Cytomegalovirus infection and disease in systemic lupus erythematosus patients at a high-complexity hospital in southwestern Colombia.

Cytomegalovirus (CMV) infection and disease is a condition usually described in immunocompromised patients, but among them, those with connective tissue diseases are poorly represented. Here we present the clinical, laboratory characteristics, management and outcomes of systemic lupus erythematosus (SLE) patients who presented with a CMV infection/disease to a high complexity hospital in southwestern Colombia between 2011 and 2020. 16 SLE patients were found to have a CMV infection. SLE was predominantly characterized by renal involvement (10 patients; 62.50%), and 14 patients (87.5%) were receiving steroids previous to the CMV infection. The entire sample required hospital admission, mainly related to acute kidney injury, and nine patients were admitted to the intensive care unit (ICU). Gastrointestinal organ damage was the most common CMV disease manifestation. All patients received ganciclovir, five of them (31.25%) suffered from septic shock, and seven (43.75%) died. Age ≥38 years and the presence of septic shock at admission were correlated to the mortality outcome. To our knowledge, this is the first publication evaluating SLE patients with CMV infection/disease in a Colombian population.

Viperidae snake envenomation from a highly complex hospital in southwestern Colombia.

Background: Snake envenomation is a medical condition with high morbidity and mortality in southwestern Colombia. Objectives: To describe the characteristics of the envenomation caused by Viperidae snakes view in a highly complex hospital in Southwestern Colombia. Methods: A cross-sectional study was carried out. Patients treated for Viperidae snake envenomation from 2001 to 2020 in a Hospital Fundación Valle del Lili, Cali, Colombia, were studied. Results: Twenty-eight patients were included. Envenomation was caused by the genera Bothrops, Bothriechis, Porthidium, and Bothrocophias. The median age was 37.7 (±20.6), and they were predominantly male (19, 68%). Bites occurred on the upper extremities in 16 (57%) patients. Pain (23, 81%) and edema (22, 78%) were the most common clinical symptoms. Thirteen (46%) patients presented coagulopathy. Prolonged prothrombin and activated partial thromboplastin times were common: (22, 78% and 15, 53%, respectively). Twenty (71%) patients were treated with polyvalent antivenom (median dose of 6 (2-15) vials). The median time between the accident and antivenom administration was 9 h (5.5-17). Door-to-needle time was 37.5 (0-62) min. Eighteen (64%) patients were admitted to the intensive care unit. Three (11%) patients had serum sickness. Seven (25%) developed infectious complications, four (14%) had surgery, one (3%) had compartment syndrome, one (3%) underwent amputation of the affected limb, and one (3%) patient died. Conclusions: Local manifestations and coagulopathy were common clinical features. Polyvalent antivenom was an effective treatment for disease control. Significant complications were associated with delays in seeking medical care.

Idiopathic gigantomastia exacerbated during pregnancy. Its relationship with autoimmunity: A case report.

Gigantomastia is a rare entity characterized by diffuse and excessive breast enlargement. It mainly occurs during puberty and pregnancy as a consequence of hormonal fluctuations. We report an unusual case of gigantomastia in a 29-year-old woman with a history of personal and familiar autoimmune phenomena. She had autoimmune thyroiditis and several positive autoantibodies, and developed 3 crises of the disease, 1 related to pregnancy (possibly hormone-mediated), and 2 unrelated to pregnancy in which an autoimmune role is raised based in clinical, histological and laboratory findings. Immunological aspects that may be involved in this presentation of the disease are discussed.

Diagnostic Performance of Anti-dsDNA Tests by Indirect Immunofluorescence and Enzyme-linked Immunosorbent Assay in Patients with Systemic Lupus Erythematosus.

