Diaphragmatic Muscle Thickness: A Complementary Tool in Assessing Lung Involvement in Patients with Systemic Sclerosis.

Background

Systemic sclerosis (SSc) is an autoimmune disease with interstitial lung disease (ILD) representing the leading cause of mortality. Diaphragmatic muscle impairment, which may contribute to respiratory dysfunction, is underexplored in SSc.

Objective

This study aimed to assess diaphragmatic thickness using HRCT in patients with SSc-ILD and to investigate whether this parameter correlates with disease severity and clinical outcomes.

Methods

We conducted a multicentric retrospective observational study that included 161 participants: 69 with SSc-ILD and 92 matched controls without pulmonary disease. Preliminary findings from this cohort were previously communicated as a conference abstract at EULAR 2024. Diaphragmatic thickness was measured on axial and coronal CT images at the celiac axis level by two independent radiologists. Clinical and functional parameters were collected, including forced vital capacity (FVC), diffusing capacity for carbon monoxide (DLCO), echocardiographic findings, and 6 min walking test performance.

Results

The left hemidiaphragm was significantly thinner in patients with SSc-ILD compared with controls (2.98 ± 1.04 mm vs. 3.44 ± 0.97 mm; p = 0.004), while the right hemidiaphragm showed a non-significant trend toward reduction (3.21 ± 1.09 mm vs. 3.52 ± 1.05 mm; p = 0.072). Thinning of the right hemidiaphragm correlated significantly with lower FVC (p < 0.05). ROC analysis identified an optimal left diaphragm cut-off of ≤3.115 mm (AUC = 0.635, 95% CI: 0.556-0.709, sensitivity = 66.7%, specificity = 60.9%). No associations were found with the autoantibody profile, disease duration, or treatment. Interobserver reliability was excellent (Bland-Altman mean difference -0.002 mm, p = 0.95).

Conclusions

HRCT-measured left hemidiaphragm thickness is significantly reduced in patients with SSc-ILD compared with controls, and right hemidiaphragm thickness correlates with impaired lung function. Although its discriminative performance is modest (AUC 0.635), this parameter may serve as a supplementary zero-burden addition to routine HRCT evaluation alongside pulmonary function tests. The retrospective cross-sectional design precludes conclusions about causality or prognosis; prospective studies incorporating concurrent functional diaphragm assessment are warranted.