Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology

Sambataro, Gianluca; Ferro, Francesco; Orlandi, Martina; Sambataro, Domenico; Torrisi, Sebastiano Emanuele; Quartuccio, Luca; Vancheri, Carlo; Baldini, Chiara; Cerinic, Marco Matucci

doi:10.1016/j.autrev.2019.102447

Cited by 75 publications

(61 citation statements)

References 46 publications

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“…Our pooled pSS-ILD prevalence confirmed that pSS-ILD was a common complication, which was supported by a recent study. 26 Further, our meta-analysis summarized the data for a generalized conclusion. Furthermore, the pSS-ILD prevalence was stable in sensitivity analysis, suggesting the conclusion is robust.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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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“…The finding of an increased portion of α2 globulins is considered a sign of inflammation, and the study of γ globulins in SPEP is also of some interest in ILD patients. Although hypergammaglobulinemia is not included in any classification criteria of CTDs, it has commonly been found in these patients, mainly in SLE and SjS [69]. On the contrary, hypogammaglobulinemia is a marker of common variable immunodeficiency, a condition that can produce ILD in the lung, mainly resembling an advanced stage of sarcoidosis in High-Resolution Computed Tomography (HRCT) [70].…”

Section: General Laboratory Examsmentioning

confidence: 99%

“…It should be taken into consideration that anti-SSA can be positive at ANA negative for the loss of anti-SSA60 kD during preparation, but also because SSA52 kD recognizes a cytoplasmic antigen [96]. SSA52 kD is mainly associated with ILD in both IIMs and SjS [69]. The ENA profile also includes anti-Jo1, the most frequent antisynthetase antibody and the unique one included in ENA commercial kits.…”

Section: First-line Autoimmunity Examsmentioning

confidence: 99%

“…Indeed, up to 35% of these patients show non-caseating necrotizing granulomas; so, for them a possible alternative diagnosis of AAV or RA should be considered [136]. Minor salivary gland biopsy can be very useful in the diagnosis of ILD underlying SjS, mainly considering the possible seronegative subset of these patients [69]. The kidney and the upper and lower respiratory tract may be involved by inflammation during AAV, but about 30% of histological exams of the respiratory tract can give a false negative result [137].…”

Section: Second-line Instrumental Examsmentioning

confidence: 99%

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Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Sambataro

Pignataro³

et al. 2020

Diagnostics

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The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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“…Pulmonary manifestations are polymorphic, representing a leading cause of morbidity and mortality in these patients, particularly when an interstitial lung disease (ILD) is diagnosed ( pSS-ILD) [21].…”

Section: Clinical Manifestations and Prognosis Of Pulmonary Involvementmentioning

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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Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology

Cited by 75 publications

References 46 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

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Lung complications of Sjogren syndrome

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