Subclinical synovitis impact on the progression of lupus joint disease: A 10-year longitudinal multicenter study.

OBJECTIVE: To determine the effect of subclinical synovitis on the progression of joint disease in a cohort of patients with systemic lupus erythematosus over a mean follow-up of 10 years. METHODS: A longitudinal follow-up of 96 patients diagnosed with lupus was performed. All patients were considered clinically free of joint disease or with minimal joint impairment at baseline and were studied through ultrasound study of their dominant hand to assess the prevalence of subclinical synovitis. Now, over 10 years after we contacted them and reviewed their evolution to determine the impact of had or had not been diagnosed with subclinical synovitis in their current joint condition. RESULTS: Thirty-one of the 91 reached patients developed clinical progression in their joint manifestations (at least one ordinal degree of worsening). Of these, 23 (74,9%) had demonstrated subclinical synovitis at baseline. In the group of patients who did not progress clinically, 46 (76,6%) did not have this finding at the start of follow-up (p < .01, OR 9,44 95%CI 3,46-25,74). The patients in whom clinical progression was demonstrated had worse combined ultrasound scores than the rest of the patients: 6,41 SD 1,45 vs. 1,15 SD 0,97 (p < .01). CONCLUSIONS: The finding of subclinical synovitis in patients with systemic lupus erythematosus is associated with the development of joint disease progression both clinically and ultrasonographically.

Off-label use of rituximab in patients with systemic lupus erythematosus with extrarenal disease activity: a retrospective study and literature review.

Introduction: Off-label rituximab is commonly used for patients with systemic lupus erythematosus (SLE) with extrarenal disease activity. Methods: The outcomes and tolerability of rituximab in adult patients with non-renal SLE treated at our hospital from 2013 to 2020 were described. Patients were followed-up until December 2021. Data were retrieved from electronic medical records. Response was classified into complete, partial or no response according to the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2 K)-based definitions. Results: A total of 44 cycles were administered to 33 patients. Median age was 45 years and 97% were female. Median follow-up was 5.9 years (IQR 3.7-7.2). The most frequent symptoms that motivated rituximab use were thrombocytopenia (30.3%), arthritis (30.3%), neurological manifestations (24.2%) and cutaneous lupus (15.2%). After most treatment cycles a partial remission was achieved. The median SLEDAI-2 K score declined from 9 (IQR 5-13) to 1.5 (IQR 0-4) (p < 0.00001). The median number of flares significantly declined after receiving rituximab. Platelet counts significantly improved in patients with thrombocytopenia and patients with skin disorders or neurological manifestations also had a partial or complete response. Only 50% of patients with a predominant joint involvement had either a complete or a partial response. The median time to relapse after the first cycle was 1.6 years (95% CI, 0.6-3.1). Anti-dsDNA levels decreased significantly after rituximab from a median of 64.3 (IQR 12-373.9) to 32.7 (IQR 10-173), p = 0.00338. The most frequent adverse events were infusion-related reactions (18.2%) and infections (57.6%). All patients needed further treatment to maintain remission or to treat new flares. Conclusion: A partial or complete response was documented after most rituximab cycles in patients with non-renal SLE. Patients with thrombocytopenia, neurolupus, and cutaneous lupus had better response than those with a predominant joint involvement.

The SLICC-FI Predicts Damage Accrual in SLE Patients. Data From the Almenara Lupus Cohort.

OBJECTIVE: To evaluate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI) as a predictor of damage accrual in a primarily Mestizo SLE patient cohort. METHODS: Patients from a single-center prevalent cohort were included. Damage accrual was defined as the increase in the SLICC/American College of Rheumatology (ACR) damage index (SDI) scores between the baseline and the last visits. The SLICC-FI was measured at baseline. Univariable and multivariable Cox regression models were performed to determine the association between the baseline SLICC-FI (per 0.05 increase) and the increase in the SDI, adjusted for possible confounders. Alternative analyses using negative binomial regression models including the difference between the last and the first SDI as outcome were performed. RESULTS: Of the 265 patients included, 248 (93.6%) were female with mean (SD) age of 35.1 (13.6) years at diagnosis. At baseline, mean (SD) SLE disease duration was 7.3 (6.5) years, SDI was 1.0 (1.2) and the SLICC-FI was 0.22 (0.05). After a mean (SD) of 5.2 (2.2) years of follow-up, the SDI increased in 126 (47.5%) patients, and the final mean (SD) SDI score was 1.7 (1.7). Higher SLICC-FI scores at baseline predicted greater damage accrual in the univariable analysis [Hazard Ratio (HR) =1.38, (CI95% 1.16-1.65); p < 0.001] and in the multivariable model, after adjustment for possible confounders [HR = 1.30 (CI95% 1.02-1.66); p = 0.033]. CONCLUSION: SLICC-FI predicts the occurrence of damage accrual in a prevalent SLE Latin-American cohort with short or long disease duration, supporting the relevance of this index in the evaluation of SLE patients.

