Construction and validation of a reproductive behavior questionnaire for female patients with rheumatic diseases.

INTRODUCTION/OBJECTIVES: Active autoimmune rheumatic diseases (ARDs) before conception increase the risk of flares and adverse pregnancy outcomes. We aimed to construct and validate a reproductive behavior questionnaire in Spanish for patients with ARDs to assess their knowledge and their reproductive behavior. METHOD: We constructed and validated a reproductive behavior questionnaire in two phases: (1) a literature review followed by interviews of reproductive-age female patients, and (2) a cross-sectional study to complete validation. Convenience sampling was carried out with 165 female patients: 65 participated in the cross-cultural adaptation phase and 100 in the validation phase. The internal consistency was evaluated by estimating Cronbach's alpha and tetrachoric correlation coefficients. Values ≥ 0.40 were considered acceptable (p < 0.05). RESULTS: The initial instrument included 38 questions. Thematic analysis identified 8 important dimensions or topics, which were combined to create the Rheuma Reproductive Behavior interview questionnaire. A final total of 41 items across 10 dimensions were obtained. The test-retest analysis showed perfect correlations in 34 of the 41 items, moderate in 6 items, and negative in one of the items. The mean age of the patients was 35.65 years (SD 9.02), and the mean time to answer the survey was 13.66 min (SD 7.1). CONCLUSIONS: The Rheuma Reproductive Behavior questionnaire showed good reliability and consistency capturing patients' reproductive health knowledge and reproductive behavior. Key Points • We designed and validated a questionnaire to assess reproductive health knowledge and reproductive behavior among female patients with ARDs. • The questionnaire was comprehensible for participants, and showed good reliability and consistency capturing reproductive knowledge and behavior. • This tool may aid in the design of strategies to improve reproductive decision-making for female patients with ARDs.

Mycophenolate mofetil and mycophenolic acid for the treatment of eosinophilic fasciitis: report of two cases and literature review.

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

Evolution of clinical, histological and serological features in a Primary Sjὄgren´s Syndrome cohort and the limitations of the current classification criteria.

OBJECTIVE: The classification and/or diagnosis of Primary Sjögren's Syndrome (PSS) requires a multidimensional approach. Although age and the duration of sicca symptoms can affect the clinical, serological and histological features found at initial evaluation, these are not considered when using classification criteria as a guide for PSS diagnosis. Our study aimed to explore if there is any relationship between the duration of symptoms and clinical, histopathological and serological findings. METHODS: An observational, retrospective study was performed. All the evaluated subjects were part of the "sicca cohort". Patients' clinical, serological and histological characteristics were assessed according to the duration of symptoms. A Receiving Operator Characteristic (ROC) curve was performed to establish the duration of symptoms (months) that predicted a PSS diagnosis. Binary regression models and odds ratios were used to evaluate the association between the duration of symptoms and the clinical, serological, and histopathological profiles. RESULTS: One hundred and sixteen patients were included; 97(83.62%) fulfilled PSS criteria. Of the 116 patients, thirty-six (31.03%) had < 15 months presenting with sicca symptoms when receiving a diagnostic approach. A duration of symptoms >15 months was associated with an altered Schirmer test (OR 2.76; 95% CI 1.15-6.61, P=0.02), low salivary flow rate (OR 3.5; 95% CI 1.34-9.13, P=0.01), ≥1 foci score (OR 1.21; 95% CI 1-1.45, P=0.04), ocular (OR 7.8; 95% CI 1.49-40.81, P=0.02) and severe oral symptoms (OR 2.61; 95% CI 1.16-5.87, P=0.02). CONCLUSION: The time of evolution of symptoms plays a fundamental role in the clinical, histological and serological profiles in PSS.

Índice de Placa Bacteriana, Índice Gingival y Prueba de Silometría en Pacientes con Síndrome de Sjögren Primario y Secundario

RESUMEN: El síndrome de Sjögren (SS) es una enfermedad crónica autoinmune presente en el 0.1 - 3.0 % de la población, en la que se encuentran involucradas las glándulas salivales, trayendo consigo manifestaciones orales como caries dental y enfermedad periodontal. El objetivo de este trabajo fue evaluar el índice de placa, el índice gingival y la prueba de sialometría en pacientes con síndrome de Sjögren primario y secundario. Fueron evaluados clínicamente 40 pacientes con SS primario (n=20) y SS secundario (n=20), ambos grupos diagnosticados con periodontitis crónica. Se registró el índice de placa de Quigley-Hein modificado por Turesky, el índice gingival de Löe y la prueba de silometría estimulada, recolectando saliva parotídea con el dispositivo Carlson-Crittenden. Los pacientes con SS primario tuvieron un índice de placa de (3,53±0,5954) e índice gingival (2,41±0,2608). En pacientes con SS secundario el índice de placa fue (1,62±0,3795) y el índice gingival (1,48±0,3994), con diferencia significativa (p=0,0001) entre ambos grupos. El flujo salival se elevó en pacientes con SS secundario. El presente estudio concluye que el índice de placa y el índice gingival fueron mayores en pacientes con SS primario comparados con pacientes con SS secundario. La prueba de sialometría fue mayor en pacientes con SS secundario.

