity of RP attacks when compared to pre-treatment values, as was the case for intensity of pain due to ulcers and the subjective cutaneous elasticity (p=0.04). The treatment was well tolerated and no side effects were observed.
Continuous i.v. infusion of high-dose NAC increases peripheral tissue perfusion, induces modifications in some biochemical parameters and improves clinical symptoms in SSc patients, possibly by interfering with the fibrotic skin process.
To cite this abstract, please use the following information:
Sulli, Alberto, Ruaro, Barbara, Zampogna, Giuseppe, Ferrone, Carmela, Ravera, Francesca, Seriolo, Bruno, et al; High-Dose I.v. N-Acetylcysteine Increases Peripheral Tissue Perfusion and Improves Clinical Symptoms of Systemic Sclerosis Patients. A Pilot Study. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1473