Polyautoimmunity and Major Organ Involvement Prevalence in Sjögren´s Syndrome: Thyroid, Liver, Lung and Kidney as Targets. a Single Center Cross Sectional Study

Larissa Valor¹, Hannah Schenker ², Johannes Knitza ³, Melanie Hagen ³, Jürgen Rech ⁴ and Georg Schett ⁵, ¹Friedrich-Alexander-University Erlangen-Nürnberg. Department of Internal Medicine 3 - Rheumatology and Immunology. Erlangen., Erlangen, Germany, ²Friedrich-Alexander-University Erlangen-Nürnberg. Department of Internal Medicine 3 - Rheumatology and Immunology. Erlangen. Germany., Erlangen, Germany, ³Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁴Department of Internal Medicine 3, Friedrich-Alexander-University Erlangen-Nuremberg (FAU) and Universitätsklinikum Erlangen, Erlangen, Germany, ⁵Department of Internal Medicine 3, Friedrich Alexander University Erlangen-Nuremberg, Erlangen, Germany

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: renal disease and Lung Disease, Sjogren's syndrome, thyroid

Session Information

Date: Monday, November 11, 2019

Title: Sjögrenʼs Syndrome – Basic & Clinical Science Poster II

Session Type: Poster Session (Monday)

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

Background/Purpose: Polyautoimmunity has been described to be associated with primary Sjögren´s syndrome (SjS) and the most frequent observed associated autoimmune diseases (AID) are autoimmune thyroid disease, autoimmune hepatitis and primary biliary cirrhosis, which are common organ-specific AID. In the same track, renal and lung involvement has increasingly been documented in SjS further highlighting its systemic nature. Objective: To describe and classify prevalence of polyautoimmunity and major organ involvement in a primary SjS-cohort.

Methods: This cross-sectional study included 179 patients [160 (89%) females and 19 (11%) males] diagnosed with primary SjS and fulfilling the ACR classification criteria that had been admitted to our outpatient clinic between December 2008 and December 2018. Demographic and disease-specific characteristics were recorded in all patients.

Results: In our cohort the median age at diagnosis was 57 years (range: 20-85). Thyroid AID was found in 55/179 (30%) patients, with the following distribution: Hashimoto thyroiditis without (n=21) and with hypothyroidism (n=22), Graves’s disease without (n=4) and with thyroidectomy (n=8). Liver AID was detected in 8/179 patients (4%), 3 patients with autoimmune hepatitis and 5 patients with primary biliary cirrhosis. Regarding major organ involvement, 20/179 (11%) patients had renal manifestations: renal insufficiency (n=12), glomerulonephritis (n= 3), interstitial nephritis (n=2) and IgA nephritis (n=3). Eight/179 (4%) patients had lung manifestations: interstitial fibrosis (n=6), emphysema (n=1) and chronic obstructive pulmonary disease (n=1).

Conclusion: Our results add evidence for the presence of polyautoimmnunity and major organ involvement in SjS. We found a slightly lower prevalence of polyautoimmunity and major organ involvement compared to recently reported data. Nonetheless, extra-glandular organ involvement should be assessed in order to elucidate cumulative damage and how it might impact outcome, prognosis and therapeutic approaches in SjS.

Disclosure: L. Valor, None; H. Schenker, None; J. Knitza, None; M. Hagen, None; J. Rech, AbbVie, 8, Biogen, 8, BMS, 5, 8, Celgene, 5, 8, Chugai, 5, MSD, 8, Novartis, 5, 8, Roche, 5; G. Schett, AbbVie, BMS, Celgene, Janssen, Lilly, Novartis, Pfizer, 8, AbbVie, BMS, Celgene, Janssen, Lilly, Novartis, UCB, 5, BMS, Celgene, GSK, Lilly, Novartis, 2.

To cite this abstract in AMA style:

Valor L, Schenker H, Knitza J, Hagen M, Rech J, Schett G. Polyautoimmunity and Major Organ Involvement Prevalence in Sjögren´s Syndrome: Thyroid, Liver, Lung and Kidney as Targets. a Single Center Cross Sectional Study [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/polyautoimmunity-and-major-organ-involvement-prevalence-in-sjogrens-syndrome-thyroid-liver-lung-and-kidney-as-targets-a-single-center-cross-sectional-study/. Accessed .

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