ABSTRACT: Sjögren's syndrome (SS) is a chronic autoimmune disease present in 0.1 - 3.0 % of the population, in which the salivary glands are involved, bringing with it oral manifestations such as dental caries and periodontal disease. The aim of the present study was to evaluate the plaque index, gingival index and the sialometry test in patients with primary and secondary Sjögren's syndrome. Forty patients with primary SS (n = 20) and secondary SS (n = 20), both groups diagnosed with chronic periodontitis, were evaluated clinically. The Quigley-Hein plaque index modified by Turesky, the gingival index of Löe and stimulated sialometry test collected from parotid gland by using Carlson-Crittenden device were recorded. The patients with primary SS had plaque index (3.53 ± 0.5954) and gingival index (2.41 ± 0.2608). The patients with secondary SS had a plaque index (1.62 ± 0.3795) and gingival index (1.48 ± 0.3994), with significant difference between both groups. The present study concludes that plaque index and gingival index were higher in patients with primary SS than patients with secondary SS. Sialometry test were higher in patients with secondary SS.

Reproductive health counseling and contraceptive use in Mexican women with rheumatic diseases: a cross-sectional study.

BACKGROUND: There is an overall increased risk of adverse pregnancy outcomes and maternal morbidity in patients with most autoimmune rheumatic diseases (ARD); outcomes are generally improved when the pregnancy is planned and the disease is in control. OBJECTIVE: The objective of the present study was to describe the sexual and reproductive health characteristics and contraceptive use of Mexican women in childbearing age with ARD. METHODS: We conducted an observational, cross-sectional, and descriptive study. All non-pregnant childbearing age women with an ARD were invited to participate. A self-administered questionnaire of ten items that included questions about sexuality, use of contraceptive methods, pregnancy desire, and contraceptional counseling was applied. RESULTS: A total of 135 women were evaluated. The median age was 33 (25-39) years. Contraceptive use was referred by 49 (71%) of the patients that had sexual activity the last month, while 20 (28.9%) patients denied use. From the patients who had initiated sexual activity (N = 112), 41 (36.6%) did not use any contraceptive method, and 16 (14%) used a method classified as ineffective. The question about contraceptive counseling was answered by 112 patients. Eighty (70.4%) said they had received counseling from health-professional and 64 (57.1%) from their rheumatologist. A total of 57% of the women with teratogenic drugs did not employ a contraception method. CONCLUSION: Contraceptive use and reproductive health counseling are suboptimal in Mexican women with ARD. A high proportion of women taking teratogenic drugs did not employ a highly effective contraceptive method. Strategies to improve reproductive and sexual health are necessary.

Cognitive dysfunction in Sjögren's syndrome using the Montreal Cognitive Assessment Questionnaire and the Automated Neuropsychological Assessment Metrics: A cross-sectional study.

AIM: To describe the prevalence of cognitive impairment and the most affected cognitive domains, employing the Montreal Cognitive Assessment (MoCA) and the Automated Neuropsychological Assessment Metrics (ANAM) of a Latin American primary Sjögren's syndrome (pSS) cohort, and compare these patients to secondary Sjögren's syndrome (sSS) subjects and controls. METHODS: This was a comparative cross-sectional study of patients with a diagnosis of pSS who fulfilled the American-European Consensus Group 2002 criteria and/or American College of Rheumatology/European League Against Rheumatism 2016 criteria; clinical information was evaluated prior to cognitive evaluation, which consisted of a single session in which the MoCA and ANAM were applied. RESULTS: A total of 122 subjects were included in the analysis (51 pSS, 20 sSS and 51 controls); mean age of pSS was 56 years (SD 10.4), of which 47 (92.15%) were women. Moderate-severe cognitive impairment by MoCA was 17% in pSS, 5% in sSS, and 15% in controls, and by ANAM were 29% in pSS and 10% in sSS (P > .05). Visuospatial/executive subdomain in the MoCA was different between the pSS and the control group (P = .005). We encountered a statistically significant difference between pSS patients and control scores from the program in 6 of the 7 domains tested by the ANAM. CONCLUSION: No difference was found in the prevalence of cognitive impairment between pSS subjects and controls by MoCA. Several subdomain scores differed between groups in both scales. Evaluation of cognitive disorders in patients with SS, even in early stages of the disease, seems advisable but the best strategy is yet to be elucidated.