Human Papilloma Virus Vaccination in Patients with Rheumatic Diseases in France: A Study of Vaccination Coverage and Drivers of Vaccination.

Objectives: To describe human papillomavirus (HPV) vaccination practices in adolescent girls with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA) and to identify barriers to and motivators for vaccination. Methods: Cross-sectional, multicenter study on girls aged 9 to 19 years and their accompanying adults. The measurement criteria were the proportion of girls who were vaccinated against HPV, compliance with the vaccination schedule, factors associated with vaccination, and reasons for vaccination and non-vaccination through a self-administered questionnaire. Results: Seventy-one patients (16 with SLE and 55 with JIA) were included with a mean age of 13 years old (rank 11−18). According to parental questioning, 39% of patients were vaccinated against HPV or in progress (44% and 38% of SLE and JIA, respectively). This rate was 82% for the 22 patients ≥ 15 years of age. The vaccine was administered as often by a general practitioner (39%) as by a hospital pediatrician (also 39%). Two factors were significantly associated with vaccination: Older age (OR 53.68, 95% CI 5.85−429.29, p < 0.001) and previous hepatitis B vaccination (OR 4.97, 95% CI 1.03−24.01, p = 0.040). Recommendation of the vaccine by a health professional and fear of HPV-related diseases were the main facilitators. Lack of knowledge about the vaccine, lack of recommendation by a health professional, and fear of vaccine side effects were the main barriers. Conclusions: HPV vaccination coverage remains insufficient among patients with autoimmune disease. Education and awareness of health professionals about HPV infections are crucial elements in vaccine acceptance.

Neutropenia al diagnóstico de lupus eritematoso sistémico: prevalencia y correlaciones clínicas y serológicas. Nuestra cohorte / Neutropenia at diagnosis of systemic lupus erythematosus: prevalence and clinical and serological correlations. Our cohort

Introducción: las manifestaciones hematológicas en el lupus eritematoso sistémico (LES) son frecuentes. La leucopenia se presenta del 50 al 60% de los casos, pero solo el 17% tiene un recuento leucocitario <1.000/mm3. La neutropenia en pacientes con leucopenia ocurre entre un 20-40% (según el valor de corte del laboratorio). Los mecanismos posibles de neutropenia descriptos son: aumento en la destrucción de granulocitos periféricos por anticuerpos antineutrófilos, opsonización y destrucción por monocitos; cambios en el pool esplénico y marginal; y disminución en la producción medular. La formación de trampas extracelulares de neutrófilos (neutrophil extracellular traps, NETs) contribuye en la producción de interferón tipo 1 (IFN-1) a partir de plasmocitos y células dendríticas causando daño endotelial y cambios protrombóticos. La NETosis y el clearence anormal de material apoptótico promueven mayor liberación de antígenos y la consiguiente formación de autoanticuerpos. Las consecuencias infecciosas de la neutropenia al diagnóstico de LES se desconocen. Los objetivos del presente estudio fueron conocer la prevalencia de la neutropenia al diagnóstico de LES, determinar su correlación con otras variables de la patología, y estudiar su relación con una mayor probabilidad de actividad, daño, infecciones y mortalidad. Materiales y métodos: estudio descriptivo, retrospectivo. Se incluyeron pacientes con diagnóstico de LES (Systemic Lupus International Collaborating Clinics, SLICC 2012) de la cohorte del Sanatorio, desde enero de 2010 a diciembre de 2020. Se consignaron variables demográficas y asociadas a la enfermedad (criterios clínicos y de laboratorio). Escala de actividad: Systemic Lupus Erythematosus Disease Activity Index 2k (SLEDAI-2k). Se dividieron en dos grupos según la presencia de neutropenia (<1.500/mm3). Se definió un subgrupo de neutropenia severa: <500/mm3. En pacientes con neutropenia se evaluó la presencia de infección viral, bacteriana y tratamiento con factor de crecimiento de colonias de granulocitos y monocitos (GM-GSF). Análisis estadístico: los datos descriptivos se presentaron como medias y sus desvíos estándar (±DS) (variables continuas) y porcentajes (variables categóricas). Se compararon variables independientes de acuerdo con su distribución con test Mann Whitney. Se utilizó prueba t de Student para comparación de medias, y chi cuadrado (X2) para variables cualitativas. Se consideró como estadísticamente una p≤0,05. Resultados: se incluyeron 70 pacientes. Mujeres 59 (84%), edad media 38,6 años (18-72). Leucopenia 24 (34%), linfopenia 30 (42,8%), neutropenia 12 (17%), neutropenia severa 2 (2,8%) y plaquetopenia 7 (10%). Grupo con neutropenia (n=12): Sicca 12 (100%). Media índice neutrófilo/linfocito (INL) 1,33 (DS 0,69), infecciones: virus de Epstein-Barr (VEB) IgM (+) uno, parvovirus y CMV solicitados y negativos dos. PAMO realizada una: normal. Pacientes en tratamiento con GM-GSF: dos, sin eventos adversos. Dos infecciones urinarias. Conclusiones: en nuestro estudio se observó correlación entre neutropenia con síntomas Sicca, leucopenia y linfopenia, y un INL menor. Se desconoce si se relacionó a peor evolución. La presencia de infección fue baja (16%). Dos pacientes requirieron GM-GSF (con neutropenia severa), sin haber presentado eventos adversos.

