The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry. sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) of IgG4-RD patients, respectively. There were no differences in sFLC among IgG4-RD phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher κ sFLC and λ sFLC. The AUC for κ sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for κ sFLC and κ/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels. sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.
Background: Notwithstanding the frequent coexistence of autoimmune thyroid disease (ATD) and primary Sjögren’s Syndrome (SS), it is still unknown how often this association is studied along with its clinical impact. Objective: This study aimed to describe real-world screening practices for ATD in patients with SS and evaluate clinical outcomes of patients with both diagnoses using validated activity and chronicity indexes. Methods: It is a retrospective study of 223 patients with SS attending a tertiary referral center. Patients were under rheumatology surveillance and might have attended other clinics, including internal medicine and/or endocrinology. We registered glandular and extraglandular features, serology and scored the activity (ESSDAI) and the accrual damage (SSDDI) indexes. We also identified any thyroid function tests (TFT) performed, anti-thyroid antibodies, images, and histological thyroid examinations. A single endocrinologist reviewed all data. Results: One hundred forty-nine patients had at least one set of TFT. Younger age was associated with a lack of screening (OR 0.98, 95% CI 0.95-0.99, p=0.01). Sixty-nine patients had thyroid disease, with the most common diagnosis being ATD (n=24). Patients with ATD had a lower prevalence of Ro/SSA and anti-La/SSB antibodies but similar cumulative SS activity and damage scores. Conclusion: At least one-third of our patients were not screened for thyroid disease, with these patients being the youngest. Thyroid disorders were found in about 40% of patients with SS, with ATD being the most common. Having SS/ATD did not confer the worst disease activity or damage accrual. These results highlight the importance of making treating physicians aware of screening for thyroid disease in this population.
Background:Previous studies have linked the participation of multiple chemokines and cytokines in the physiopathology of primary Sjögren’s syndrome (PSS), however data regarding their presence in tears is scarceObjectives:To evaluate a panel of chemokines/cytokines in the tears of patients with PSS and correlate them with ocular symptoms as well as objective ocular tests.Methods:We included 21 patients with PSS (EULAR/ACR criteria). A single expert ophthalmologist in dry eye evaluated the patients and assessed the tear film break-up time, Schirmer-I test, tear meniscus height, the Van Bijsterveld staining score and the SICCA Ocular Staining Score (OSS). We classified lacrimal dysfunction severity in two categories (1=mild, mild/moderate or moderate, and 2= moderate/severe and severe). We scored the ESSPRI, and ocular dryness VAS as well as the Ocular Surface Disease Index (OSDI), a 12-item scale for the assessment of symptoms related to dry eye disease and their effect on vision. Tear samples were collected using sterile tear flow strips, that were immediately frozen at -86°C until assayed. Once defrosted, the tears were extracted from the strips using a buffer containing 0.5 M NaCl and 0.5% Tween-20. We tested IFN-γ, IL-10, IL-12, IL-17A, IL-1β, IL-2, IL-21, IL-23, IL-5, IL-6, IL-8, TNF-α, BAFF, CXCL10 and CCL2 by Luminometry. We also included 21 healthy controls without dry eye, to test chemokines/cytokines that after our initial screening were meaningful.Results:Most patients were females (90.4%), mean age 59.3±13 years and median disease duration 7.9 years (0.5-27). All of them had ocular and oral symptoms. The median tear film break-up time was 6 seconds (2-9), median Schirmer-I test 6 mm (1-25), median lacrimal meniscus height 1.5 mm (0.5-2), median Van Bijsterveld staining score 10 points (2-18), median OSS 7 points (2-11), median ESPPRI score 6.7 points (2-9.2) and median ocular dryness EVA score 9 points (1-10).We did not detect most of the evaluated chemokines/cytokines with the exception of IL-8, CXCL10, and CCL2. The former was similar in both, patients and controls. PSS patients had lower levels of CXCL10 (472.8 pg/μL vs 1652 pg/μL, p=0.01) and CCL2 (1.08 pg/μL vs 9 pg/μL) than controls. Indeed, patients with worst lacrimal dysfunction severity had the lowest levels of CXCL10 (239.3 pg/μL vs. 646.2 pg/μL, p=0.02). We found correlations among CXCL10 and CCL2 (T=0.30, p=0.02) and lacrimal meniscus height (T=0.55, p=0.005), as well as with CCL2 and lacrimal meniscus height (T=0-57, p=0.01). None of the other variables were correlated.Conclusion:We identified CXCL10 and CCL2 as the main chemokines in tears of patients with PSS. CXCL10 seems to participate in the normal eye homeostasis.Disclosure of Interests:None declared
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