ReviewInterstitial lung disease in primary Sjögren's syndrome
Introduction
Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease affecting the exocrine glands and other organs, especially the lungs resulting in interstitial lung disease (ILD) and small airways disorders [1], [2], [3], [4], [5], [6]. The prevalence of pulmonary involvement has been reported to be 9 to 75% in pSS, depending on the diagnostic tests used to detect ILD; pulmonary disorders are still considered to be a cause of morbidity in these patients [7], [8], [9], [10], [11], [12]. ILD has been observed in 3 to 11% of patients with pSS and may lead to life-threatening complications, including ventilatory failure and secondary pulmonary hypertension [12], [13], [14]. Previous small series have shown that ILD was responsible for 42.9% to 90% of deaths in these patients [14], [15]. The early detection of ILD is thus crucial in patients with pSS. The aims of this retrospective study were to: 1) assess the features and outcome of ILD in patients with pSS; and 2) determine factors that are predictive of: (i) ILD onset; and (ii) ILD deterioration in pSS.
Section snippets
Patients
This retrospective study began with a search of the institutional centers' medical record index, which provides access to the diagnoses of the centers' patients. The first electronic search involved use of the codes SS to identify patients with a diagnosis of pSS seen from January 1996 to January 2012 in two academic centers (Rouen: Departments of Internal medicine, Rheumatology, Pneumology and Nephrology; Amiens: Departments of Internal medicine).
The diagnosis of pSS was based on
Patient characteristics
Among 263 consecutive pSS patients, 21 patients (8%) had ILD. The patients with ILD consisted of 3 men (14.3%) and 18 women (85.7%) with a median age of 63 years [range: 42–81 years] at pSS diagnosis.
Characteristics of ILD
ILD onset preceded initial pSS clinical manifestations in 5 patients, was concurrently identified in association with pSS in 6 patients and developed after pSS onset in 9 patients (Table 1).
At ILD diagnosis, pulmonary symptoms consisted of dyspnea (n = 14), cough (n = 14), hemoptysis (n = 3), fever (n = 3)
Discussion
ILD occurs in 9 to 75% of pSS patients [7], [8], [9], [10], [11], [12]. However, to date, the outcome of ILD still remains poorly defined in these patients. In the current study, 263 patients were included without any prior selection based on clinical presentation. Because our population of patients (who were referred to tertiary care centers) was reasonably homogeneous, we believe that the evaluation and response to treatment of ILD, under standard conditions, is possible. Our study confirms
Conclusion
In conclusion, our series highlights that ILD results in high morbidity in pSS patients. Our study also suggests that the following parameters could be considered as predictive of ILD onset: older age, Raynaud's phenomenon and digestive involvement, particularly esophageal impairment. Moreover, older age and digestive involvement are associated with a poorer prognosis in pSS patients with ILD. Finally, the presence of these factors may suggest a closer follow-up and a more aggressive therapy of
Take-home messages
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Our series shows that ILD results in high morbidity in pSS patients.
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The following parameters may be considered as predictive of ILD onset: older age, Raynaud's phenomenon and digestive involvement, particularly esophageal impairment.
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Older age and digestive involvement are associated with a poorer prognosis in pSS patients with ILD. The presence of these factors may suggest a closer follow-up and a more aggressive therapy of pSS patients with ILD.
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