Lung function in primary Sjogren's syndrome: a cross sectional and longitudinal study.

Kelly, Clive; Gardiner, Philip; Pal, Badal; Griffiths, I. D.

doi:10.1136/thx.46.3.180

Cited by 90 publications

(62 citation statements)

References 17 publications

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“…The prevalence of clinically significant lung disease in Sjögren's syndrome is 9-20% [35] with a female predominance [36]. When investigated systematically, prevalence increases to between 43% and 75% [37]. Computed tomography (CT) scan abnormalities are found in 34-50% of patients [38].…”

Section: Sjögren's Syndrome Patients With Pulmonary Involvementmentioning

confidence: 99%

“…Pulmonary involvement is mostly associated with systemic manifestations, hypergammaglobulinaemia and anti-SSA and anti-SSB antibodies [37,39,43,44]. Other biological markers proposed as risk factors for pulmonary involvement include the presence of anti-nuclear antibodies or rheumatoid factors [39,45].…”

Section: Sjögren's Syndrome Patients With Pulmonary Involvementmentioning

confidence: 99%

“…The majority of Sjögren's syndrome patients have preserved lung function [37,40,47]. When it is abnormal, the PFT generally shows a restrictive rather than an obstructive pattern.…”

Section: Pulmonary Function Test In Sjögren's Syndromementioning

confidence: 99%

“…The estimated frequency of distal airway disease in Sjögren's syndrome patients, assessed by PFT, varies from 22% to 46% [47-49, 56, 61]. Obstructive ventilatory syndrome is rare (11-14%) [37,47] and seems to be related to severity of the disease. Severe forms are rarely observed [62].…”

Section: Airway Disease 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: Sjögren's Syndrome Patients With Pulmonary Involvementmentioning

confidence: 99%

Section: Sjögren's Syndrome Patients With Pulmonary Involvementmentioning

confidence: 99%

“…The majority of Sjögren's syndrome patients have preserved lung function [37,40,47]. When it is abnormal, the PFT generally shows a restrictive rather than an obstructive pattern.…”

Section: Pulmonary Function Test In Sjögren's Syndromementioning

confidence: 99%

Section: Airway Disease In Sjögren's Syndromementioning

confidence: 99%

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Pulmonary manifestations of Sjögren's syndrome

et al. 2016

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“…Over 40% of patients with primary SS examined had pulmonary symptoms [9], and approximately five per cent of patients showed interstitial infiltration radiographically [1,9]. Patients with extraglandular primary SS most often had diffuse interstitial lung disease [1].…”

Section: Discussionmentioning

confidence: 99%

A superfemale with primary Sjögren's syndrome which involved systemic organs

Miyakawa

Iyonaga

Arima

et al. 1997

Journal of Internal Medicine

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Miyakawa H, Iyonaga K, Arima S, Yonekawa Y, Suga M, Ando M (First and Third Departments of Internal Medicine, Kumamoto University School of Medicine, Kumamoto, Japan). A superfemale with primary Sjögren's syndrome which involved systemic organs (Case report). J Intern Med 1997; 242: 261–5. A 52‐year‐old Japanese woman complicated by a sex chromosomal anomaly as a superfemale, a mosaic of XXXXX/XXXX/XXX/XX/XO, with mild mental retardation, was hospitalized for dry mouth, dry eyes, and proteinuria. The sialography of the right parotid gland showed a globular‐type gland enlargement. A definite diagnosis of primary Sjögren's syndrome (SS) was made, and further examinations revealed not only typical sicca syndrome but also systemic extraglandular lymphocytic infiltration; interstitial pneumonitis, glomerular‐ and interstitial nephritis, superficial gastritis, thyroiditis, and a severe excitation conductive impairment of heart. We report a very rare case of superfemale with primary SS which involved systemic organs.

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