Anxiety and depression in reproductive age women with rheumatic diseases.

Women in reproductive age with rheumatic diseases (RD) are especially vulnerable for depression and anxiety which negatively impacts the pregnancy, birth, and RD. The purpose of this study is to describe the frequency of anxiety and depression symptoms employing the Hospital Anxiety and Depression Scale (HADS) in women in reproductive age. We conducted an observational, single-center, cross-sectional, and descriptive study in reproductive-age, non-pregnant women without a prior psychiatric diagnosis. Differences between disease groups, subscale results, and disease activity were analyzed with the Chi square, Mann-Whitney U test, or Kruskal-Wallis test. A total of 100 women were included. Mean age was 35.3 years (SD = 10.07). The most frequent diagnosis was rheumatoid arthritis (RA) with 48, followed by systemic lupus erythematosus (SLE) with 30. A total of 66 (66%) patients had an abnormal HADS score (probable or possible cases) in either subscale. More than 50% of RA patients had an abnormal HADS score. We found an association between RA disease activity groups and total HADS score (p = 0.003). Furthermore, we found a statically significant association between RA activity groups and HADS anxiety subscales group classification (p = 0.01). No differences between disease activity groups of SLE or other diseases and HADS classification or total score was found (p = 0.277). A high frequency of probable or possible cases of depression and anxiety were recognized in reproductive-age women with RD. A high RA disease activity was associated with a high total HADS score and an increased presence of anxiety symptoms.

Salivary rheumatoid factor in primary and secondary Sjögren's syndrome / Factor reumatoide salival en síndrome de Sjögren primario y secundario

Sjögren's syndrome is a chronic autoimmune disease, characterized by the presence of hyposalivation and xerophthalmia, which in addition to other factors is diagnosed by the presence of rheumatoid factor in blood. The objective of the present study is to evaluate the presence of rheumatoid factor (IgG-IgM) in the saliva of patients with primary and secondary Sjögren's syndrome. Materials and methods: Forty samples from patients with primary and secondary Sjögren's syndrome previously diagnosed by the Arthritis and Rheumatism Specialist Center of the Autonomous University of Nuevo Leon were analyzed. Samples were taken from the saliva using the Carlson-Crittenden device to evaluate the IgG-IgM immunocomplex using the ELISA method. Results: No significant difference was found between the presence of IgM in primary (0.099±0.016) and secondary Sjögren syndrome (0.098±0.017), however, a high presence of IgG was found in the group of patients with secondary Sjögren's syndrome (0.134±0.054). Conclusion: The search for diagnostic tools using salivary biomarkers has come with economic and clinical advantages, however, in the present study no significant changes were found in salivary rheumatoid factor between both groups.

El síndrome de Sjögren es una enfermedad autoinmune crónica, caracterizada por la presencia de hiposalivación y xeroftalmia, la cual además de otros factores es diagnosticada por la presencia del factor reumatoide en sangre. El objetivo del presente estudio es evaluar la presencia del factor reumatoide (IgG-IgM) en saliva parotídea de pacientes con síndrome de Sjögren primario y secundario. Materiales y métodos: Se analizaron 40 muestras de pacientes con síndrome de Sjögren primario y secundario previamente diagnosticados por el Centro de Especialistas en Artritis y Reumatismo de la Universidad Autónoma de Nuevo León, a los cuales se les tomó una muestra de saliva parotídea mediante el dispositivo Carlson-Crittenden para evaluar mediante el método ELISA el inmunocomplejo IgG-IgM. Resultados: No se encontró diferencia significativa entre la presencia de IgM en el síndrome de Sjögren primario (0.099±0.016) y secundario (0,098±0,017), sin embargo en cuanto a la presencia de la IgG se encontró elevada en el grupo de pacientes con síndrome de Sjögren secundario (0,134±0,054). Conclusión: La búsqueda de herramientas diagnósticas mediante biomarcadores salivales ha traído consigo ventajas económicas y clínicas, sin embargo en el presente estudio no se encontró un cambio significativo en el factor reumatoide salival entre ambos grupos.

Early hearing loss detection in rheumatoid arthritis and primary Sjögren syndrome using extended high frequency audiometry.