Introduction: hematological manifestations are frequent in systemic erythematosus lupus (SLE). Leukopenia is seen in between 50 to 60% of cases, but only 17% has a leukocyte count <1,000/mm3. Neutropenia in patients with leukopenia occurs between 20-40% of cases, depending on the cut-off value used. Possible described mechanisms for neutropenia are: an increase in destruction of granulocytes by anti-neutrophil antibodies, opsonization and destruction by monocytes; change in the splenic and marginal neutrophil pool; a diminished production in the bone marrow. The formation of NETs contributes to the production of INF-1 from plasmocytes and dendritic cells, causing endothelial damage and pro-thrombotic changes. NETosis and apoptotic abnormal clearence promote the formation of antigens and subsequent autoantibodies. Infectious consequences of neutropenia in SLE are still unknown. The objectives of this article were to know the prevalence of neutropenia at diagnosis of SLE in our hospital, and secondly to determine its correlation with other variables of the disease and to investigate whether it's related with a greater probability of infections. Materials and methods: descriptive, retrospective study. Patients with diagnosis of SLE (SLICC 2012) from our cohort were included. Demographic and related to disease variables were stated. Activity scale: SLEDAI-2k. Patients were divided into two groups according to the presence or absence of neutropenia (<1.500/mm3 ) and multivariate analysis was performed to clinical and analytical variables. A subgroup with severe neutropenia (<500/mm3) was evaluated. Multivariate analysis was performed to detect correlations between a diminished neutrophil count and clinical manifestations, disease severity, autoantibodies profile, infections, and associated diseases. In neutropenic patients, the presence of viral or bacterial infection and the use of GM-GSF were evaluated. Statistical analysis was performed as mean +/-SD for continuous variables and percentage for categorical variables. T-Test or Mann-Whitney were used to compare independent variables according to distribution. Student's T and Chi-Square for qualitative variables. Statistical significance: p<0.05. Results: 70 patients were included. Female 59 (84%), mean age 38.6 years (18- 72). Leukopenia 24 (34%), lymphopenia 30 (42.8%), neutropenia 12 (17%), severe 2 (2.8%), thrombocytopenia 7 (10%). Neutropenic group: Sicca 12 (100%), neutrophil/lymphocyte index (NLI) 1.33 (DS 0.69), infections: EBV IgM+1/12, parvovirus and CMV negative 2/12. BMA 1/12, without pathologic findings. GM-GSF 2/12. Infections: 2/12 (urinary). Conclusions: we observed a correlation between Sicca symptoms, leuko and lymphopenia, and a lower NLI. The clinical significance of these findings was uncertain. The presence of infection was low (16%). Two required GM-GSF, having not presented adverse events.

The Role of Extracellular Vesicles in Systemic Lupus Erythematosus.

Extracellular Vesicles (EVs) are small vesicles that can be actively secreted by most cell types into the extracellular environment. Evidence indicates that EVs can carry microRNAs (miRNAs), long non-coding RNAs (lncRNAs), tRNA-derived small RNAs (tsRNAs), proteins, and lipids to target cells or tissue organizations. Latest studies show that EVs play a vital role in the immune modulation and may contribute to the pathogenesis of autoimmune diseases. Systemic lupus erythematosus (SLE) is a common autoimmune disease characterized by abnormal T cell activation and sustained production of autoantibodies against self-antigens, resulting in inflammation and damage to multiple systems. Pathogenic mechanisms of SLE, however, are still not well understood. In this review, we summarize the latest research advances on the functions and mechanisms of EVs, and its role in the pathogenesis, diagnosis, and treatment of SLE.

