Characteristics and mortality in primary Sjögren syndrome–related interstitial lung disease

Abstract

Aim of the study was to determine the characteristics and prognosis, and to identify the risk factors for mortality in patients with primary Sjögren syndrome (pSS) with interstitial lung disease (pSS-ILD). A total of 1422 patients with SS were screened and 178 patients with pSS-ILD were recruited. The medical records and outcomes were retrospectively reviewed. Overall survival and case control study were performed to explore the predictors of death. Among 178 pSS-ILD patients, 87.1% were women. Mean age was 61.59 ± 11.69-year-old. Median disease duration was 72.0 (24.0, 156.0) months. Nonspecific interstitial pneumonia was the predominant high-resolution computed tomography pattern (44.9%). Impairment in diffusion capacity was the most common abnormality of pulmonary function test (75.8%) and the most severe consequence. Type 1 respiratory failure and hypoxia were observed in 15.0% and 30.0% patients, respectively. Mean survival time after confirmation of pSS-ILD diagnosis was 9.0 (6.8, 13.0) years. The 10-year survival rate for all patients with pSS-ILD was 81.7%. Forty-four (24.7%) of 178 patients died during the follow-up period. The most predominant cause of death was respiratory failure (n = 27). Twenty-seven patients died of ILD and formed study group. The 78 patients who survived formed control group. Age and smoking were risk factors for mortality in patients with pSS-ILD. In addition, severity of ILD, as reflected by high-resolution computed tomography, pulmonary function test, and arterial blood gas, was an independent risk factor. However, inflammation status (erythrocyte sedimentation rate, C-reactive protein) and anti-Sjögren syndrome–related antigen A and anti-Sjögren syndrome–related antigen B were not. ILD is a severe complication of pSS. Age, smoking, and severity of lung involvement are more critical for prognosis rather than inflammation status and autoantibodies.

Interstitial Lung Disease and Pulmonary Damage in Primary Sjögren’s Syndrome: A Systematic Review and Meta-Analysis

“…Eleven studies had a prospective design, and among them four were conducted in Greece [ 17 , 18 , 19 , 20 ], three in Sweden [ 21 , 22 , 23 ], one in Spain [ 24 ], one in the Netherlands [ 25 ], two in Italy [ 26 , 27 ], and one in China [ 28 ]. Nineteen studies had a retrospective design, and among them ten were conducted in China [ 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 ], three in Turkey [ 38 , 39 , 40 ], two in France [ 41 , 42 ], one in Italy [ 26 ], one in the Netherlands [ 43 ], one in Japan [ 44 ], one in Spain [ 45 ], and one in Germany [ 46 ]. Many of them referred to pSS patients fulfilling the revised criteria proposed by the American–European Consensus Group [ 47 ].…”

“…Many of them referred to pSS patients fulfilling the revised criteria proposed by the American–European Consensus Group [ 47 ]. In 23 studies out of the 30 included HRCT was performed to investigate lung involvement [ 19 , 20 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 ]. The main characteristics of the selected studies are reported in Table 1 .…”

Patterns of lung fibrosis in patients with interstitial pneumonia with autoimmune features and connective tissue diseases-associated interstitial lung disease—a narrative review

“…Esophageal dilatation, esophageal motor dysfunction also associated with higher prevalence of ILD(66) CTDs, connective tissue diseases; MCTD, mixed connective tissue disease; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; MCTD-ILD, interstitial lung disease in course of mixed connective tissue disease; PFTs, pulmonary function tests; NSIP, non-specific interstitial pneumonia; GGO, ground glass opacity; ILD, interstitial lung disease.…”

Blood KL-6 predicts prognosis in primary Sjögren’s syndrome-associated interstitial lung disease

“…Moreover, there was no difference in the frequency of positive autoantibodies such as ANA and anti-SSB between the non-survivors and survivors. The previous study support our findings; Gao et al, in 178 patients with SJS-ILD, reported that the frequency of positive ANA (65.2 vs 75.8%, P = 0.329), anti-SSA (56.0 vs 57.3%, P = 0.907), and anti-SSB (40.0 vs 37.3%, P = 0.812) was not different between the non-survivors and survivors, suggesting that autoantibodies were not associated with prognosis of ILD 44 . However, there is also a contradictory report; Boitiaux et al, in 45 newly diagnosed patients with idiopathic interstitial pneumonia, showed that the anti-SSA (+) group (n = 15) had lower vital capacity (63 ± 22 vs. 87 ± 23% predicted, P = 0.006) and more frequent GGO (87 vs 67%, P = 0.001) and reticulation (33 vs 21%, P = 0.030) on HRCT than the anti-SSA(−) group (n = 30) 45 .…”