The aim of this study is to evaluate the hearing behavior of rheumatoid arthritis (RA) and primary Sjögren syndrome (PSS) patients and compare them with a healthy control group and with each other. A comparative cross-sectional study was performed with a group of 117 female RA patients, a group of 60 female PSS patients, and a 251 female healthy control group. Every subject underwent a series of studies including high-frequency audiometry, speech audiometry, and tympanometry. The high-frequency audiometry measured 250 to 16,000 Hz. The 117 patients with RA and the 60 with PSS were diagnosed according to American College of Rheumatology criteria / ACR 2010, and the validated classification of the American-European Consensus Group. Hearing loss was present in 36.8% of the RA group in 500-3000 Hz, 68.4% in 4000-8000 Hz, and 94.9% in 10,000-16,000 Hz. Hearing loss was present in 60% of the PSS group in 500-3000 Hz, 70% in 4000-8000 Hz, and 100% in 10,000-16,000 Hz. The hearing impairment prevalence of both groups was significantly different (p < 0.05) when compared with the healthy control group. We also compared the hearing thresholds between RA and PSS patients, finding a significant hearing threshold increase in 500-3000 Hz of the PSS group. This study consolidates the association between RA and PSS with hearing impairment. A deeper hearing loss was reported in PSS than in RA patients, demonstrating a greater auditory and speech recognition repercussion.

Salivary stimulation by prolonged release of pilocarpine in sjõgren’ syndrome

Introduction: Prolonged drug delivery in the oral cavity offers many advantages, such as reducing adverse effects. Pilocarpine is an FDA-approved parasympathomimetic drug for the treatment of glandular hypofunction; however, its adverse effects limit its use. Objective: To evaluate the stimulation of salivary flow by the use of pilocarpine-releasing films, as well as their effects on the symptoms of xerostomia and adverse effects in patients with Sjõgren’s syndrome (SS). Materials and methods: Hydroxypropylmethylcellulose (Methocel K4MCR) films were prepared in 1 percent acetic acid and pilocarpine was added under magnetic stirring. The pH and thickness, as well as diffusion uniformity and kinetics of drug release per cm2 were evaluated by spectrophotometry. The films were tested sublingually in 40 patients with Sjõgren’s syndrome for a period of two weeks. Changes in their salivary flow were evaluated by analyzing samples of total saliva. Additionally, patients were screened for symptoms of xerostomia and adverse effects. Results: The films had a pH of 2.91 +/- 0.035, a thickness of 0.06866 +/- 0.00152μm, and a diffusion uniformity of 91 percent per cm2. Use of the films resulted in an increase in salivary flow in both primary and secondary Sjõgren’s syndrome, but this increase was only significant in primary SS. Conclusion: Films showed optimal physicochemical properties for their administration, and proved effective in stimulating salivary flow without causing adverse effects during their administration.

Beta-2 Microglobulin in Whole Unstimulated Saliva Can Effectively Distinguish Between Sjögren's Syndrome and Non-Autoimmune Sicca Symptoms.

OBJECTIVES: This study aims to describe salivary beta-2 microglobulin (sB2M) levels in our setting and to assess the performance of sB2M for the diagnosis of Sjögren's syndrome (SS). PATIENTS AND METHODS: This cross-sectional, comparative study included 192 SS patients (2 males, 190 females; mean age 53.1 years; range 23 to 84 years) and 64 healthy controls (1 male, 63 females; mean age 46.9 years; range 21 to 82 years). Patients were divided into three groups as those with primary SS, secondary SS, and sicca non-Sjögren's syndrome (snSS). sB2M was measured by enzyme-linked immunosorbent assay in whole unstimulated saliva (ng/mL). Differences in sB2M were evaluated using the Kruskal-Wallis test. Receiver operating curves were generated to determine the performance of sB2M for distinguishing between SS and non-autoimmune snSS groups, and between SS group and healthy controls. RESULTS: The primary SS and secondary SS groups had a significantly higher concentration of sB2M than the other two groups. There was no significant difference in the concentration of sB2M between primary SS and secondary SS groups, and neither between snSS group and healthy controls. The receiver operating curve analysis for distinguishing SS and snSS showed an area under the curve of 0.661 (95% confidence interval 0.590-0.728, p=0.0001) with an optimal cutoff value of 0.582 ng/mL. Sensitivity, specificity, positive predictive value, and negative predictive value were 68.7%, 59.3%, 20.2%, and 92.7%, respectively. The reported prevalence of SS in Mexico was considered when calculating the last two values. CONCLUSION: In our setting, sB2M effectively distinguished between SS patients and non-autoimmune sicca symptoms. Including sB2M in our conventional diagnostic arsenal may assist in the evaluation of patients in whom SS is suspected; however, further studies are needed to clarify this hypothesis.