Roles of IL-25 in Type 2 Inflammation and Autoimmune Pathogenesis.

Interleukin-17E (IL-25) is a member of the IL-17 cytokine family that includes IL-17A to IL-17F. IL-17 family cytokines play a key role in host defense responses and inflammatory diseases. Compared with other IL-17 cytokine family members, IL-25 has relatively low sequence similarity to IL-17A and exhibits a distinct function from other IL-17 cytokines. IL-25 binds to its receptor composed of IL-17 receptor A (IL-17RA) and IL-17 receptor B (IL-17RB) for signal transduction. IL-25 has been implicated as a type 2 cytokine and can induce the production of IL-4, IL-5 and IL-13, which in turn inhibits the differentiation of T helper (Th) 17. In addition to its anti-inflammatory properties, IL-25 also exhibits a pro-inflammatory effect in the pathogenesis of Th17-dominated diseases. Here, we review recent advances in the roles of IL-25 in the pathogenesis of inflammation and autoimmune diseases.

Post-translational modifications in T cells in systemic erythematosus lupus.

Systemic erythematosus lupus (SLE) is a classic autoimmune disease characterized by multiple autoantibodies and immune-mediated tissue damage. The aetiology of this disease is still unclear. A new drug, belimumab, which acts against the B-lymphocyte stimulator (BLyS), can effectively improve the condition of SLE patients, but it cannot resolve all SLE symptoms. The discovery of novel, precise therapeutic targets is urgently needed. It is well known that abnormal T-cell function is one of the most crucial factors contributing to the pathogenesis of SLE. Protein post-translational modifications (PTMs), including phosphorylation, glycosylation, acetylation, methylation, ubiquitination and SUMOylation have been emphasized for their roles in activating protein activity, maintaining structural stability, regulating protein-protein interactions and mediating signalling pathways, in addition to other biological functions. Summarizing the latest data in this area, this review focuses on the potential roles of diverse PTMs in regulating T-cell function and signalling pathways in SLE pathogenesis, with the goal of identifying new targets for SLE therapy.

COVID GEAS: COVID-19 National Survey in Patients With Systemic Autoimmune Diseases.

OBJECTIVES: COVID-19 outcomes in population with systemic autoimmune diseases (SAD) remain poorly understood. The aim was to examine demographic and clinical factors associated with COVID-19 infection in people with rheumatic disease. METHODS: Two phases cross-sectional survey of individuals with rheumatic disease in April 2020 and October 2020. COVID infection, severity of disease, age, sex, smoking status, underlying rheumatic disease diagnosis, comorbidities and rheumatic disease medications taken immediately prior to infection were analyzed. RESULTS: A total of 1,529 individuals with autoimmunity disease diagnosis were included. Out of 50 positive patients, 21 required telephone medical assistance, 16 received assessment by primary care physician, 9 were evaluated in Emergency Department and 4 patient required hospitalization. Multivariate analysis was performed without obtaining differences in any of the systemic autoimmune diseases. Regarding the treatments, significant differences were found (p 0.011) in the treatment with anti-TNF-alpha agents with OR 3.422 (1.322-8.858) and a trend to significance (p 0.094) was observed in patients receiving mycophenolate treatment [OR 2.016 (0.996-4-081)]. CONCLUSIONS: Anti-TNF-alpha treatment was associated with more than 3-fold risk of suffering from SARS-CoV-2 infection, although in all cases infection was mild. Cumulative incidence in patients with SAD was up to 5 times higher than general population but with great differences between autoimmune diseases.

Biological and Clinical Changes in a Pediatric Series Treated with Off-Label JAK Inhibitors.

