Enamel fluorosis related with fluoride-containing water ingestion and urinary excretion in schoolchildren.

Background: Natural water sources are considered as the major environmental exposure of fluoride, resulting in increased prevalence of enamel fluorosis. This type of natural exposure should be permanently monitored to avoid the interactions with other non-natural fluoride sources. We evaluated the prevalence of enamel fluorosis in Colombian schoolchildren and its relationship with fluoride-containing water ingestion exposure dose and urinary fluoride excretion. Material and Methods: We included 923 schoolchildren aged 7-12 years residing in eight municipalities in Colombia. Sampling of consumption water was performed in major aquifers used for daily supply. Samples were collected in 98 polyethylene containers and refrigerated until analysis. Water and urine fluoride concentrations were measured using the fluoride selective electrode method. Enamel fluorosis was evaluated using Thylstrup and Ferjerskov Index (TFI). Demographic and anthropometric characteristics were assessed. Besides, other exposures to non-natural fluoride were also evaluated. Logistic regression was applied for multiple analyses. Results: The median fluoride concentration in water and urine samples was 10.5 mg/L and 0.63 mg/L respectively, with the highest value found in Algarrobo-Magdalena, and the lowest value found in Manzanares-Caldas. The overall prevalence of enamel fluorosis was 86.1%, being more frequent the mild codes with TFI-1 to TFI-2. The highest prevalence was found in Margarita-Bolívar and Manzanares-Caldas, and the most severe codes (TFI-5 to TFI-9) were detected in Manzanares-Caldas. The multiple analysis revealed water ingestion exposure dose, urinary excretion, involuntary intake of toothpaste, amount of table salt consumption and sex as significant factors (p< 0.001). Conclusions: The fluoride ingestion exposure dose and its subsequent urinary excretion could be used as estimators of past fluoride exposure, explaining the current prevalence of enamel fluorosis in Colombian schoolchildren. Key words:Fluoride, groundwater ingestion, enamel fluorosis, prevalence, severity.

Therapy for Chikungunya Arthritis: A Study of 133 Brazilian Patients.

Chikungunya fever is a global vector-borne viral disease. Patients with acute chikungunya are usually treated symptomatically. The arthritic phase may be self-limiting. However, many patients develop extremely disabling arthritis that does not improve after months. The aim of this study was to describe the treatment of chikungunya arthritis (CHIKA) patients. A medical records review was conducted in 133 CHIKA patients seen at a rheumatology practice. Patients were diagnosed by clinical criteria and confirmed by the presence of anti-chikungunya IgM. Patients were treated with methotrexate (20 mg/week) and/or leflunomide (20 mg/day) and dexamethasone (0-4 mg/day) for 4 weeks. At baseline visit and 4 weeks after treatment, Disease Activity Score 28 (DAS28) and pain (using a visual analog scale) were ascertained. Five months after the end of treatment, patients were contacted to assess pain, tender joint count, and swollen joint count. The mean age of patients was 58.6 ± 13.7 years, and 119 (85%) were female. After 4 weeks of treatment, mean (SD) DAS28-erythrocyte sedimentation rate (6.0 [1.2] versus 2.7 [1.0], P < 0.001) and pain (81.8 [19.2] to 13.3 [22.9], P < 0.001) scores significantly decreased. A total of 123 patients were contacted 5 months after the end of treatment. Pain score, tender joint count, and swollen joint count significantly declined after 4 weeks of treatment, and the response was sustained for 5 months. In this group of patients with CHIKA, 4-week treatment induced a rapid clinical improvement that was maintained 5 months after the end of therapy; however, the contribution of treatment to these outcomes is uncertain.

Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders.

Relapsing polychondritis (RPC) is an uncommon autoimmune systemic disease characterized by recurrent inflammation of the cartilage tissue. It can occur alone or in association with other autoimmune diseases, vasculitis, or hematologic disorders. However, the association of RPC with dermatomyositis is extremely rare. Herein, we present a case of a 38-year-old man who developed concurrent RPC and clinically amyopathic dermatomyositis (CADM) manifested by auricular chondritis, nasal chondritis, polyarthritis, gottron papules, fingertip papules, skin biopsy consistent with dermatomyositis, and positive antimelanoma differentiation-associated gene 5 (MDA5) antibodies. RPC features resolved with corticosteroids, but CADM manifestations were resistant to corticosteroids, cyclophosphamide, azathioprine, and hydroxychloroquine. Subsequent therapy with rituximab was effective to control CADM manifestations. This case highlights the importance of recognizing CADM as part of the autoimmune diseases linked with RPC and maintaining a high level of awareness to initiate effective therapy to avoid the long-term complications associated with these conditions.

