Patients with Very Early Diagnosis of Systemic Sclerosis (VEDOSS) Present Esophageal and Anorectal Involvement: Data From a Single Centre

Gemma Lepri¹, Silvia Bellando-Randone², Serena Guiducci³, Iacopo Giani⁴, Cosimo Bruni⁵, Giulia Carnesecchi², Jelena Blagojevic², Alessandra Radicati², Filippo Pucciani⁶ and Marco Matucci Cerinic², ¹Department of Internal Medicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy, ²Department of Biomedicine, Division of Rheumatology, University of Florence, Florence, Italy, ³Department of Biomedicine, Division of Rheumatology AOUC, Excellence Centre for Research, Florence, Italy, ⁴General Surgery, ASL 8, Arezzo, Italy, Arezzo, Italy, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶General and Urgency Surgery, University of Florence, Florence, Italy, Florence, Italy

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Systemic sclerosis

Session Information

Session Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s – Clinical Aspects and Therapeutics

Session Type: Abstract Submissions (ACR)

Background/Purpose: Systemic Sclerosis (SSc) affects gastrointestinal tract in more than 80% of patients. Esophageal involvement is the most common manifestation with a prevalence ranging between 50-90%, followed by anorectal involvement (prevalence of 50-70%) that had a high impact on patients quality of life. Objectives of the study: evaluation of esophageal and anorectal involvement and of their correlations in very early SSc patients.

Methods: 56 VEDOSS patients (55 females), mean age 49,2 ± 14, were evaluated with esophageal and anorectal manometry. The demographic data, esophageal and anorectal symptoms (dysphagia, typical GERD symptoms and fecal incontinence and constipation), Raynaud phenomenon (presence/absence, duration) autoantibodies profile (anticentromere antibodies [ACA], antinuclear antibodies [ANA], anti-Scl70 [Scl70]), videocapillaroscopy patterns (Normal, Early, Active, Late), puffy fingers, digital ulcers were recorded for all patients.

Results:

Esophageal body dysmotility (absence of peristalsis or abnormal mean pressure of peristalsis) was present in 49 patients (94,2%) and it was associated with an hypotensive lower esophageal sphincter (LES) in 26 (53,1%). Anorectal manometry was abnormal in 85% of patients and in all these patients an esophageal involvement was found (absence of peristalsis in 29% of patients and an abnormal peristalsis in 67,7% of patients). Esophageal symptoms were present in 26 patients (50%). 22 patients (42,3%) showed puffy fingers that were associated with a smaller area of LES (p-value: 0,011). Only five patients (12,5%) complained anorectal symptoms. In 4 patients esophageal manometry was not performed because of scarce tolerance of the procedure and in 16 patients anorectal manometry was not performed for the same reason.

Conclusion: In VEDOSS patients esophageal and anorectal disorders are frequently detected even in asymptomatic patients. Our data showed that VEDOSS is characterized by simultaneous esophageal and anorectal involvement. Esophageal disorders seem to correlate with the presence of puffy fingers.

Disclosure:

G. Lepri,
None;

S. Bellando-Randone,
None;

S. Guiducci,
None;

I. Giani,
None;

C. Bruni,
None;

G. Carnesecchi,
None;

J. Blagojevic,
None;

A. Radicati,
None;

F. Pucciani,
None;

M. Matucci Cerinic,
None.

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