Clinical Characteristics, Imaging Patterns and Management in Male and Female Patients with Primary Sjögren's Syndrome-associated Interstitial Lung Disease.

Abstract

Introduction: The influence of sex on primary Sjögren's disease-associated interstitial lung disease (pSjD-ILD) remains poorly understood. Therefore, we aimed to investigate the sex-related differences in the clinical characteristics and outcomes of pSjD-ILD.

Methods: Consecutive patients with pSjD-ILD in Nanjing University Affiliated Drum Tower Hospital from November 2007 to November 2022 were enrolled. Demographics, comorbidities, laboratory parameters, imaging patterns, pulmonary function tests, and treatments were compared between male and female patients. Kaplan-Meier method was used to assess survival. Cox proportional hazards model was employed to identify prognostic factors.

Results: A total of 125 male and 433 female patients were enrolled. Male patients had a higher prevalence of pulmonary hypertension (33.0% vs 20.6%, p = 0.014), overall cancer (14.4% vs 6.7%, p = 0.006), and radiological usual interstitial pneumonia (UIP, 28.0% vs 16.2%, p = 0.003). They also had higher usage of antifibrotic drugs (24.8% vs 16.2%, p = 0.027). In contrast, female patients had higher positive rates for antinuclear antibody (83.6% vs 73.6%, p = 0.011) and anti-Ro52 antibody (59.8% vs 44.8%, p = 0.003). No differences existed in the total ESSDAI score between male and female patients. Follow-up data showed that male patients had higher 3-year (32.3% vs 18.0%, p = 0.003) and 5-year mortality (52.4% vs 35.0%, p = 0.006) compared to female patients. Multivariate Cox model indicated that age (HR 1.058, p < 0.001) and UIP (HR 1.874, p = 0.009) were significant prognostic factors.

Conclusions: In pSjD-ILD, male patients tend to have more severe pulmonary manifestations, higher frequencies of pulmonary hypertension and cancer, and poorer outcomes compared to female patients. Key points • Male pSjD-ILD patients were more likely to suffer from pulmonary hypertension and cancer than their female counterparts. • Radiological UIP pattern was more frequently observed in male patients with pSjD-ILD than in female patients. • Male patients with pSjD-ILD had higher 3-year and 5-year mortality than female patients.