Off-label use of medications is still a common practice in pediatric rheumatology. JAK inhibitors are authorized in adults in the treatment of rheumatoid arthritis, psoriatic arthritis and ulcerative colitis. Although their use is not authorized yet in children, JAK inhibitors, based on their mechanism of action and on clinical experiences in small series, have been suggested to be useful in the treatment of pediatric interferon-mediated inflammation. Accordingly, an increased interferon score may help to identify those patients who might benefit of JAK inhibitors. We describe the clinical experience with JAK inhibitors in seven children affected with severe inflammatory conditions and we discuss the correlation between clinical features and transcriptomic data. Clinical improvements were recorded in all cases. A reduction of interferon signaling was recorded in three out of seven subjects at last follow-up, irrespectively from clinical improvements. Other signal pathways with significant differences between patients and controls included upregulation of DNA repair pathway and downregulation of extracellular collagen homeostasis. Two patients developed drug-related adverse events, which were considered serious in one case. In conclusion, JAK inhibitors may offer a valuable option for children with severe interferon-mediated inflammatory disorders reducing the interferon score as well as influencing other signal pathways that deserve future studies.

Development of Systemic Lupus Erythematosus After Infectious Mononucleosis in a 64-Year-Old Woman.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by heterogeneous symptoms that can manifest in any organ, and often presents at a young age. Infectious mononucleosis (IM) is the acute clinical manifestation of Epstein-Barr virus (EBV). It is characterized by low-grade fever, malaise, lymphadenopathy, splenomegaly, and occasionally symmetrical arthralgias. It has been proposed that EBV is a trigger for new-onset SLE, and patients with autoimmune disorders such as SLE are more likely to have recurrent IM infections. The patient, a 64-year old Caucasian female who's only past medical history was hypertension, developed several months-long period of vague symptoms, including fatigue, malaise, nausea, and nonbilious vomiting with oral intake. She presented with symmetrical polyarthritis involving the hands and elbows, with no history of arthritis before this episode. At the 5-month follow-up, she presented with worsening arthritis bilaterally in her elbows and in her right knee. For several decades, there has been a theoretical association between EBV and SLE, with EBV thought to be one of the many possible triggers for development of SLE. Based on the disease course, we theorize that the patient's IM and EBV infection led to development of SLE. A small fraction of SLE cases have been reported in literature to be associated with EBV. This case adds to that literature with EBV triggering development of SLE in a seemingly previously asymptomatic patient.

Serum IgG2 levels predict long-term protection following pneumococcal vaccination in systemic lupus erythematosus (SLE).

Systemic lupus erythematosus (SLE) patients are at risk for pneumococcal infection. Twenty-one consecutive SLE patients (40[25-75] years) received the sequential PCV13/PPSV23 vaccine and factors associated with long-term protection were analyzed. Immune protection, defined by an antigen-specific IgG concentration ≥1.3 µg/mL for at least 70% of 7 pneumococcal serotypes was assessed at baseline, 2, 6, 12 and 36 months defining long-term protection. Only 10 patients showed pneumococcal immune protection 36 months after vaccination. Eleven (52.4%) patients had no long-term protection with a seroconversion that never or only transiently occurred. SLE disease features, treatment received and immunological characteristics did not differ between protected and unprotected patients except for the pre-vaccination IgG2 serum levels. Serum IgG2 level >2.125 µg/ml showed a sensitivity of 100% and a specificity of 90.9% for long-term protection. Sequential pneumococcal vaccination conferred poor immune protection in SLE. Baseline IgG2 serum level identified patients able to benefit from pneumococcal vaccination.

Prevalence of subclinical synovitis of the hand in patients with systemic lupus erythematosus.

OBJECTIVES: To determine the prevalence of subclinical synovitis in Lupus patients without peripheral joint symptoms, in those with arthralgias without arthritis and those with episodic arthritis but without radiological structural damage. METHODS: We conducted a multicentre cross-sectional study. Patients with lupus from those three categories were recruited to take part in a greyscale ultrasound scan performed by an expert blinded rheumatologist. Data from a historical control group from a previous study was also included for comparisons. Images were assessed separately in order to determine the presence and level of synovitis following Eular recommendations. RESULTS: Ninety-six patients (88.5% female) with an average age of 40 ± 6.2 years old, were included. SLICC/ACR score was 0.6 ± 0.3 in the group without joint symptoms (group 0), 0.8 ± 0.3 in the group with arthralgias (group I) and 1.1 ± 0.4 in the group with episodic arthritis. The global prevalence of subclinical synovitis was 38.5%. In group 0, that prevalence was 30%. The time since onset of symptoms of patients with subclinical synovitis was longer than the rest of the patients (9.4 ± 2.2 vs 6.5 ± 4.0 years, P < 0.001). No other remarkable association was founded with clinical features of the disease. CONCLUSIONS: This is the first study focused on subclinical synovitis in patients with lupus. Other previous studies had included patients with different levels of arthropathy. Subclinical synovitis does exist in lupus patients in over a third of patients. Its meaning remains unclear and must be a topic of further studies.