BACKGROUND: Several laboratory techniques for anti double-stranded (ds) DNA detection in systemic lupus erythematosus (SLE) are available, with variable diagnostic performance. We aimed to evaluate anti-dsDNA's diagnostic performance by indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (EIA). METHODS: We conducted a single-center retrospective (2015 to 2020) study. Patients with anti-dsDNA tests by IIF and EIA were included. We evaluated the indications, applications, concordance, positive predictive value (PPV) of anti-dsDNA to confirm SLE diagnosis or flares, and associations of disease manifestations with positivity with each technique. RESULTS: A total of 1368 reports of anti-dsDNA tests by IIF and EIA and the corresponding medical records of the patients were analyzed. The main indication for anti-dsDNA testing was to help in the diagnosis of SLE in 890 (65%) of the samples, and the main application after obtaining the results was SLE exclusion in 782 (57.2%) cases. The combination with the highest frequency was the negativity result by both techniques in 801 (58.5%) cases (Cohen kappa 0.57). Both methods were positive in 300 patients with SLE (Cohen kappa 0.42). The PPVs of anti-dsDNA tests to confirm diagnosis/flare was 79.64% (95% CI, 75.35-83.35) by EIA, 78.75% (95% CI, 74.27-82.62) by IIF, and 82% (95% CI, 77.26-85.93) when both were positive. CONCLUSIONS: Anti-dsDNA detection by IIF and EIA are complementary and may indicate different clinical patterns in patients with SLE. The detection of anti-dsDNA antibodies by both techniques has a higher PPV than either separately for confirming SLE diagnosis or flares. These results highlight the need for evaluating both methods in clinical practice.

Lymphoid follicular hyperplasia in patients with systemic lupus erythematosus after multiple cycles of rituximab.

Rituximab is indicated in some patients with refractory systemic lupus erythematosus (SLE). Occasionally, this medication is required in chronic form to maintain control of the disease. We described two patients who developed lymphoid follicular hyperplasia (LFH) after multiple cycles of rituximab and evaluated the expression of B cell activating factor belonging to the tumor necrosis factor (TNF) family (BAFF) and its receptors [BAFF-receptor (BAFF-R) and B cell maturation antigen (BCMA)], as possible factors related to lymphoid node enlargement. Two patients with SLE completed six and nine cycles of rituximab (1 g every 2 weeks) indicated each 9 months, achieving remission for 5 and 7 years, respectively, when developed prominent lymphadenopathies. Biopsies showed LFH. Haematological neoplasms were ruled out. Immunohistochemistry showed BAFF overexpression in the follicles, and moderate expression of BAFF-R confined to the mantle zone and BCMA to the germinal centre. Belimumab B cell activating factor belonging to the TNF family (anti-BAFF therapy) was started with positive effects on the clinical condition. LFH can develop in patients with SLE who received multiple cycles of rituximab. BAFF overexpression and moderate expression of BAFF-R and BCMA in lymph nodes were seen. These findings added to the improvement with the change to belimumab could suggest that LFH after cluster of differentiation (CD20) depletion therapy may be associated with a compensatory overexpression of BAFF and its receptors.

B-cell activating factor (BAFF) and its receptors' expression in pediatric nephrotic syndrome is associated with worse prognosis.

AIM: Immune pathogenesis of nephrotic syndrome (NS) is not completely understood. We aimed to evaluate the expression of B-cell activating factor (BAFF) and its receptors in renal samples from pediatric NS patients and its relationship with renal function survival. MATERIALS AND METHODS: We conducted an ambispective study on 33 patients with pediatric NS. Immunohistochemistry for BAFF, TACI, BCMA and BR3 was performed. Markers were evaluated on podocytes and interstitial inflammatory infiltrates (III). We performed Kaplan-Meier curves to describe renal function survival according to markers' expression. RESULTS: Thirty-three NS patients were included. Minimal change disease was seen in 21 (63.6%) patients, and focal segmental glomerulosclerosis in 12 (36.4%). BAFF was found in podocytes (18.2% of samples) and III (36.4% of samples), BAFF-R in one sample, TACI in 4 (podocytes and III), and BCMA in 5 samples of podocytes and 7 of III. BAFF on podocytes and III was associated with worst renal function at follow-up; those patients had 25% probability of having GFR >90 mL/min/1.73m2, versus 84.9% when absent (p = 0.0067). Patients with BAFF in III had 42.9% probability of having GFR>90 mL/min/1.73 m2, versus 94.1% when absent (p = 0.0063). CONCLUSION: BAFF expression in renal biopsies could be a prognostic factor for renal function.

Profile of specific and associated autoantibodies in patients with idiopathic inflammatory myopathies in a Colombian population.