[Sjögren's syndrome (SS), a review of the subject and saliva as a diagnostic method]. / Síndrome de Sjögren (SS), revisión del tema y saliva como método diagnóstico.

Sjögren's syndrome is a chronic autoimmune disease whose main clinical manifestation is oral dryness (xerostomia) and ocular dryness (xerophthalmia). It is characterized by progressive mononuclear infiltration of the exocrine glands and can affect a variety of organ systems. The prevalence of primary Sjögren's syndrome varies from 0.01 up to 4.8%; this variability reflects differences in definition, application of diagnostic criteria, and geographic differences in age groups. The etiology of primary Sjögren's syndrome is unknown, but the interaction between genetic and environmental factors (viruses, hormones, vitamins, stress) is important. There are few reported cases of concordance in monozygotic twins, and it is common for patients with primary Sjögren's syndrome to have relatives with other autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, thyroid disease, psoriasis, and multiple sclerosis. Among the most common findings is hypergammaglobulinemia. Elevated levels of γ-globulins contain autoantibodies directed against nonspecific antigens such as rheumatoid factor, antinuclear antibodies, and cellular antigens SS-A/Ro and SS-B/La. Regarding diagnosis, there have been 11 different published criteria for Sjögren's syndrome since 1965; none have been approved by the American College of Rheumatology or the European League Against Rheumatism. The current criteria were published in 2012 jointly with the progressive advance in the knowledge of the human salivary proteome that has gained wide acceptance in Sjögren's syndrome, with the possibility of using saliva as a useful tool in both diagnosis and prognosis in this field because the analysis of salivary proteins may reflect the state of locally underlying disease of the salivary glands, which are the target organs in this disease.

Application of a prediction model for the progression of rheumatoid arthritis in patients with undifferentiated arthritis.

INTRODUCTION: Different prediction rules have been applied to patients with undifferentiated arthritis (UA) to identify those that progress to rheumatoid arthritis (RA). The Leiden Prediction Rule (LPR) has proven useful in different UA cohorts. OBJECTIVE: To apply the LPR to a cohort of patients with UA of northeastern Mexico. METHODS: We included 47 patients with UA, LPR was applied at baseline. They were evaluated and then classified after one year of follow-up into two groups: those who progressed to RA (according to ACR 1987) and those who did not. RESULTS: 43% of the AI patients developed RA. In the RA group, 56% of patients obtained a score ≤ 6 and only 15% ≥ 8. 70% who did not progress to RA had a score between 6 and ≤ 8. There was no difference in median score of LPR between groups, p=0.940. CONCLUSION: Most patients who progressed to RA scored less than 6 points in the LPR. Unlike what was observed in other cohorts, the model in our population did not allow us to predict the progression of the disease.

Epidemiology of rheumatic diseases. A community-based study in urban and rural populations in the state of Nuevo Leon, Mexico.

OBJECTIVE: To estimate the prevalence of rheumatic diseases in rural and urban populations using the WHO-ILAR COPCORD questionnaire. METHODS: We conducted a cross-sectional home survey in subjects > 18 years of age in the Mexican state of Nuevo Leon. Results were validated locally against physical examination in positive cases according to an operational definition by 2 rheumatologists. We used a random, balanced, and stratified sample by region of representative subjects. RESULTS: We surveyed 4713 individuals with a mean age of 43.6 years (SD 17.3); 55.9% were women and 87.1% were from urban areas. Excluding trauma, 1278 individuals (27.1%, 95% CI 25.8%-28.4%) reported musculoskeletal pain in the last 7 days; the prevalence of this variable was almost twice as frequent in women (33% vs 17% in men); 529 (11.2%) had pain associated with trauma. The global prevalence of pain was 38.3%. Mean pain score was 2.4 (SD 3.4) on a pain scale of 0-10. Most subjects classified as positive according to case definition (99%) were evaluated by a rheumatologist. Main diagnoses were osteoarthritis in 17.3% (95% CI 16.2-18.4), back pain in 9.8% (95% CI 9.0-10.7), undifferentiated arthritis in 2.4% (95% CI 2.0-2.9), rheumatoid arthritis in 0.4% (95% CI 0.2-0.6), fibromyalgia in 0.8% (95% CI 0.6-1.1), and gout in 0.3% (95% CI 0.1-0.5). CONCLUSION: This is the first regional COPCORD study in Mexico performed with a systematic sampling, showing a high prevalence of pain. COPCORD is a useful tool for the early detection of rheumatic diseases as well as for accurately referring patients to different medical care centers and to reduce underreporting of rheumatic diseases.