Short- and mid-term outcomes in systemic lupus erythematosus patients presenting with disease exacerbation after SARS-CoV-2 mRNA vaccination: A cohort study from Puerto Rico.

OBJECTIVE: To determine if SARS-CoV-2 mRNA vaccination has an impact on the clinical course of systemic lupus erythematosus (SLE). METHODS: Puerto Ricans with SLE who received mRNA COVID-19 vaccines were studied. Demographic parameters, clinical manifestations, disease activity (per Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), disease damage (per Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), emergency room visits, hospitalizations, and pharmacologic therapy were determined. Baseline variables (prior to vaccination) were compared between patients with and without exacerbation after SARS-CoV-2 vaccination. Among those with exacerbation, clinical outcomes were determined up to 1 year after vaccination. RESULTS: Of the entire cohort (n = 247), 14 (5.7%) had post-vaccination exacerbations. Photosensitivity, oral ulcers, anti-Ro antibodies, higher SLEDAI score, and corticosteroids exposure were associated with post-vaccination flares. Among those with post-vaccination flares, 10 (71.4%) had major organ involvement. No significant differences were observed for mean SLEDAI scores, emergency room visits, hospitalizations, disease damage, and exposure to immunosuppressive drugs before and after SARS-CoV-2 mRNA vaccination. At 12 months of follow-up, all patients were fully controlled without evidence of active disease. CONCLUSION: In our group of SLE patients, 5.7% had a disease flare after SARS-CoV-2 mRNA vaccination. Most had exacerbations involving major organs/systems. Mucocutaneous manifestations, anti-Ro antibodies, disease activity, and corticosteroids were associated with flares. Awareness of these factors and the possibility of a major lupus flare after vaccination with COVD-19 vaccines is critical to provide timely and effective therapy.

Pathogenesis of chronic chikungunya arthritis: Resemblances and links with rheumatoid arthritis.

Chikungunya virus (CHIKV) infection results from transmission by the mosquito vector. Following an incubation period of 5-7 days, patients develop an acute febrile illness, chikungunya fever (CHIKF), characterized by high fevers, maculopapular rash, headaches, polyarthritis/arthralgias, myalgias, nausea, vomiting, and diarrhea. Joint pain is often severe, and most often involves the hands, the wrists, the ankles, and the metatarsal-phalangeal joints of the feet. Many patients recover within several weeks, but up to 50% develop chronic joint pain and swelling for more than 12 weeks, then we refer to these symptoms as chronic chikungunya arthritis (CCA). The pathogenesis of CCA is not well understood. In this article, we suggest that mesenchymal stem cells (MSCs) may play an important role in this pathogenesis. This heterogeneous group of multipotent cells, morphologically similar to fibroblasts, may undergo epigenetic changes capable of generating aberrant progenies. However, we believe that there is no need for a latent infection. In our pathogenic hypothesis, CHIKV infection of MSCs would cause epigenetic changes both in MSCs themselves and in their progenies, without the need for reactivation of dormant viruses.

Association of Systemic Lupus International Collaborating Clinics Frailty Index With Damage in Systemic Lupus Erythematosus Patients: Results From a Multiethnic, Multicenter US Cohort of Patients With Lupus.

OBJECTIVE: To evaluate the association between the Systemic Lupus International Collaborating Clinics frailty index (SLICC-FI) and damage accrual in systemic lupus erythematosus (SLE) patients. METHODS: Patients from the multiethnic, multicenter LUpus in MInorities, NAture versus nurture (LUMINA) cohort were included. Damage was ascertained with the SLICC/American College of Rheumatology Damage Index (SDI) at last visit (range 0-51). The first visit in which the SLICC-FI score could be derived was considered as the baseline (range 0-1). Univariable and multivariable negative binomial regression models were performed to determine the association between the baseline SLICC-FI score (per 0.05 increase) and the change in the SDI score (difference between last and baseline SDI score), adjusted for sex, age at diagnosis, ethnicity, insurance, prednisone daily dose, and antimalarial and immunosuppressive drug use at baseline. Age and sex were included a priori in the multivariable model; the other variables were included if they reached P < 0.10 in the univariable models. RESULTS: Of the 503 patients included, 454 (90.3%) were female, with a mean ± SD age of 37.1 ± 12.5 years at diagnosis. The mean ± SD baseline SLICC-FI score was 0.26 ± 0.06. The mean ± SD baseline SDI score was 0.6 ± 1.0, and the mean ± SD change in the SDI score was 1.9 ± 2.2. Higher SLICC-FI scores at baseline (per 0.05 increase) were associated with greater damage accrual in the multivariable model after adjustment for possible confounders (incidence rate ratio 1.20 [95% confidence interval 1.08-1.33], P = 0.0015). CONCLUSION: The SLICC-FI is associated with damage accrual in SLE patients from a multiethnic cohort, supporting the importance of this index in the evaluation of SLE patients, combining several aspects of their disease.