Plasmaféresis en lupus eritematoso sistémico neuropsiquiátrico

De las complicaciones sistémicas más temidas del lupus eritematoso sistémico, destaca el compromiso neuropsiquiátrico y en especial la psicosis asociada. A pesar del gran espectro de presentaciones clínicas y ambigua definición, la sospecha clínica y el registro de casos ha ido en aumento en los últimos años. Poca evidencia científica existe sobre el tratamiento de la psicosis asociada al lupus eritematoso sistémico; sin embargo, se reconoce como un fenómeno autoinmune mediado por anticuerpos, sin que exista daño estructural cerebral. Los esteroides, ciclofosfamida y rituximab forman parte de las opciones terapéuticas. Se presenta el caso de una adolescente de 13 años, con diagnóstico reciente de lupus eritematoso sistémico y en quien una de las principales manifestaciones iniciales fue afección neuropsiquiátrica, en especial psicosis refractaria. Los tratamientos con glucocorticoides y ciclofosfamida fueron inefectivos y se logró remisión exitosa únicamente con el uso de plasmaféresis terapéutica. En total, se ofrecieron 4 sesiones de plasmaféresis con albúmina humana, logrando rápida remisión de su psicosis.

One of the most feared clinical presentations of systemic lupus erythematosus is neuropsychiatric involvement, specially associated psychosis. Diagnostic definitions are not well stablished to date however clinical suspicion and case registry has been increasing over time. There is scarce evidence regarding correct therapeutic approach to psychiatric presentations of systemic lupus erythematosus, however, it is well known that it is consequence of noxious autoimmune activity of the brain without structural compromise. Corticosteroids, cyclophosphamide and rituximab are part of the pharmacologic tools available. We present a case a 13 year old adolescent with new onset systemic lupus erythematosus in whom one of the principal initial concerns was how to address intense neuropsychiatric manifestations and specially refractory psychosis. First, high doses of steroid boluses were offered with no benefit in psychiatric symptoms, then cyclophosphamide was administered, with no success. Finally, therapeutic plasma exchange was offered to the patient with rapid clinical improvement. A total of 4 sessions of plasmapheresis with human albumin were done.

Infección por Listeria monocytogenes en una mujer gestante con lupus eritematoso sistémico y síndrome HELLP / Listeria monocytogenes infection in a pregnant woman with systemic lupus erythematosus and HELLP syndrome / Infeção por Listeria monocytogenes em uma mulher gestante com antecedente de lúpus eritromatoso sistemático e síndrome HELLP

La listeriosis es una enfermedad transmitida principalmente por alimentos contaminados con Listeria monocytogenes. Se presenta con mayor frecuencia en neonatos, mayores de 65 años, mujeres gestantes y pacientes inmunosuprimidos. La infección por L. monocytogenes durante la gestación se asocia a una importante morbimortalidad materno-fetal.Se reporta el caso de una mujer gestante de 29 años de edad con lupus eritematoso sistémico, a quien se le diagnosticó bacteriemia por L. monocytogenes. Durante la hospitalización, el cuadro clínico se complicó con hipertransaminasemia y, ante la presencia de trombocitopenia, se estableció el diagnóstico presuntivo de síndrome HELLP. El alto riesgo de morbimortalidad llevó a una finalización precoz de la gestación.La importancia de este trabajo clínico radica en presentar la dificultad en el diagnóstico y manejo en una paciente gestante de gran complejidad con una infección relativamente frecuente que puede pasar desapercibida.

Listeriosis is a disease mainly transmitted by food contaminated with bacteria called Listeria monocytogenes. It occurs more often in newborns, elder population, pregnant women and immunosuppressed patients. L. monocytogenes infection during pregnancy is associated to significant maternal mortality and morbidity. The case of a 29-year-old pregnant woman with history of Systemic Lupus Erythematosus is review. The said woman was diagnosed with bacteremia related to L. monocytogenes. During hospitalization, the patient experienced complications with hipertransaminasemia, which led to the presumptive diagnosis of HELLP in presence of thrombocytopenia. Given the high risk of mortality and morbidity, the pregnancy was terminated. The importance of the present clinical work lays in showing the difficulties embedded in diagnosing and handling a high-complexity pregnant patient presenting a frequent infection that would otherwise go undetected.