Objectives: Idiopathic inflammatory myopathies (IIMs) are chronic, autoimmune diseases with several forms of presentation. Diagnosis is mostly clinical in our region. Our aim was to evaluate the autoantibody profile of patients with IIMs. Methods: This study is a cross-sectional study with a prospective recollection of data, conducted between 2019-2021, in a single center in Cali, Colombia. Patients with a clinical diagnosis or suspicion of IIM were included. The presence of myositis-specific/associated antibodies was evaluated by immunoblotting in serum samples. Phenotypic characterization was performed. Results: A total of 36 patients were included. The mean age was 50.6 (16.7) years, and 20 (55.6%) were female. Eighteen (50%) patients were seropositive, of which 11 (30.5%) presented one positive antibody, with anti-TIF1É£being the most frequent (n = 4, 11.1%), followed by anti-Ro52 (n = 2, 5.6%). Seven patients (19.4%) showed >1 positive antibody. Dermatomyositis was the most frequent type of IIM in seropositive patients (n = 8, 44.4%), followed by anti-synthetase syndrome (n = 4, 22.2%). Weakness was symmetric and presented in the upper and lower extremities in 11 (61.1%) patients each. Both respiratory insufficiency and weight loss were seen in 7 (38.9%) patients, Gottron papules in six (33.3%) patients, and heliotrope rash, esophageal dysmotility, and myalgia in 5 (27.8%) patients. Pulmonary interstitial disease was seen in 4 (22.2%, with antibodies for anti-Ro52, anti-MDA5 + anti-Jo1 + anti-TIF1É£, anti-MDA5 + anti-SAE1 + anti-NXP2, and anti-cN1A + anti-Ro52) patients, and malignancy was seen in 2 (11.1%) patients (1 with anti-Mi2ß and 1 with anti-TIF1É£ + anti-Mi2α). In all, 7 (19.4%) patients required intensive care (2 seropositive, 1 with anti-PL7, 1 with anti-MDA5 + anti-Jo1 + anti-TIF1É£), and 1 (2.8%) (seronegative) patient died. Conclusion: This study is the first study in the Southwest of Colombia that evaluates myositis-specific/associated antibodies in IIM. Half of the patients were seropositive. Anti-TIF1É£was the most frequent MSA and anti-Ro52 was the most frequent MAA. Several patients presented antibody combinations. Further studies are needed to fully associate phenotypes with antibodies.

Frequency of anti-neutrophil cytoplasmic antibodies in patients with systemic lupus erythematosus / Frecuencia de los anticuerpos anticitoplasma de neutrófilo en pacientes con lupus eritematoso sistémico

ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Patients with SLE exhibit multiple serum autoantibodies, including anti-neutrophil cytoplasmic antibodies (ANCAs). There are two main techniques to detect ANCAs: indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA). In this study, an attempt was made to determine the frequency and clinical associations of ANCAs in patients with SLE. Methods: A cross-sectional study was conducted in a tertiary care hospital in Colombia that included 74 patients with SLE. The presence of ANCAs was assessed using IIF with ethanol-fixed slides, and ELISA was used to detect antibody specificities for myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA. Results: Of the 74 patients with SLE evaluated, 60 (81.1%) of them were ANCA-positive by IIF. By contrast, only one patient showed specificity for PR3-ANCA by ELISA. The relevance of ANCA positivity by IIF and clinical and serological features was significant for renal involvement (p = .0174), and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) (p = .0308). Conclusion: ANCAs are common in the serum of patients with SLE, as detected by ethanol-fixed slides with IIF staining. However, detection of specificity to PR3 and/or MPO is rare, thus highlighting the importance of detecting these autoantibodies by different techniques.

RESUMEN Introducción: El lupus eritematoso sistémico (LES) es una enfermedad autoinmune sistémica. Los pacientes con LES muestran múltiples autoanticuerpos séricos, incluyendo los anticuerpos anticitoplasma de neutrófilo (ANCA, por sus siglas en inglés). Existen 2 técnicas principales para la detección de ANCA: inmunofluorescencia indirecta (IFI) y ensayo por inmunoadsorción ligado a enzimas (ELISA). En este estudio nuestro objetivo fue determinar la frecuencia y las asociaciones clínicas de los ANCA en pacientes con LES. Métodos: Realizamos un estudio transversal de 74 pacientes con LES en un hospital de alta complejidad de Colombia. La presencia de ANCA se evaluó por IFI, utilizando láminas con fijación de etanol, y con ELISA para determinar las especificidades para mieloperoxidasa (MPO)-ANCA y proteinasa 3 (PR3)-ANCA. Resultados: Fueron evaluados 74 pacientes con LES, 60 (81,1%) de ellos fueron positivos para ANCA. Por el contrario, solo un paciente mostró especificidad para PR3-ANCA por ELISA. La relación entre la positividad para ANCA por IFI y las características clínicas y serológicas fue estadísticamente significativa para compromiso renal (p = 0,0174) y para el índice de actividad de la enfermedad (Systemic Lupus Erythematosus Disease Activity Index [SLEDAI]) (p = 0,0308). Conclusiones: Los ANCA detectados mediante fijación con etanol por técnicas de IFI, son comunes en pacientes con LES. Sin embargo, la detección de especificidades para PR3 o MPO es rara; se destaca la importancia de la evaluación de estos autoanticuerpos mediante diferentes técnicas.