Health-Related Quality of Life in Adults With Adolescent- and Adult-Onset Systemic Lupus Erythematosus: A Longitudinal Study of a Multiethnic US Cohort.

OBJECTIVE: The long-term impact of childhood-onset systemic lupus erythematosus (SLE) on health-related quality of life (HRQoL) in adult SLE patients in comparison to those with adult-onset SLE is unknown. We aim to examine and compare HRQoL trajectories in adults with adolescent- and adult-onset SLE. METHODS: Patients enrolled in the LUpus in MInorities: NAture versus Nurture cohort were included. Adolescent-onset SLE were those diagnosed before 24 years of age, and adult-onset SLE were those diagnosed otherwise. Sociodemographic, clinical, medications, behavioral/psychological, and functioning data were obtained. Longitudinal trajectories of the physical component summary (PCS) and the mental component summary (MCS) Short Form 36 health survey scores were compared between the groups using a linear mixed model accounting for time-dependent and independent covariates. RESULTS: A total of 470 SLE patients were included (95 with adolescent-onset SLE and 375 with adult-onset SLE). The mean ± SD age at diagnosis was 19.7 ± 2.8 years in the adolescent group and 39.3 ± 11.0 years in the adult group. The baseline PCS scores were higher (better physical functioning) in adolescent-onset SLE than in adult-onset SLE (38.9 versus 34.3, respectively; P < 0.001); however, the baseline MCS scores were comparable between the groups (41.4 versus 40.5, respectively; P = 0.53). The HRQoL improved equally in both groups with no statistically significant difference within and between the groups (last mean PCS and MCS scores 43.9 and 45.3 in adolescent-onset SLE; 38.1 and 43 in adult-onset SLE). CONCLUSIONS: Adults with adolescent-onset SLE exhibited better physical functioning than those in the adult SLE group, despite more severe disease; noteworthy, HRQoL was below the general US population, despite clinically meaningful improvement in HRQoL over time in both groups.

Monarticular rheumatoid arthritis of the elbow.

Rheumatoid arthritis (RA) is a chronic autoimmune disease characterised by symmetric inflammatory polyarthritis. However, RA limited to a single joint is extremely rare. Here, we report a middle-aged woman who presented with insidious right elbow arthritis. She had no other peripheral joint pain, tenderness or swelling. She had high-positive anti-cyclic citrullinated peptide antibodies. An MRI of the right elbow showed capsular distension, joint effusion and bone marrow oedema. Synovial biopsy revealed hyperplasia with lymphoplasmacytic infiltrate consistent with RA. Therapy with methotrexate 7.5 mg orally weekly was effective to control her inflammatory arthritis. This case highlights the relevance of synovial tissue analysis for patients presenting with chronic inflammatory monarthritis when the cause is not clinically evident, and the importance of considering RA even in the absence of polyarticular involvement. Delayed diagnosis and treatment of inflammatory monarthritis can lead to joint destruction and disability.

New-Onset Systemic Lupus Erythematosus after mRNA SARS-CoV-2 Vaccination.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease resulting from the interaction of genetic and environmental factors. In addition, some antiviral vaccines have been associated with the onset of SLE. Few cases of SLE occurring after SARS-CoV-2 mRNA have been reported. Herein, we report the case of a 27-year-old woman with type I diabetes mellitus and family history of SLE who presented with symmetric inflammatory polyarthritis of the proximal interphalangeal joints, metacarpophalangeal joints, wrists, knees, and ankles two weeks after receiving the second dose of the SARS-CoV-2 mRNA-1273 vaccine. Laboratory results revealed positive antinuclear, anti-dsDNA, anti-Ro, and anti-La/SSB antibodies and low C4 levels. She was initially treated with low-dose prednisone and hydroxychloroquine. Hydroxychloroquine was discontinued after she developed an urticarial rash. Subsequently, mycophenolate mofetil was added after she developed proteinuria. This case highlights the importance of considering the diagnosis of SLE in patients who present with inflammatory polyarthritis after COVID-19 vaccination.

