Full Papers
Characterisation of the coexistence between sarcoidosis and Sjögren's syndrome. Analysis of 43 patients
A. Flores-Chavez1, W.-F. Ng2, A. Alunno3, N. Inanc4, C. Feijoo-Massó5, R. Seror6, G. Hernandez-Molina7, V. Devauchelle-Pensec8, B. Hofauer9, S.G. Pasoto10, A. Robles11, M. Akasbi12, M. López-Dupla13, S. Retamozo14, M. Bandeira15, V.C. Romão16, F. Carubbi17, D. Loaiza-Cabello18, J.-S. García-Morillo19, M. Benegas20, M. Sánchez21, Á. Muxí22, D. Fuster23, J. Sellarés24, X. Mariette25, M. Ramos-Casals26, P. Brito-Zéron27
- Sjögren Syndrome Research Group, GRESS-AGAUR, Barcelona; Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, and University of Barcelona, Spain. ale.floreschavez@hotmail.com
- The United Kingdom Primary Sjögren's Syndrome Registry, UK.
- Department of Life, Health & Environmental Sciences, University of L'Aquila and Internal Medicine and Nephrology Unit, Department of Medicine, ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
- Marmara University, School of Medicine, Istanbul, Turkey.
- Internal Medicine, Hospital Parc Taulí, Sabadell, Spain.
- Université Paris-Saclay, Paris, France.
- Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de Mexico, Mexico.
- Cavale Blanche-University hospital, Brest, France.
- University Hospital Klinikum rechts der Isar, Germany.
- Rheumatology Division, Hospital das Clinicas HCFMUSP, Facultades de Medicina da Universidade de São Paulo, Brazil.
- Internal Medicine, Hospital La Paz, Madrid, Spain.
- Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain.
- Department of Internal Medicine, Hospital Universitari Joan XXIII, Tarragona, Spain.
- Sjögren Syndrome Research Group, GRESS-AGAUR, Barcelona, Spain.
- Rheumatology Department, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa (CAML), Lisboa, and Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, CAML, Lisboa, Portugal.
- Rheumatology Department, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa (CAML), Lisboa, and Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, CAML, Lisboa, Portugal.
- Department of Life, Health & Environmental Sciences, University of L'Aquila and Internal Medicine and Nephrology Unit, Department of Medicine, ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
- Systemic Autoimmune and Rare Diseases Unit, Internal Medicine Department, Hospital Virgen del Rocio, Seville, Spain.
- Systemic Autoimmune and Rare Diseases Unit, Internal Medicine Department, Hospital Virgen del Rocio, Seville, Spain.
- Radiology Department, Centre de Diagnòstic per la Imatge Clínic, Hospital Clínic of Barcelona, Spain.
- Radiology Department, Centre de Diagnòstic per la Imatge Clínic, Hospital Clínic of Barcelona, Spain.
- Department of Nuclear Medicine, University of Barcelona, Hospital Clínic, Barcelona, Spain.
- Department of Nuclear Medicine, University of Barcelona, Hospital Clínic, Barcelona, Spain.
- Respiratory Institute, Hospital Clínic, University of Barcelona, Spain.
- Université Paris-Saclay, Paris, France.
- Sjögren Syndrome Research Group, GRESS-AGAUR, Barcelona; Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, and University of Barcelona, Spain.
- Research and Innovation Group in Autoimmune Diseases, Milenium Balmes-Hospital CIMA Sanitas, Barcelona, Spain.
on behalf of the Sarco-GEAS-SEMI and Sjogren Big Data Consortium
CER15915
2022 Vol.40, N°12
PI 2329, PF 2337
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PMID: 36541233 [PubMed]
Received: 02/06/2022
Accepted : 26/08/2022
In Press: 20/12/2022
Published: 20/12/2022
Abstract
OBJECTIVES:
To characterise the key epidemiological, clinical, immunological, imaging, and pathological features of the coexistence between sarcoidosis and Sjögren’s syndrome (SS).
METHODS:
All centres included in two large multicentre registries (the Sjögren Syndrome Big Data Consortium and the Sarco-GEAS-SEMI Registry) were contacted searching for potential cases of coexistence between SS and sarcoidosis seen in daily practice. Inclusion criteria were the fulfilment of the current classification criteria both for SS (2016 ACR/EULAR) and sarcoidosis (WASOG). The following features were considered for evaluating a coexisting immunopathological scenario between the two diseases: non-caseating granulomas (NCG), focal lymphocytic sialadenitis (FLS) and positive anti-Ro antibodies.
RESULTS:
We identified 43 patients who fulfilled the inclusion criteria (38 women, with a mean age of 53 years at diagnosis of SS and of 52 years at diagnosis of sarcoidosis). In 28 (65%) cases, sarcoidosis was diagnosed concomitantly with SS, or during the follow-up of patients with an already diagnosed SS, while in the remaining 15 (35%), SS was diagnosed during the follow-up of an already diagnosed sarcoidosis. Patients in whom sarcoidosis was diagnosed first showed a lower mean age (43.88 vs. 55.67 years, p=0.005) and were less frequently women (73% vs. 96%, p=0.04) in comparison with those in whom sarcoidosis was diagnosed concomitantly with SS, or during the follow-up of an already diagnosed SS. We identified the following immunopathological scenarios: a combination of NCG involving extrasalivary tissues and anti-Ro antibodies in 55% of patients, a coexistence of both pathological scenarios (extrasalivary NCG and FLS in MSGB) in 42% (with positive anti-Ro antibodies in two thirds of cases), and NCG involving salivary glands and anti-Ro antibodies in 3% of cases.
CONCLUSIONS:
We have characterised the largest reported series of patients who fulfilled the current classification criteria for both SS and sarcoidosis. This implies that sarcoidosis (and not just the presence of isolated NCG on salivary gland biopsy) may, like other systemic autoimmune diseases, coexist with SS, and that a sarcoidosis diagnosis does not preclude the development of SS in the future.