A listeriosis é uma doença transmitida principalmente por alimentos contaminados com Listeria monocytogenes. Apresentase com maior frequência em neonatos, maiores de 65 anos, mulheres gestantes e pacientes imunossuprimidos. Durante a gestação esta infeção associa-se a uma importante morbimortalidade materno-fetal. Foi reportado o caso de uma mulher gestante de 29 anos com antecedente de lúpus eritematoso sistémico, diagnosticada com bacteriemia por L. monocytogenes, na hospitalização teve complicações com hipertransaminasemia, foi estabelecido um diagnostico presuntivo de síndrome HELLP em presença de trombocitopenia. O alto risco de morbimortalidade levou a uma finalização precoce da gestação. A importância deste trabalho clínico radica em apresentar a dificuldade de diagnóstico e tratamento em pacientes gestantes de alta complexidade com infeção relativamente frequente, que pode passar desapercebida

[Variability of chloroquine and hydroxychloroquine retinopathy among various ethnicities]. / Différences ethniques parmi les patients souffrant de toxicité maculaire aux antipaludéens de synthèse.

INTRODUCTION: Current screening recommendations for chloroquine (CQ) and hydroxychloroquine (HCQ) retinopathy are based on central 10°C static perimetry and a high-resolution SD-OCT with a special attention to the inferior part of the macula where the toxicity usually starts by ellipsoid zone disruption. However, Melles and Marmor, have recently shown a great variability in the topography of the initial toxicity observed among various ethnicities, which is important to keep in mind so as not to miss early toxicity in certain subgroups of patients. METHODS: Review of the literature. RESULTS: Ethnic differences have been shown regarding the topography of the initial retinal toxicity of CQ and HCQ, particularly between Caucasian and Asian subjects. In Caucasians, the first signs of toxicity are more often localized in the inferior para-foveal area associated with a decrease in retinal sensitivity in the upper 10°C visual field. However, in Asian subjects, the first signs of toxicity appear more pericentral (still inferior) with an extramacular pattern that could be missed by the usual 10°C visual field screening. DISCUSSION/CONCLUSION: The pathophysiology of these ethnic differences is unknown and may be due to distinct genetic predisposition to CQ and HCQ toxicity. Screening strategies should be adjusted to the ethnicity and performed in Asian subjects with larger visual fields (30°C), along with SD-OCT, looking for ellipsoid disruption≥8°C from the fovea. The recognition of this pericentral topography and an adjusted screening protocol should avoid late diagnosis in Asians treated with CQ and HCQ.

Diagnóstico simultâneo de lúpus eritematoso sistêmico e chikungunya / Simultaneous diagnosis of systemic erythematosus lupus and chikungunya

Chikungunya é uma arbovirose caracterizada por febre alta, poliartralgia periférica, exantema, mialgia axial e intensa fadiga. Até 65% dos pacientes apresentam recuperação total dos sintomas em 4 semanas, contudo as dores articulares podem persistir por um período de 12 meses após a infecção em 4,1% dos pacientes e por até dois anos em 1,6% dos indivíduos acometidos. Um ano após a infecção 0,3% dos pacientes podem apresentar artrite inflamatória crônica. Os pacientes que permanecem com dores musculoesqueléticas e artrite pós-infecciosa são frequentemente tratados com drogas imunossupressoras. Por poderem apresentar quadro clínico semelhante e pelo fato de infecção ser comum nos pacientes portadores de doenças reumatológicas, o lúpus eritematoso sistêmico (LES) é uma afecção que deve ser abordada como diagnóstico diferencial em regiões endêmicas para essa arbovirose. Sabendo-se do crescente número de casos de chikungunya no Brasil e da relação fortemente estabelecida entre aumento de morbimortalidade em lúpicos nos quadros infecciosos, faz- se necessária uma ampla discussão dos casos emergentes. (AU)

Chikungunya is an arbovirose characterized by high fever, severe peripheral polyarthralgia, exanthema, axial myalgia and intense fatigue. Up to 65% of patients present complete recovery of symptoms at 4 weeks, however, joint pain may persist for a period of 12 months after infection in 4.1% of patients and for up to 2 years in 1.6% of affected individuals. One year after infection, 0.3% of patients may present with chronic inflammatory arthritis. Patients who remain with musculoskeletal pain and post-infectious arthritis are often treated with Immunosuppressive drugs. Due to the presence of similar clinics and the fact that double infection is common in patients with rheumatologic diseases in some populations, systemic lupus erythematosus (SLE) is a condition that must be addressed during the stipulation of differential diagnosis in regions endemic to arboviruses. Given the increasing number of cases of chikungunya in developing countries and the strongly established relationship between increased morbidity and mortality in lupus in infectious diseases, a broad discussion of emerging cases is required. (AU)

Therapy Side Effects in Systemic Lupus Erythematosus.