Extensive Longitudinal Transverse Myelitis after Influenza A Virus Infection in a Patient with Systemic Lupus Erythematosus.

Transverse myelitis (TM) is a rare complication seen in 1-2% of patients with systemic lupus erythematosus (SLE). Viral infections may cause TM in these patients by causing a dysregulation of their immune system. We report a 30-year-old woman with SLE who had influenza A and a few days later developed urinary retention, bilateral lower extremity paralysis, upper extremity weakness, and optic nerve and macular edema. Magnetic resonance imaging showed C4-T12 hyperintense lesions consistent with TM. She was treated with intravenous methylprednisolone 1 g daily for 3 days and then 6 cycles of monthly intravenous cyclophosphamide. This treatment was followed by oral prednisone. She had a remarkable clinical response. Visual acuity improved to her baseline, and muscle strength almost fully recovered. Clinicians should be aware that viral infections, including influenza, may induce TM. This case highlights the importance of early recognition and prompt treatment with immunosuppressive drugs in such cases.

Does hydroxychloroquine improve patient reported outcomes in patients with lupus?

BACKGROUND: Hydroxychloroquine (HCQ) use is associated with less disease activity, flares, damage and improved survival in Systemic Lupus Erythematosus (SLE). However, its effect on patient reported health outcomes (PROs) such as quality of life (QOL) is not known. METHODS: International data from Study on Outcomes of Lupus (SOUL) from 2,161 SLE patients were compared by HCQ use. Disease activity and damage were assessed using SELENA-SLEDAI and SLICC-ACR/SDI. QOL was evaluated using LupusPRO and Lupus Impact Tracker (LIT). Linear regression analyses were performed with LupusPRO summary scores health related HRQOL, non-health related NHRQOL and LIT as dependent and HCQ use as independent variable. Analyses were undertaken to test mediation of effects of HCQ use on QOL through disease activity. RESULTS: Mean age was 40.5 ± 12.8 years, 93% were women. Sixty-three (1363/2161) percent were on HCQ. On univariate analysis, HCQ use was associated with (a) better QOL (LupusPRO-HRQOL: ß 6.19, 95% CI 4.15, 8.24, P ≤ 0.001, LupusPRO NHRQOL: ß 5.83, 95% CI 4.02, 7.64, P ≤ 0.001) and less impact on daily life (LIT: ß -9.37, 95% CI -12.24, -6.50, P ≤ 0.001). On multivariate and mediational analyses, the effects of HCQ on QOL were indirectly and completely mediated through disease activity. CONCLUSIONS: HCQ use in SLE is associated with better patient reported health outcomes (LupusPRO-HRQOL and NHRQOL and impact on daily life), and the effects are mediated through disease activity. This information can facilitate patients and physician's communication with decision-making regarding the use of HCQ for SLE management.

Isolated intestinal polyarteritis nodosa in an elderly patient.

Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.

Clinical Manifestations and Outcomes in Disease-Modifying Antirheumatic Drug-Naive Adult Patients with Chronic Chikungunya Arthritis.

Most studies on chronic chikungunya virus (CHIKV) arthritis include patients treated with disease-modifying antirheumatic drugs (DMARDs), likely altering the expression of clinical manifestations and outcome. Therefore, we sought to evaluate the clinical features and correlates in DMARD-naive patients with chronic CHIKV arthritis. We conducted a case-control study in adult patients with serologically confirmed CHIKV infection in Puerto Rico. Demographic features, clinical manifestations, comorbidities, disease activity (per Clinical Disease Activity Index [CDAI]), functional status (per Health Assessment Questionnaire Disability Index [HAQ-DI]), and pharmacologic treatment were ascertained. Patients with and without chronic CHIKV arthritis were compared. Furthermore, a sub-analysis was performed among patients with chronic CHIKV who presented with mild disease activity versus moderate-to-high disease activity at study visit. In total, 61 patients were studied; 33 patients had chronic arthritis and 28 had resolved arthritis. Patients with chronic arthritis had significantly more diabetes mellitus, chronic back pain, and fever, tiredness, and myalgias on the acute phase. The mean (SD) HAQ score was 0.95 (0.56), and 57.6% had moderate-to-high disease activity. Patients with moderate-to-high disease activity had higher scores in overall HAQ-DI and HAQ-DI categories (dressing and grooming, arising, hygiene, reaching, and activities) than in those with mild activity. In conclusion, in this group of DMARD-naive patients with chronic CHIKV arthritis, nearly 58% had moderate-to-high disease activity and had substantial functional disability. Diabetes mellitus, chronic back pain, and some manifestations on acute infection were associated with chronic CHIKV arthritis.