Clinical Characteristics and Outcomes of Patients With ANCA-Associated Vasculitides in a Colombian Hospital.

BACKGROUND/OBJECTIVE: Antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) are uncommon systemic autoimmune diseases, of which few reports exist in Latin America. Our aim was to examine AAV evaluated in a high-complexity hospital in southwestern Colombia, with emphasis in severe forms. METHODS: A medical records review study of 67 patients was performed, and data were collected from electronic registries. Moderate and severe AAVs were defined as the presence of life-threatening complications, unfavorable Birmingham Vasculitis Activity Score outcomes, and hospitalization requirements at the time of diagnosis and by the last follow-up, between 2011 and 2019. Clinical manifestations, treatment, and outcomes were evaluated. The AAV subtypes were compared. RESULTS: A total of 67 cases were included. The majority were female (n = 44, 65.67%), and the median age was 52 (40-64) years. Granulomatosis with polyangiitis (GPA) was the most frequent with 42 patients (62.68%), followed by microscopic polyangiitis (MPA) and eosinophilic GPA, with 15 patients (22.38%) and 10 patients (14.92%), respectively. Forty-four patients (65.67%) presented pulmonary symptoms. The highest Birmingham Vasculitis Activity Score corresponded to MPA, with 21 (12-25) points. Fifteen patients (22.4%) were admitted to the intensive care unit throughout the course of the disease, of whom 10 had GPA. The longest stay and duration of mechanical ventilation were seen in MPA. The principal treatments were corticosteroids and cyclophosphamide, and the main outcome was end-stage renal disease. CONCLUSIONS: In this cohort of AAV, most of cases corresponded to GPA, and pulmonary manifestations were the most common. Microscopic polyangiitis was the more severe subtype as it showed worse impairment in clinical characteristics and intensive care unit requirements.

Association of Antiribosomal P Antibody with Neurological and Systemic Manifestations in Patients with Systemic Lupus Erythematosus in Southwestern Colombia.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous pathogenesis, various clinical manifestations, and a broad spectrum of autoantibodies which recognize different cellular components. This study examines the clinical significance and serological associations of serum antiribosomal P antibodies (anti-P) derived from SLE patients in a population from southwestern Colombia. METHODS: We performed a cross-sectional study of 66 SLE patients. Serum antiribosomal P0 autoantibodies were detected by line immunoassay using the ANA-LIA MAXX kit and processed on the automated HumaBlot 44FA system (Human Diagnostics, Germany). RESULTS: Of the 66 SLE patients included in the study, 17 patients (25.76%) showed anti-P positivity by line immunoassay (IA), 47 (71.21%) were negative, and results from 2 patients were indeterminate. We did not find an association with neuropsychiatric SLE (NPSLE), renal, or hepatic disorders (P > 0.05). Laboratory findings indicated that anti-P positivity was significantly associated to anti-Smith (P = 0.001), anti-Ro60/SSA (P = 0.046), and anti-dsDNA antibodies (P = 0.034), the latter being true only when performed using indirect immunofluorescence (IIF). CONCLUSION: The anti-P antibodies are not associated with clinical manifestations such as NPSLE, lupus nephritis, or hepatic involvement in the southwest Colombian SLE population. Moreover, we confirmed previously reported association between anti-P antibody, serum anti-dsDNA, and anti-Smith.

Description of the Etiologies, Clinical Characteristics, and Outcomes in Patients with Hyperferritinemia in a Colombian Tertiary Hospital.

