High-resolution ultrasound of peripheral nerves in systemic sclerosis: a pilot study of computer-aided quantitative assessment of nerve density.

OBJECTIVES: To quantitatively evaluate and compare nerve density in patients with limited cutaneous systemic sclerosis (lcSSc) and control subjects using high-resolution ultrasound (US) with a computer-aided assessment. METHODS: Forty patients and 40 age- and sex-matched control subjects were prospectively enrolled. Ultrasound (US) examination (17-5 MHz probe) of the median nerve at the elbow was performed bilaterally by one radiologist. A software quantified the ratio between the hypoechoic and hyperechoic areas of peripheral nerves on ultrasound. Two observers set the threshold in the images acquired, and three observers performed the digital analysis of nerve density. Statistical analysis included Mann-Whitney U-test of patients versus control subjects and subgroup analysis of symptomatic and non-symptomatic patients. Intra and inter-observer agreement of the three observers were assessed with the kappa statistic. RESULTS: In all, 160 median nerves were evaluated. According to the US, nerve density was significantly reduced in lcSSc patients compared to control subjects (mean and standard deviation: 41 ± 3 vs 56 ± 4, p < 0.01). Subgroup analysis showed that symptomatic patients (n = 15) had reduced nerve density compared to non-symptomatic (n = 25) patients (39 ± 5 vs 43 ± 4, p < 0.01). Intra-observer agreement was very good (K = 0.82). Inter-observer agreements were good: reader 1 vs reader 2: k = 0.78 (95% confidence interval 0.65 to 0.91); reader 2 vs reader 3: k = 0.72 (95% confidence interval 0.65 to 0.82); reader 3 vs reader 1: k = 0.71 (95% confidence interval 0.64-0.81). CONCLUSIONS: In lcSSc patients, nerve density was reduced, especially in the symptomatic group, compared to control subjects.

Gastrointestinal motility disorder assessment in systemic sclerosis.

OBJECTIVES: SSc is a clinically heterogeneous and generalized disease, characterized by thickness of the connective tissue of the skin and internal organs, such as the digestive tract, impairing gastrointestinal (GI) motility. Our aim is to evaluate retrospectively abnormalities of oesophageal motility, gastric emptying, oro-cecal transit time (OCTT) and small intestine bacterial overgrowth (SIBO) in a large cohort of SSc patients. METHODS: Ninety-nine SSc patients were included in the study. Forty-two patients underwent oesophageal conventional manometry, 45 performed a [(13)C]octanoic acid breath test to measure gastric emptying time and all 99 patients performed a lactulose breath test in order to evaluate OCTT and SIBO. Data were compared with healthy controls. RESULTS: In SSc patients, median lower oesophageal sphincter (LOS) pressure [14 mmHg (25th-75th; 8-19) vs 24 mmHg (19-28); P < 0.01] and median wave amplitude [30 mmHg (16-70) vs 72 mmHg (48-96); P < 0.01] were lower than in controls. Oesophageal involvement, defined as reduced LOS pressure and ineffective oesophageal motility pattern, was encountered in 70% of SSc patients. A delayed gastric emptying time was present in 38% of SSc patients: mean t½ was 141 ± 79 min vs 90 ± 40 min of controls (P < 0.01). Also, OCTT was significantly delayed in SSc: median OCTT was 160 min (25th-75th; 135-180) vs 105 min (25th-75th; 90-135) of controls (P < 0.01). SIBO was observed in 46% of SSc compared with 5% of controls (P < 0.01). CONCLUSION: GI involvement is very frequent in SSc patients. Oesophagus and small bowel are more frequently impaired, whereas delayed gastric emptying is less common.