Objective To characterise the key epidemiological, clinical, immunological, imaging, and pathological features of the coexistence between sarcoidosis and Sjogren's syndrome (SS). Methods All centres included in two large multicentre registries (the Sjogren 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. Conclusion 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.
Background/Objectives: To determine the proportion of elderly-onset sarcoidosis (EOS) and to assess the influence of age at diagnosis on pulmonary and extrapulmonary manifestations. Methods: Retrospective multicentre study using the Spanish SARCOGEAS registry, including adults with sarcoidosis diagnosed across 36 hospitals. EOS was defined as diagnosis at ≥65 years. Associations between clinical features and age were assessed using multivariable logistic and linear regression models, including sex-stratified analyses. Results: Among 2187 patients (mean age 48.6 ± 15.2 years, 61.36% women), 358 (16.37%) had EOS. In multivariable analyses, female sex (OR 1.86; β +5.02 years), White ethnicity (OR 5.43; β +4.85 years), and renal involvement (OR 2.29; β +6.86 years) were independently associated with EOS and older age at diagnosis, whereas cutaneous (OR 0.49; β −3.50 years), articular or bone (OR 0.37; β −3.10 years), and neurological involvement (OR 0.49; β −2.47 years) were associated with non-EOS and younger age at diagnosis. Exploratory sex-stratified analyses suggested additional patterns: in women, calcium metabolism abnormalities were associated with EOS (OR 1.74) and older age at diagnosis (β +4.91 years), and ear–nose–throat involvement was associated with older age at diagnosis (β +5.74 years). Regarding pulmonary involvement, CT-based Scadding stage II was associated with younger age at diagnosis (β −3.75 years) in women and CT-based Scadding stage IV with older age at diagnosis (β +6.57 years) in men. Conclusions: EOS accounted for 16.37% of sarcoidosis cases. Older age at diagnosis was associated with specific patterns of organ involvement, which changed gradually across the age spectrum, some of them differing by sex in exploratory analyses. Further studies are needed to confirm these findings.