Interstitial Lung Disease in Primary Sjögren’s Syndrome : Clinical Presentation, Serological Biomarkers and Long Term Outcome

Chiara Baldini¹, Ilaria Puxeddu², Martina Orlandi³, Francesco Ferro⁴, Elena Elefante⁴, Nicoletta Luciano⁴, Marco Matucci-Cerinic³, Paola Migliorini² and Marta Mosca⁴, ¹Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, ²Allergology and Clinical Immunology Unit, University of Pisa, Pisa, Italy, ³Rheumatology Unit, University of Florence, Florence, Italy, ⁴Rheumatology Unit, University of Pisa, Pisa, Italy

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Lung Disease, RANTES and Sjogren's syndrome

Session Information

Date: Monday, November 6, 2017

Title: Sjögren's Syndrome Poster II: Clinical Research

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Interstitial lung disease (ILD) is a rare but potentially severe manifestation of primary Sjögren’s syndrome (pSS). The aims of this study were to: 1) analyze clinical presentation, characteristics and outcome of ILD in pSS; 2) evaluate predictive factors associated with ILD onset and evolution; 3) explore whether the inflammatory chemokines RANTES, IL-8 and MCP-1 may be associated to pSS-related ILD.

Methods: Patients with a diagnosis of pSS (AECG 2002) were included in this single center study. Demographic, clinical and radiological data were collected retrospectively. Serum levels of RANTES,MCP-I and IL-8 were measured by commercial ELISA kits in a subgroup of pSS patients with and without ILD.

Results: We included in this study 285 (12 M:273 F) patients with pSS. Eighteen out of 285 (6.3%) presented ILD: 12/18 NSIP and 6/18 UIP pattern. Patients with ILD were more frequently males (4/18 (22%) vs 8/267 (3%), p=0.004) and older at the diagnosis with respect to non-ILD pSS patients (62±13 vs 50±13 yrs, p=0.000). The clinical presentation of ILD was acute-subacute in 8 cases, slow progressive in 3 and subclinical in 7 patients. ILD diagnosis preceded pSS diagnosis in 8/18 cases. These patients presented more often a UIP pattern (5/8 vs 1/10, p=0.04). Patients were treated with steroids in monotherapy (7/18) or in association with immunosuppressive drugs (11/18) including cyclophosphamide (n=4), azathioprine (n=5), mycophenolate mofetil (n=1) and rituximab (n=1). During the disease course 5 patients improved, 11 were stabilized and 2 worsened. Patients that at the end of the follow-up presented a DLCO <60%, were more frequently positive for anti-Ro/SSA antibodies (p=0.04) and all presented a UIP pattern (p=0.000). Finally, circulating levels of RANTES, but not of IL-8 and MCP-1 were significantly able to discriminate pSS-ILD from non-ILD pSS patients (620.4±318.4 vs 280.2±180.9, p=0.02).

Conclusion: This study showed that, despite rare, ILD can be a presenting feature of pSS especially in males and older patients. The inflammatory chemokine RANTES, promoting lymphocyte infiltration, may play a role in the pathophysiology of pSS-related ILD and may have a role as diagnostic biomarker for ILD especially in less symptomatic pSS patients.

Disclosure: C. Baldini, None; I. Puxeddu, None; M. Orlandi, None; F. Ferro, None; E. Elefante, None; N. Luciano, None; M. Matucci-Cerinic, None; P. Migliorini, None; M. Mosca, None.

To cite this abstract in AMA style:

Baldini C, Puxeddu I, Orlandi M, Ferro F, Elefante E, Luciano N, Matucci-Cerinic M, Migliorini P, Mosca M. Interstitial Lung Disease in Primary Sjögren’s Syndrome : Clinical Presentation, Serological Biomarkers and Long Term Outcome [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/interstitial-lung-disease-in-primary-sjogrens-syndrome-clinical-presentation-serological-biomarkers-and-long-term-outcome/. Accessed .

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