Risk factors for primary Sjögren syndrome-associated interstitial lung disease

Wang, Yong; Hou, Ziliang; Qiu, Meihua; Ye, Qiao

doi:10.21037/jtd.2018.03.120

Cited by 71 publications

(67 citation statements)

References 37 publications

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“…remains controversial. Positive rheumatoid factor (RF), anti-SSA antibody, and antinuclear antibody (ANA) have been proposed as the risk factors for pSS-ILD, 9,13 which were not confirmed in the other studies. 14,15 To address these points, we systematically reviewed all available public data on pSS-ILD and performed a meta-analysis of the prevalence and risk factors for pSS-ILD.…”

mentioning

confidence: 91%

Prevalence and risk factors of interstitial lung disease in patients with primary Sjögren's syndrome: A systematic review and meta‐analysis

Chen

Liu

et al. 2020

Int J of Rheum Dis

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Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by exocrinopathy, with an estimated prevalence of 0.33%-0.77% in China and 0.06% worldwide. 1,2 pSS patients primarily suffer from xerophthalmia and xerostomia and 30%-40% of pSS patients present extra-glandular manifestations including arthritis, nephritis, and neuropathy. 3 Interstitial lung diseases (ILD) are a group of heterogeneous inflammatory and fibrotic pulmonary parenchymal disorders, which are frequently complicated in connective tissue diseases. 4,5 ILDs are diagnosed based on clinical presentations, high-resolution computed tomography (HRCT), pulmonary function test (PFT), and lung biopsy. 6,7 HRCT is a sensitive diagnostic tool for ILD, and is strongly correlated with pulmonary histology and PFT, with nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, and bronchiolitis as the most common HRCT patterns in pSS patients. 8,9 Interstitial lung diseases is a poor prognosis factor and accounts for one-tenth of deaths in pSS patients. 10,11 However, the reported prevalence of ILD in pSS patients varies significantly, ranging from 1.5% to 78%. 9,12 Furthermore, the risk factors for ILD in pSS patients

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mentioning

confidence: 91%

Prevalence and risk factors of interstitial lung disease in patients with primary Sjögren's syndrome: A systematic review and meta‐analysis

Chen

Liu

et al. 2020

Int J of Rheum Dis

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“…204 In a recent study from China, 201 subjects with newly diagnosed pSS were followed up for a median of 24 months (range: 18-30). 205 Overall, ILD was diagnosed in 78.6%; factors associated with increased risk of ILD included male gender, older age, history of smoking, and (þ) ANA. 205 Lung biopsies showed NSIP pattern in 45.5%.…”

Section: Interstitial Lung Diseasementioning

confidence: 96%

“…205 Overall, ILD was diagnosed in 78.6%; factors associated with increased risk of ILD included male gender, older age, history of smoking, and (þ) ANA. 205 Lung biopsies showed NSIP pattern in 45.5%. The course was generally benign.…”

Section: Interstitial Lung Diseasementioning

confidence: 96%

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

Chung

Wilgus

Fishbein

et al. 2019

Semin Respir Crit Care Med

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Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). B cell hyperactivity, autoantibody production, and hypergammaglobulinemia are cardinal features of SS. Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed “secondary SS.” Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.

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“…Various studies suggest that the main risk factors for pulmonary involvement are male sex, being active smokers, late onset of the disease and having a long-lasting disease [6,22,23].…”

Section: Clinical Manifestations and Prognosis Of Pulmonary Involvementmentioning

confidence: 99%

“…Lymphocytic interstitial pneumonia (LIP) has a classic association with pSS [45], although it does not appear as the most common pathological subtype. Nonspecific interstitial pneumonia (NSIP) is recognised as the most common ILD disorder [5,23] followed by organising pneumonia (OP) and usual interstitial pneumonia (UIP), the latter considered particularly frequent in some case series with early onset of ILD [46]. Rare cases of acute fibrinous and organising pneumonia and pleuroparenchymal fibroelastosis in SS are also reported [47][48][49].…”

Section: Pulmonary Manifestationsmentioning

confidence: 99%

Lung complications of Sjogren syndrome

et al. 2020

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Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.

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Risk factors for primary Sjögren syndrome-associated interstitial lung disease

Cited by 71 publications

References 37 publications

Prevalence and risk factors of interstitial lung disease in patients with primary Sjögren's syndrome: A systematic review and meta‐analysis

Prevalence and risk factors of interstitial lung disease in patients with primary Sjögren's syndrome: A systematic review and meta‐analysis

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

Lung complications of Sjogren syndrome

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