Abstract：
OBJECTIVE: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis.
METHODS: We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE.
RESULTS: Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren\'s syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud\'s phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression.
CONCLUSIONS: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease.
METHODS: We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE.
RESULTS: Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren\'s syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud\'s phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression.
CONCLUSIONS: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease.
摘要：
目的：与其他结缔组织疾病相比，系统性红斑狼疮（SLE）很少发生慢性间质性肺病（ILD）。这项对2005-2020年间来自OrphaLung和法国SLE网络的SLE-ILD患者进行的多中心回顾性研究旨在描述SLE-ILD患者的特征并分析与预后相关的因素。
方法：我们分析了89例SLE-ILD患者的数据（82例女性，92.1％）（SLE诊断时的中位年龄：35岁[四分位距27-47]）。所有患者均符合2019年EULAR/ACR诊断SLE标准。
结果：42例（47.2％）患者抗核糖核蛋白抗体阳性，45例（50.6％）患者抗SSA/Ro抗体阳性。共有58例（65.2％）患者患有另一种结缔组织疾病：干燥综合征（n=33，37.1％），系统性硬化症（n=14，15.7%），炎性肌病（n=6，6.7％），或类风湿性关节炎（n=6，6.7％）。在25例（28.1%）患者中，在SLE诊断后的中位数为6年（0-14年）时，ILD与SLE一起被诊断出。最常见的CT模式提示非特异性间质性肺炎（n=41，46.0％）伴或不伴叠加的机化性肺炎。在中位随访86.5[39.5-161.2]个月后，18例（20.2%）患者死亡，6例（6.7%）接受肺移植。5年和10年无移植生存率分别为96%(92-100)和87%(78-97)。总的来说,44例（49.4%）患者出现ILD进展。皮肤表现和雷诺现象与更好的生存率相关。只有强迫肺活量与生存率和ILD进展显着相关。
结论：ILD是SLE的罕见表现，总体预后良好，但可能存在ILD进展的风险。SLE-ILD患者经常患有另一种结缔组织疾病。
方法：我们分析了89例SLE-ILD患者的数据（82例女性，92.1％）（SLE诊断时的中位年龄：35岁[四分位距27-47]）。所有患者均符合2019年EULAR/ACR诊断SLE标准。
结果：42例（47.2％）患者抗核糖核蛋白抗体阳性，45例（50.6％）患者抗SSA/Ro抗体阳性。共有58例（65.2％）患者患有另一种结缔组织疾病：干燥综合征（n=33，37.1％），系统性硬化症（n=14，15.7%），炎性肌病（n=6，6.7％），或类风湿性关节炎（n=6，6.7％）。在25例（28.1%）患者中，在SLE诊断后的中位数为6年（0-14年）时，ILD与SLE一起被诊断出。最常见的CT模式提示非特异性间质性肺炎（n=41，46.0％）伴或不伴叠加的机化性肺炎。在中位随访86.5[39.5-161.2]个月后，18例（20.2%）患者死亡，6例（6.7%）接受肺移植。5年和10年无移植生存率分别为96%(92-100)和87%(78-97)。总的来说,44例（49.4%）患者出现ILD进展。皮肤表现和雷诺现象与更好的生存率相关。只有强迫肺活量与生存率和ILD进展显着相关。
结论：ILD是SLE的罕见表现，总体预后良好，但可能存在ILD进展的风险。SLE-ILD患者经常患有另一种结缔组织疾病。
