Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre

Ann Rheum Dis. 2015 Jan;74(1):124-8. doi: 10.1136/annrheumdis-2013-203889. Epub 2013 Oct 15.

Abstract

Background: The oesophagus is the first gastrointestinal (GI) tract involved in systemic sclerosis (SSc), followed by the anorectum.

Objective: Evaluation of oesophageal and anorectal involvement and their correlations in patients with very early diagnosis of SSc (VEDOSS).

Patients and methods: 59 patients with VEDOSS, evaluated with oesophageal and anorectal manometry and investigated with lung function tests and chest HRCT. Demographic data, oesophageal and anorectal symptoms, Raynaud's phenomenon, autoantibodies, videocapillaroscopy patterns, puffy fingers and digital ulcers were recorded for all patients.

Results: In 4 patients oesophageal manometry and in 17 patients anorectal manometry was not performed because of scarce tolerance. Oesophageal peristalsis was absent in 14 patients; its pressure and speed were significantly lower in 41 patients (p<0.001 and p=0.005, respectively). The maximum pressure and mean pressure (Pmax and Pm) of lower oesophageal sphincter were significantly lower (p=0.012 and p=0.024, respectively). Patients with a diffusing capacity of the lung for carbon monoxide<80% presented a hypotonic lower oesophageal sphincter (p=0.008) and an abnormal peristalsis (p<0.001); patients with a diffusing capacity of the lung for carbon monoxide>80% showed only an abnormal peristalsis (<0.001). The anal resting pressure (ARP) at 4.3 cm and 2 cm from anal edge and the anal canal Pm were significantly decreased (p<0.001 and p=0.010, respectively). The maximum voluntary contraction was significantly abnormal in its Pmax and Pm (p=0.017 and p=0.005) and in its duration (p=0.001). In patients with a positive HRCT, the ARP and the canal Pmax and Pm were significantly lower; patients with negative HRCT presented only an abnormal ARP.

Conclusions: In patients with VEDOSS, oesophageal and anorectal disorders are frequently detected, showing that very early SSc is characterised by GI involvement.

Keywords: Autoantibodies; Disease Activity; Pulmonary Fibrosis; Systemic Sclerosis.

MeSH terms

  • Adult
  • Anal Canal / physiopathology
  • Anus Diseases / diagnosis*
  • Anus Diseases / etiology
  • Anus Diseases / physiopathology
  • Early Diagnosis
  • Esophageal Diseases / diagnosis*
  • Esophageal Diseases / etiology
  • Esophageal Diseases / physiopathology
  • Esophageal Sphincter, Lower / physiopathology
  • Female
  • Humans
  • Lung / diagnostic imaging*
  • Lung / physiopathology
  • Lung Diseases / diagnosis*
  • Lung Diseases / etiology
  • Lung Diseases / physiopathology
  • Male
  • Manometry
  • Microscopic Angioscopy
  • Middle Aged
  • Radiography
  • Raynaud Disease / etiology
  • Rectal Diseases / diagnosis
  • Rectal Diseases / etiology
  • Rectal Diseases / physiopathology
  • Respiratory Function Tests
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / diagnosis*
  • Scleroderma, Systemic / physiopathology