Achievement of the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology Criteria for Systemic Lupus Erythematosus and Amount of Damage Accrual: Results From a Multiethnic Multicenter Cohort.

OBJECTIVE: To determine the difference in outcomes in patients who achieved or did not achieve the 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria. METHODS: Patients from the LUpus in MInorities, NAture versus nurture (LUMINA) cohort were included. For these analyses, we compared those patients who achieved the 2019 EULAR/ACR criteria any time during follow-up to those who did not. The predefined outcomes were the last Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI) scores and survival. Univariable and multivariable negative binomial regression models were performed; adjustment models were based on a forward selection process. RESULTS: In total, 98 of 640 patients never achieved the 2019 EULAR/ACR criteria. There was no difference in mean baseline SDI score among the patients who did not achieve the criteria compared to those who did. Conversely, the mean ± SD SDI score at last visit was lower for those who never achieved the criteria (1.2 ± 1.7 versus 2.0 ± 2.3, P = 0.0004). In the final adjusted model, the SDI score at last visit was 31% lower for those who never achieved the criteria (P = 0.0077). These patients were also more likely to survive, but this was not statistically significant. CONCLUSION: In our cohort, patients who did not achieve the 2019 EULAR/ACR criteria accrued less damage, suggesting that these criteria could allow us to identify a subset of patients with more severe disease than allowed by previous criteria.

Applying the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology Lupus Criteria to Patients From the LUMINA Cohort: Results From the Multiethnic, Multicenter US Cohort.

OBJECTIVE: To evaluate the performance of the 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria for systemic lupus erythematosus (SLE) in terms of earlier SLE classification in comparison to the ACR or the Systemic Lupus International Collaborating Clinics (SLICC) criteria. METHODS: Patients from a multiethnic, multicenter cohort, the Lupus in Minorities: Nature versus Nurture cohort, where SLE was defined using the 1982/1997 ACR criteria were included. Demographic, clinical, and immunologic criteria were compared among the 2019 EULAR/ACR and the 1982/1997 ACR and the 2012 SLICC timing categories. RESULTS: The 2019 EULAR/ACR criteria allowed an earlier SLE classification in 13.3% of patients (mean 0.66 years) and 15.3% of patients (mean 0.63 years) compared to the 1982/1997 ACR and the 2012 SLICC criteria, respectively. Patients accruing the 2019 EULAR/ACR criteria later than the 1982/1997 ACR criteria had a lower disease activity, were less likely to have positivity to anti-double-stranded DNA and anti-Sm, as well as lupus nephritis classes II or V; they were more likely to have mucocutaneous manifestations, serositis, leukopenia, and antiphospholipid antibodies positivity. These differences were less pronounced when compared to the 2012 SLICC criteria CONCLUSION: The 2019 EULAR/ACR criteria classified SLE patients earlier than the 2 other criteria sets in real-life clinical practice scenarios in a relatively small proportion of the patients. However, these criteria could allow earlier classification of a subset of patients with a more severe disease.

Characterization of Actinomycetes Strains Isolated from the Intestinal Tract and Feces of the Larvae of the Longhorn Beetle Cerambyx welensii.

