In individuals with systemic sclerosis (SSc), an association has been observed between motor esophageal disorders and pulmonary involvement, according to findings from a retrospective, longitudinal analysis conducted in Genoa, Italy, and published in the journal Rheumatology International.

SSc is a rare autoimmune disorder of connective tissue that can affect multiple organs, including the gastrointestinal tract, lungs, heart, skin, kidneys, and musculoskeletal system. Two distinct types of SSc have been identified: limited cutaneous SSc (lcSSc), which is a thickening of the skin is distal to the elbows, knees, and face (the trunk is spared), and diffuse cutaneous SSc (dcSSc), which is a thickening of the skin is proximal, with extension to the trunk.

Although both types of SSc can involve internal organs, individuals with dcSSc experience the greatest likelihood of such involvement. The esophagus is the most affected organ in the gastrointestinal tract, with 50% to 90% of patients reporting involvement. The primary manifestations of esophageal involvement include aperistalsis and ineffective esophageal motility (IEM). Individuals frequently experience symptoms of gastroesophageal reflux disease, including regurgitation, heartburn, and noncardiac-related chest pain or dysphagia resulting from impaired motility.

Further, interstitial lung disease is a key feature of SSc and is a diagnostic criterion of the disorder. The investigators of the current study sought to assess the association and prognostic implication between motor esophageal disorders on high-resolution manometry (HRM), based on the latest Chicago Classification 4.0, and pulmonary involvement among patients with SSc.

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All participants had received a referral to the Digestive Pathophysiology Outpatient Section of the Gastroenterological Clinic of the IRCCS San Martino Polyclinic Hospital in Genoa from 2009 to 2021. Patients underwent HRM and pulmonary assessments, which included pulmonary function tests and high-resolution computed tomography (HrCT) scans with the Warrick score. A total Warrick HrCT score of 7 or more was predictive of interstitial lung disease, whereas a score of less than 10 was indicative of interstitial involvement with a specificity of 100%.

A cohort of 42 participants was enrolled in the study. The mean patient age was 64 years (range, 36-91 years). Patients’ mean body mass index was 23 kg/m2 (range, 17-31 kg/m2). Among the participants, 39 of the 42 individuals were nonsmokers. Overall, 67% (28 of 42) of the patients had dcSSc and 33% (14 of 42) of them had lcSSc.

Results of the study showed that 54.77% (23 of 42) of the participants had a CT score of 7 or more and 45.23% (19 of 42) of patients had a CT score of less than 7. On HRM, 33.33% (14 of 42) of participants had an absent contractility, 19.04% (8 of 42) of them had an IEM, and 47.62% (20 of 42) of patients had normal contractility.

A Warrick score of 7 or greater was reported in 11 individuals with aperistalsis, 6 with IEM, and 6 with normal HRM. Further, a score of less than 7 was seen in 3 participants with aperistalsis, in 2 with IEM, and in 14 with normal contractility. Distal contractile integral and HrCT scores were inversely correlated in linear (P =.002) and logarithmic (P =.001) regression analyses. At follow-up, a significant association was observed between lower baseline esophageal sphincter pressure and higher CT scores (P <.0001).

“Future prospective studies are necessary to confirm [these] correlation[s] and to find tailored therapeutic strategies in order to improve clinical outcomes in patients with SSc,” the researchers concluded.


Canavesio YM, Pasta A, Calabrese F, et al. Association between esophageal motor disorders and pulmonary involvement in patients affected by systemic sclerosis: a retrospective study. Rheumatol Int. Published online August 5, 2023. doi:10.1007/s00296-023-05399-y