Glucosteroids (GS) are widely used drugs for various inflammatory pathologies (Nephrotic syndrome, Proliferative glomerulonephritis, Extramembrane glomerulonephritis, Nephropathy of the Nodous Poliarterita (PAN), Nephropathy from purple Henoch-Schonlein, lupus nephropathy (LN), Acute adrenal insufficiency Waterhouse-Friederichsen, Chronic adrenal insufficiency Addison, Systemic Lupus Erythematosus (SLE), Polymyositis and dermatomyositis, Chronic granulomatosis, Crohn's disease, Hemorrhagic rectocolitis, Hemolytic anemias, Acute leukemias and chronic lymphocytic leukemia, Hodgkin's lymphoma). Although they are prescribed for their anti-inflammatory and immunosuppressive properties, they also have many side effects, hyperglycemia being one of the most common and representative, which is why these drugs need careful monitoring when administered over the long term. This paper presents the case of a 39 year old patient diagnosed with systemic lupus erythematosus (SLE) with class IV lupus nephropathy (LN) who developed numerous complications due to the pathogenic side effects: diabetes, amenorrhea, recurrent infections, and depression.

Mortality from systemic erythematosus lupus in Brazil: evaluation of causes according to the government health database / Mortalidade por lúpus eritematoso sistêmico no Brasil: avaliação das causas de acordo com o banco de dados de saúde do governo

Abstract Objective: To characterize the causes of mortality in patients with systemic lupus erythematosus (SLE) in Brazil between 2002 and 2011. Methods: An exploratory ecological study of a time series using data from the Mortality Information System of DATASUS, the Department of the Unified Health System (Brazil's National Health System). Results: Brazil's SLE mortality rate was 4.76 deaths/105 inhabitants. The mortality rate was higher in the Midwest, North and Southeast regions than in the country as a whole. There were 6.3% fewer and 4.2% more deaths than expected in the Northeast and Southeast regions, respectively. The mean age at death was 40.7 ± 18 years, and 45.61% of deaths occurred between the ages of 20 and 39. Incidence was highest in women (90.7%) and whites (49.2%). Disorders of the musculoskeletal system and connective tissue were mentioned as an underlying cause of death in 77.5% of cases, and diseases of the circulatory system and infectious and parasitic diseases were also noted in fewer cases. SLE was mentioned as an underlying cause of death in 77% of cases, with no difference between the Brazilian regions (p = 0.2058). The main SLE-related causes of death were, sequentially, diseases of the respiratory and circulatory systems and infectious and parasitic diseases. Conclusions: This study identified a need for greater control of risk factors for cardiovascular diseases and a better understanding of the pathogenesis of atherosclerosis in SLE. Infectious causes are still frequent, and management should be improved, especially in the early stages of the disease.

Resumo Objetivo: Caracterizar as causas de mortalidade em pacientes com lúpus eritematoso sistêmico (LES) no Brasil entre 2002 e 2011. Métodos: Estudo ecológico exploratório de uma série cronológica com dados do Sistema de Informações sobre Mortalidade do Departamento de Informática do Sistema Único de Saúde (Datasus). Resultados: A taxa de mortalidade por LES no Brasil foi de 4,76 mortes/105 habitantes. A taxa de mortalidade foi maior nas regiões Centro-Oeste, Norte e Sudeste do que no país como um todo. Houve 6,3% menos e 4,2% mais mortes do que o esperado nas regiões Nordeste e Sudeste, respectivamente. A média de idade ao óbito foi de 40,7 ± 18 anos e 45,61% dos óbitos ocorreram entre 20 e 39 anos. A incidência foi maior nas mulheres (90,7%) e nos brancos (49,2%). Os distúrbios do sistema musculoesquelético e do tecido conjuntivo foram mencionados como a causa subjacente de morte em 77,5% dos casos; também foram observadas doenças do sistema circulatório e infecciosas e parasitárias, embora em menor frequência. O LES foi mencionado como a causa subjacente de óbito em 77% dos casos, sem diferença entre as regiões brasileiras (p = 0,2058). As principais causas de morte associadas ao LES foram, em ordem, doenças dos sistemas respiratório e circulatório e doenças infecciosas e parasitárias. Conclusões: Este estudo identificou a necessidade de maior controle dos fatores de risco para doenças cardiovasculares e uma melhor compreensão da patogênese da aterosclerose no LES. As causas infecciosas ainda são frequentes e o manejo deve ser melhorado, especialmente nos estágios iniciais da doença.