BACKGROUND: This study analyzes the clinical characteristics, outcomes, and conditions associated with hyperferritinemia (≥5000 ng/mL) in a high-complexity center in Colombia. METHODS: This retrospective and descriptive study was performed between 2011 and 2020, at the Fundación Valle del Lili, Cali, Colombia, by reviewing medical charts from patients who had serum ferritin measurements equal to or greater than 5000 ng/mL. RESULTS: We found 350 reports of ferritin values ≥5000 ng/mL, corresponding to 317 patients, with a median ferritin value of 8789 (6001-15 373) ng/mL. The most frequent etiologies were infection (n = 198, 56.57%), hematologic disorders (n = 104, 29.71%), and blood transfusion (n = 98, 28.00%). These last 2 etiologies cooccurred in 37 (10.57%) cases. The main clinical signs accompanying hyperferritinemia were fever in 199 (56.86%) cases, multiorgan involvement in 125 (35.71%), and hepatomegaly in 95 (27.14%) cases. Ninety-four (29.65%) patients died in the hospital, and 11 (3.47%) died within 30 days after medical discharge, mainly due to infection (n = 51, 48.57%). Intrahospital mortality was associated with significantly higher ferritin levels (10 846, IQR: 6425-23 459) than survival (8452, IQR: 5980-13 932) (P = 0.018). CONCLUSIONS: Hyperferritinemia is related to many underlying causes, with infection being the principal cause in our cohort, followed by hematologic disorders. Additionally, in-hospital mortality was related to higher ferritin levels.

Lymphomagenesis predictors and related pathogenesis.

Sjögren's syndrome (SS) is a systemic autoimmune disease characterised by a wide range of clinical manifestations and complications, including B-cell lymphoma. This study aims to describe the predictors associated with lymphomagenesis in patients with Sjögren's syndrome, emphasising the pathophysiological bases that support this association. We performed a review of the literature published through a comprehensive search strategy in PubMed/MEDLINE, Scopus, and Web of science. Forty publications describing a total of 45,208 patients with SS were retrieved. The predictors were grouped according to their pathophysiological role in the lymphoproliferation process. Also, some new biomarkers such as MicroRNAs, P2X7 receptor-NLRP3 inflammasome, Thymic stromal lymphopoietin, and Three-prime repair exonuclease 1 (TREX1) were identified. The knowledge of the pathophysiology allows the discrimination of markers that participate in the initial stages. Considering that the lymphoproliferation process includes the progression of lymphoma towards more aggressive subtypes, it is essential to recognise biomarkers associated with a worse prognosis.

Utility of anti-C1q and anti-nucleosome antibodies in systemic lupus erythematosus and lupus nephritis in southwestern Colombia / Utilidad de los anticuerpos anti-C1q y anti-nucleosomas en el lupus eritematoso sistémico y la nefritis lúpica en el suroccidente colombiano

ABSTRACT Introduction: Lupus nephritis (LN) is one of the most prevalent and severe complications of systemic lupus erythematosus (SLE), requiring reliable urine and serum biomarkers to evaluate it. Anti-nucleosome and anti-C1q antibodies are associated with LN in several geographic regions. Also, southwest Colombia has a heterogeneous ethnicity, which motivated the evaluation of the frequency and relationship of such markers with LN in this region. Methods: A cross-sectional study was conducted in a health centre in south-west Colombia in 84 patients diagnosed with SLE (57 without LN; 27 with LN) between 2016 and 2018. Demographic and clinical and laboratory features, including anti-dsDNA, complement, and anti-C1q and anti-nucleosome antibodies were compared in these patients. ELISA immunoassays were performed to measure the antibodies of interest in blood samples. Statistical analysis was carried out using STATA14 software (StataCorp, College Station, Texas, USA). Quantitative variables were summarised as means or medians and compared with Mann-Whitney or Two-sample t test. Categorical variables were shown as proportions, and compared with Chi-squared or Fisher's exact test. Correlation analysis between quantitative variables was calculated using Spearman's correlation. Results: Of all 84 patients, 27 patients had LN, of which 16 (59.2%) had a positive test for anti-nucleosome antibodies and 10 (37%) for anti-C1q antibodies. An association was found between anti-C1q and proliferative forms of LN and newly diagnosed LN. A correlation was found between anti-nucleosome and anti-C1q antibodies, and anti-dsDNA and low serum complement concentrations. Conclusion: Although both markers were found in variable percentages in SLE patients and seem not to be specific markers of LN in our population, anti-C1q was associated with proliferative forms of LN and de novo LN.

