title: Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre.
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).
  
  
  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.
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Gemma
authors.initials: G
authors.lastname: Lepri
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Guiducci
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Bellando-Randone
authors.affiliation: General Surgery, USL-8 Arezzo, Arezzo, Italy.
authors.forename: Iacopo
authors.initials: I
authors.lastname: Giani
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Cosimo
authors.initials: C
authors.lastname: Bruni
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Jelena
authors.initials: J
authors.lastname: Blagojevic
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Carnesecchi
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Radicati
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Filippo
authors.initials: F
authors.lastname: Pucciani
authors.affiliation: Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
authors.forename: Matucci-Cerinic
authors.initials: MC
authors.lastname: Marco
mesh_terms: D000328:Adult; D001003:Anal Canal / Q000503:physiopathology; D001004:Anus Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D042241:Early Diagnosis; D004935:Esophageal Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D049630:Esophageal Sphincter, Lower / Q000503:physiopathology; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008171:Lung Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008365:Manometry; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011859:Radiography; D011928:Raynaud Disease / Q000209:etiology; D012002:Rectal Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology
keywords: Autoantibodies; Disease Activity; Pulmonary Fibrosis; Systemic Sclerosis