Elsevier

Autoimmunity Reviews

Volume 16, Issue 1, January 2017, Pages 48-54
Autoimmunity Reviews

Review
Interstitial lung disease in primary Sjögren's syndrome

https://doi.org/10.1016/j.autrev.2016.09.017Get rights and content

Abstract

Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n = 5), was concurrently identified in association with pSS (n = 6) and developed after pSS onset (n = 9). Presenting ILD manifestations were: acute/subacute (n = 11) onset of ILD, symptomatic progressive onset of ILD (n = 5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n = 5). ILD therapy included: steroids (n = 21), cyclophosphamide (n = 1), azathioprine (n = 4) and rituximab (n = 1). The course of ILD was as follows: improvement (15.8%), stabilization (47.4%) or deterioration (36.8%). Predictive parameters of ILD onset were: older age (p = 0.044), Raynaud's phenomenon (p = 0.001) and esophageal involvement (p = 0.001). Factors associated with ILD deterioration were: older age (p = 0.038) and esophageal involvement (p = 0.038). Thus, this study underscores the poor outcome of ILD during pSS; thus, systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at early stage of ILD. We also suggest that patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy.

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

  • Our series shows that ILD results in high morbidity in pSS patients.

  • The following parameters may be considered as predictive of ILD onset: older age, Raynaud's phenomenon and digestive involvement, particularly esophageal impairment.

  • 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.

References (41)

  • R. Vij et al.

    Diagnosis and treatment of connective tissue disease-associated interstitial lung disease

    Chest

    (2013)
  • P.Y. Hatron et al.

    Pulmonary manifestations of Sjogren's syndrome

    Presse Med

    (2011)
  • C. Kelly et al.

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

    Thorax

    (1991)
  • M. Kreider et al.

    Pulmonary involvement in Sjögren syndrome

    Semin Respir Crit Care Med

    (2014)
  • C. Nannini et al.

    Primary Sjogren's syndrome 1976–2005 and associated interstitial lung disease: a population-based study of incidence and mortality

    BMJ Open

    (2013)
  • O. Palm et al.

    Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality--a retrospective study based on registry data

    Rheumatology (Oxford)

    (2013)
  • M. Ramos-Casals et al.

    Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients

    Medicine (Baltimore)

    (2008)
  • D. Launay et al.

    Pulmonary arterial hypertension: a rare complication of primary Sjogren syndrome: report of 9 new cases and review of the literature

    Medicine (Baltimore)

    (2007)
  • I. Ito et al.

    Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study

    Am J Respir Crit Care Med

    (2005)
  • C. Vitali et al.

    Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group

    Ann Rheum Dis

    (2002)
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