Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements

Ramos‐Casals, Manuel; Brito-Zerón, Pilar; Séror, Raphaèle; Bootsma, Hendrika; Bowman, Simon; Dörner, Thomas; Gottenberg, Jacques-Éric; Mariette, Xavier; Theander, Elke; Bombardieri, Stefano; Vita, Salvatore De; Mandl, Thomas; Ng, Wan‐Fai; Kruize, Aike A.; Tzioufas, Athanasios G.; Vitali, Claudio

doi:10.1093/rheumatology/kev200

Cited by 244 publications

(263 citation statements)

References 26 publications

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“…However, nonspecific interstitial pneumonitis (NSIP) seems to be the most common pathological subtype revealed by lung biopsy [36,46,51,[87][88][89]. However, these studies are based on small samples.…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

“…However, these studies are based on small samples. A recent review of 146 histological cases in the literature found 45% NSIP, 16% usual interstitial pneumonitis (UIP), 7% organising pneumonia, 15% LIP and 17% other pathologies [89]. The main symptoms of these ILDs are dyspnoea and cough.…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

“…New and extensive areas of airspace consolidation or ground-glass attenuation on CT should be considered as probable signs of acute exacerbation of interstitial pneumonia. Nonspecific interstitial pneumonia NSIP is the most common subtype of ILD observed in Sjögren's syndrome patients (45% of patients) [89]. The histological features of the NSIP pattern consist of varying amounts of interstitial inflammation and fibrosis with a uniform appearance (figure 3c).…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

“…UIP represents ∼16% of cases of ILD in Sjögren's syndrome [89]. It is regarded as an irreversible lung disease with a high risk of progression, despite therapy.…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

“…LIEBOW and CARRINGTON [86] found that in a cohort of patients with LIP, 28% had Sjögren's syndrome. LIP seems to account for 15% of cases of Sjögren's syndrome patients with ILD [89]. Most patients have respiratory symptoms (particularly dyspnoea and cough), and bilateral inspiratory crackles" may be heard [103].…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

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Pulmonary manifestations of Sjögren's syndrome

et al. 2016

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In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

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Section: Ild In Sjögren's Syndromementioning

confidence: 99%

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

“…UIP represents ∼16% of cases of ILD in Sjögren's syndrome [89]. It is regarded as an irreversible lung disease with a high risk of progression, despite therapy.…”

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

Section: Ild In Sjögren's Syndromementioning

confidence: 99%

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Pulmonary manifestations of Sjögren's syndrome

et al. 2016

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Study on the mechanism of Cutaneous and Respiratory Comorbidities with Chinese and Western medicine theories and research

Liu,

Xie,

Yan

et al. 2024

Advanced Chinese Medicine

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In the realms of traditional Chinese medicine and Western medicine research, the skin and lungs are recognized for their integral roles in both the physiological functions and pathological mechanisms affecting the human body. These organs are not only complementary and coordinated in their physiological functions but also interact pathologically, providing feedback and influencing each other. The etiology of immune‐mediated skin diseases is multifaceted, involving numerous factors beyond mere skin characteristics, often including multisystem diseases. Cutaneous and Respiratory Comorbidities denote the concurrent presence of skin and lung diseases, sharing a common pathogenic mechanism. Although research on Cutaneous and Respiratory Comorbidities has received increasing attention, the foundational theories and the specifics of related diseases remain unclear. This paper aims to elucidate the theoretical basis of both traditional Chinese and Western medicine concerning immune‐mediated Cutaneous and Respiratory Comorbidities, thereby laying a groundwork for the early detection and integrated treatment of these diseases.

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Severe Health‐Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient‐Reported Outcomes: Data From a Large Therapeutic Trial

Cornec

Devauchelle‐Pensec

Mariette

et al. 2017

Arthritis Care & Research

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Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements

Abstract: The proposals included in this article are a first step to developing an optimal diagnostic approach to systemic involvement in primary SS and may pave the way for further development of evidence-based diagnostic and therapeutic guidelines.

Cited by 244 publications

References 26 publications

Pulmonary manifestations of Sjögren's syndrome

Pulmonary manifestations of Sjögren's syndrome

Study on the mechanism of Cutaneous and Respiratory Comorbidities with Chinese and Western medicine theories and research

Severe Health‐Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient‐Reported Outcomes: Data From a Large Therapeutic Trial

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