Actinomycetes constitute a large group of Gram-positive bacteria present in different habitats. One of these habitats involves the association of these bacteria with insects. In this work, we have studied twenty-four actinomycetes strains isolated from the intestinal tract and feces from larvae of the xylophagous coleopteran Cerambyx welensii and have shown that seventeen strains present hydrolytic activity of some of the following substrates: cellulose, hemicellulose, starch and proteins. Fourteen of the isolates produce antimicrobial molecules against the Gram-positive bacteria Micrococcus luteus. Analysis of seven strains led us to identify the production of a wide number of compounds including streptanoate, alpiniamide A, alteramides A and B, coproporphyrin III, deferoxamine, demethylenenocardamine, dihydropicromycin, nocardamine, picromycin, surugamides A, B, C, D and E, tirandamycins A and B, and valinomycin. A significant number of other compounds, whose molecular formulae are not included in the Dictionary of Natural Products (DNP), were also present in the extracts analyzed, which opens up the possibility of identifying new active antibiotics. Molecular identification of ten of the isolated bacteria determined that six of them belong to the genus Streptomyces, two of them are included in the genus Amycolatopsis and two in the genus Nocardiopsis.

Incident arterial vascular events in a cohort of Puerto Ricans with rheumatoid arthritis.

OBJECTIVE: The increased morbidity and mortality associated with cardiovascular events in patients with rheumatoid arthritis has been linked to traditional and nontraditional factors. However, these factors vary among different ethnicities. Few studies have described these features in Hispanic populations. Thus, we determined the clinical correlates of arterial vascular events in Hispanics from Puerto Rico. METHODS: A cross-sectional study was performed in a cohort of 405 Puerto Ricans with rheumatoid arthritis. Demographic parameters, health-related behaviors, clinical manifestations, disease activity (per Disease Activity Score 28), functional status (per Health Assessment Questionnaire), comorbidities, and pharmacotherapy were compared in patients with and without incident arterial vascular events. The latter was defined as the occurrence of myocardial infarction, angina pectoris, vascular procedures for coronary artery disease, stroke, or peripheral artery disease. Study groups were analyzed using bivariate and multivariate analyses. RESULTS: Of the total study population, 87.2% were woman. The mean age at study visit was 56.1 ± 13.9 years, and the mean disease duration was 15.0 ± 13.2 years. Arterial vascular events occurred in 43 patients (10.6%). In the multivariate analysis adjusted for age and sex, arterial hypertension, dyslipidemia, metabolic syndrome, extra-articular manifestations, higher Health Assessment Questionnaire score, and number of hospitalizations were associated with arterial cardiovascular events. CONCLUSION: In this cohort of Puerto Ricans with rheumatoid arthritis, traditional and nontraditional factors, particularly extra-articular manifestations and functional disability, were associated with arterial vascular events. Awareness of these associations may help to implement clinical strategies in this group of rheumatoid arthritis patients at risk of arterial vascular events.

Characterization of a subset of patients with primary Sjögren's syndrome initially presenting with C3 or C4 hypocomplementemia.

OBJECTIVE: This study aimed to determine the association of C3 and C4 hypocomplementemia at the diagnosis of primary Sjögren's syndrome (pSS) with clinical manifestations, disease activity, and disease damage. METHODS: A cross-sectional study was conducted in 94 Puerto Ricans with pSS. Patients were aged ≥21 years and met the 2012 American College of Rheumatology Classification Criteria for pSS. Demographic characteristics, health-related features, cumulative extraglandular manifestations, serologic tests at pSS diagnosis, comorbidities, disease activity (per European League Against Rheumatism Sjögren's Syndrome Disease Activity Index [ESSDAI]), disease damage (per Sjögren's Syndrome Disease Damage Index [SSDDI]), and pharmacologic therapy were determined. Serum C3 and C4 levels were measured at pSS diagnosis by immunoturbidimetry. Patients with and without hypocomplementemia were analyzed using bivariate and multivariate logistic regression analyses adjusted for age, sex, and disease duration. RESULTS: The mean age and disease duration of the study population were 52.4±12.4 years and 5.9±4.8 years, respectively; of the total study population, 94% were female. C3 and C4 hypocomplementemia were observed in 9.6% and 13.8% of the patients, respectively. In the multivariate analysis, C3 hypocomplementemia was associated with leukocytoclastic vasculitis, interstitial lung disease, higher SSDDI score, and exposure to rituximab. C4 hypocomplementemia was associated with leukocytoclastic vasculitis, interstitial lung disease, and higher ESSDAI and SSDDI scores. CONCLUSION: In this population of patients with pSS, low C3 and C4 levels at diagnosis were associated with extraglandular manifestations such as vasculitis and interstitial lung disease, as well as disease activity and damage accrual. These results suggest that complements C3 and C4 have clinical and prognostic value in patients with pSS.