Gastrointestinal involvement in very early and established systemic sclerosis: insights from the SPRING-SIR national Italian registry

Abstract

Objectives: To describe the prevalence of gastrointestinal (GI) symptoms in SSc and Very Early Diagnosis of SSc (VEDOSS), identify clinical and serological features associated with GI involvement and explore a cranio-caudal pattern of symptom distribution, using data from the Italian SPRING-SIR registry.

Methods: This cross-sectional analysis included patients fulfilling 2013 ACR/EULAR SSc or VEDOSS criteria. GI involvement was defined as symptoms in at least one GI tract segment and categorized as upper and lower. Associations between GI involvement and clinical variables were assessed using logistic and ordinal regression models, adjusting for demographics, disease characteristics and autoantibodies.

Results: Among 1917 SSc patients, 56% had GI symptoms, associated with longer disease duration, dcSSc, interstitial lung disease (ILD), digital ulcers (DU), telangiectasias and tobacco exposure. Extensive GI involvement correlated with more severe disease. Ordinal regression identified female sex, dcSSc, ILD, DU, telangiectasias, tobacco exposure and anti-centromere antibodies as variables significantly associated with more extensive GI involvement. Disease duration did not show a significant association with GI symptom extent. Among 211 VEDOSS patients, 41.2% reported GI symptoms (mostly oesophageal), significantly associated with puffy fingers and dyspnoea. Among VEDOSS, puffy fingers and anti-centromere antibodies were independent predictors of presence of oesophageal symptoms.

Conclusion: GI involvement in SSc is linked to more severe disease and longer disease duration. Disease duration resulted linked to the presence of GI symptoms rather than extent of GI involvement. Puffy fingers and anti-centromere antibodies may associate with presence of early oesophageal symptoms in VEDOSS.

Keywords: VEDOSS; autoantibodies; disease progression; gastrointestinal involvement; oesophageal dysmotility; systemic sclerosis.

Graphical abstract

Figure 1.

Stratification of GI extent according to disease duration, representing the time from the first non-Raynaud’s phenomenon sign or symptom

Figure 2.

Regression models of determinants of presence, extent and distribution of GI involvement in SSc. Forest plots showing the multivariable logistic (A, C) and ordinal (B) regression models evaluating factors associated with (A) the presence of GI involvement, (B) the extent of GI involvement (increase in number of affected GI segments) and (C) the pattern of GI involvement (upper GI vs combined upper-and-lower GI involvement) in patients with SSc. The plot displays odds ratios (ORs) and 95% confidence intervals (CIs) for each covariate included in the model. All variables included in the models were categorical, except for age and disease duration (years)