Interstitial lung disease in primary Sjögren's syndrome

Roca, Frédéric; Dominique, S.; Schmidt, J.; Смаил, А.; Duhaut, P.; Lévesque, H.; Marie, I.

doi:10.1016/j.autrev.2016.09.017

Cited by 142 publications

(166 citation statements)

References 40 publications

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“…Primary SS is a systemic autoimmune disease affecting exocrine glands, resulting in xerostomia/dry-mouth and xerophthalmia/dry-eyes (“sicca syndrome”), with variable extraglandular and lung involvement. Population-based estimates of SS-associated ILD range from 3% to 11% and is associated with worse survival [36,118]. In a recent multi-centre study of 263 French patients with SS, there was a non-significant trend towards more frequent ANA-positivity in patients with ILD, but no association with anti-SSA/Ro or anti-SSB/La antibodies [36].…”

Section: Ctd Associated Autoantibodiesmentioning

confidence: 99%

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

Jee

Adelstein

Bleasel

et al. 2017

JCM

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The diagnosis of interstitial lung disease (ILD) requires meticulous evaluation for an underlying connective tissue disease (CTD), with major implications for prognosis and management. CTD associated ILD (CTD-ILD) occurs most commonly in the context of an established CTD, but can be the first and/or only manifestation of an occult CTD or occur in patients who have features suggestive of an autoimmune process, but not meeting diagnostic criteria for a defined CTD—recently defined as “interstitial pneumonia with autoimmune features” (IPAF). The detection of specific autoantibodies serves a critical role in the diagnosis of CTD-ILD, but there remains a lack of data to guide clinical practice including which autoantibodies should be tested on initial assessment and when or in whom serial testing should be performed. The implications of detecting autoantibodies in patients with IPAF on disease behaviour and management remain unknown. The evaluation of CTD-ILD is challenging due to the heterogeneity of presentations and types of CTD and ILD that may be encountered, and thus it is imperative that immunologic tests are interpreted in conjunction with a detailed rheumatologic history and examination and multidisciplinary collaboration between respiratory physicians, rheumatologists, immunologists, radiologists and pathologists.

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Section: Ctd Associated Autoantibodiesmentioning

confidence: 99%

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

Jee

Adelstein

Bleasel

et al. 2017

JCM

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“…17 Moreover, cases of ILD preceding the diagnosis of pSS have been described. 5,6,9,[18][19][20][21][22] All of these findings indicate that it is challenging to establish the SS diagnosis in this subgroup. Limited studies have focused on this aspect of pSS-ILD patients.…”

Section: Introductionmentioning

confidence: 99%

Interstitial lung disease in non‐sicca onset primary Sjögren's syndrome: a large‐scale case‐control study

et al. 2018

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“…Nevertheless, in 60% of the patients, the diagnosis of ILD preceded that of pSS (23). Conversely, Roca et al reported that only 5 (24%) out of 21 patients were diagnosed with ILD prior to the diagnosis of pSS (24). The histopathological patterns observed were NSIP, UIP, OP and LIP (in this order) and the majority of ILD patients had an acute/subacute onset (24).…”

Section: N Cutaneous Involvementmentioning

confidence: 99%

“…Conversely, Roca et al reported that only 5 (24%) out of 21 patients were diagnosed with ILD prior to the diagnosis of pSS (24). The histopathological patterns observed were NSIP, UIP, OP and LIP (in this order) and the majority of ILD patients had an acute/subacute onset (24). Overall, in 84% of patients pSS-ILD was either stable over time or worsening, while older age was associated with ILD deterioration.…”

Section: N Cutaneous Involvementmentioning

confidence: 99%

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

et al. 2017

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SUMMARYAlthough primary Sjögren's syndrome (pSS) is a mild indolent chronic disease mainly characterized by mucosal dryness in the majority of cases, a consistent subgroup of patients display extra-glandular manifestations. Virtually any organs and systems can be affected, leading to a more serious disease prognosis. Therefore, the prompt identification of patients at higher risk of extra-glandular manifestations is necessary to start a thorough follow up and an aggressive treatment. The aim of this review article is to provide an overview of epidemiological, clinical and serological features of extra-glandular manifestations in pSS as well as current knowledge about putative biomarkers useful in clinical practice. Key words:Sjögren's syndrome; Extra-glandular manifestations; Cardiovascular risk. Reumatismo, 2017; 69 (3): 93-100 n INTRODUCION P rimary Sjögren's syndrome (pSS) is a complex and heterogeneous chronic autoimmune disease primarily characterized by a focal lymphocytic chronic inflammation of exocrine glands leading to progressive loss of secretory function (1). Therefore, the specific case-history of pSS is dominated by signs and symptoms of mucosal dryness. However, although salivary and lachrymal glands represent the main target of pSS, several other organs and systems may be affected during the disease course in at least one third of patients, resulting in a plethora of systemic clinical manifestations, serological abnormalities and complications (2). The most severe complication of pSS is non-Hodgkin lymphoma occurring in about 5% of patients (3) and dramatically worsening disease prognosis. In this regard, the prompt identification of pSS patients with higher risk of developing extraglandular manifestations is mandatory for correct management. Low C3 and C4 levels, cryoglobulins, monoclonal component, anti-Ro/ SSA, anti-La/SSB, rheumatoid factor and hypergammaglobulinaemia represent poor prognostic serological factors in pSS associated with lymphoma and severe extraglandular features (2). Furthermore, histological features such as the presence of germinal centre-like structures in the minor salivary glands have been associated with higher risk of developing lymphoma (4). The purpose of this review article is to discuss current knowledge of extra-glandular manifestations in pSS not secondary to mucosal dryness, excluding pathogenesis and clinical manifestations of lymphoproliferative disorders. n MUSCULOSKELETAL INVOLVEMENTMusculoskeletal manifestations such as myalgia, arthralgia and morning stiffness are present in as many as 90% of patients, while clinically evident arthritis is found in up to 17%. In a recent study conducted on 17 female patients with pSS and 18 patients with secondary SS (sSS), ultrasonography (US) was used to confirm the non-erosive nature of joint involvement in pSS and to discriminate between pSS and RA-associated SS. Synovitis was commonly detected in pSS, mainly in metacarpophalangeal joints (MCP), wrists, and knees. Bone erosions also may occur. Power Doppler signal ...

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Interstitial lung disease in primary Sjögren's syndrome

Cited by 142 publications

References 40 publications

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

Interstitial lung disease in non‐sicca onset primary Sjögren's syndrome: a large‐scale case‐control study

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

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