RESUMEN Introducción: La nefritis lúpica (NL), una de las complicaciones más frecuentes y graves del lupus eritematoso sistémico (LES), requiere biomarcadores confiables de orina y suero para su evaluación. Los anticuerpos anti-nucleosoma y anti-C1q se asocian con la NL en varias regiones geográficas. En el suroccidente colombiano se asienta una etnia heterogénea, lo que motivó la evaluación de la frecuencia y la relación de dichos marcadores con NL en dicha región. Métodos: Realizamos un estudio transversal en un centro de salud en el suroccidente de Colombia, con 84 pacientes diagnosticados con LES (57 sin NL; 27 con NL) entre los anos 2016 y 2018. Se compararon las características demográficas, clínicas y de laboratorio, incluidos los anticuerpos anti-dsDNA, complemento, anti-C1q y anti-nucleosomas entre estos pacientes. Se realizaron inmunoensayos ELISA para medir los anticuerpos de interés en muestras de sangre. El análisis estadístico se llevó a cabo con el software Stata v.14 (Stata-Corp, College Station, Texas, EE. UU.). Las variables cuantitativas se resumieron como medias o medianas y se compararon con la prueba t de Mann-Whitney o Two-sample t test; las variables categóricas se mostraron como proporciones y se compararon con Chi-cuadrado o con la prueba exacta de Fisher. Para el análisis de correlaciones entre variables cuantitativas se calculó el coeficiente de correlación de Spearman. Resultados: Entre los 84 pacientes, 27 presentaban LN, de los cuales 16 (59,2%) tuvieron una prueba positiva para anticuerpos anti-nucleosoma y 10 (37%) para anticuerpos anti-C1q. Se encontró una asociación entre anti-C1q y formas proliferativas de NL, así como formas recientemente diagnosticadas de NL. Hubo una correlación entre los anticuerpos anti-nucleosoma y anti-C1q y el anti-dsDNA y las bajas concentraciones de complemento sérico. Conclusión: Aunque los 2 marcadores se encontraron en porcentajes variables de pacientes con LES y no parecen ser marcadores específicos de NL en nuestra población, la presencia de anti-C1q se asoció con formas proliferativas de NL y NL de novo.

Reproducibility of minor salivary gland biopsy reports in Sjögren's syndrome and its correlation with disease biomarkers.

INTRODUCTION/OBJECTIVE: Sjögren's syndrome (SS) is a systemic autoimmune disease that is challenging to diagnose. Although minor salivary gland biopsy (MSGB) is a useful ancillary study, different factors make its interpretation difficult. Also, the significance of distinct histopathological findings is unknown. We aimed to determine the concordance between pathologists and rheumatologists in interpreting the MSGB results, as well as the correlation between MSGB findings, paraclinical features, and SS diagnosis. METHODS: This descriptive retrospective study reviewed medical charts from 998 individuals from a single center where MSGBs had been performed. Rheumatologists interpreted biopsy reports from pathologists, and interobserver variability was calculated. Logistic regression using immunological parameters and histological findings was performed. RESULTS: We included 998 patients with a median age of 55 years (45-64 years); the majority of patients were females (n = 934, 93.6%). Chisholm and Mason's scoring system was the most frequently used scale (55.1%). There was a good correlation between pathologists and rheumatologists for diagnosing SS using MSGB findings (Cohen's kappa 0.91). We observed a strong association between interstitial plasmocytes and SS (OR 24, 95% CI 9.09-64.94, p = 0). CONCLUSION: The MSGB is an essential tool for the diagnosis of SS. Although different factors may negatively affect its reproducibility, histological findings, such as interstitial plasmocytes, may predict the risk of developing SS. Key Points • We provide information based on 998 patients with suspected SS diagnosis. • Chisholm and Mason's scale is the most frequently used compared to Greenspan's and Tarpley's scales. • There is good correlation between pathologists and rheumatologists for the diagnosis of SS using MSGB.

Clinical characterization, outcomes, and prognosis in patients with systemic lupus erythematosus admitted to the intensive care unit.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a clinically heterogeneous autoimmune disease, and in some conditions, admission to the intensive care unit (ICUs) is required. This study describes the clinical and prognostic factors in SLE patients admitted to the ICU. METHODS: We conducted a retrospective study that reviewed all clinical records of patients with SLE admitted to the ICU between 2011 and 2018. RESULTS: We evaluated 188 patients, with 279 ICU admissions. Most patients were female (n = 159; 84.57%) with a median age of 35 years (interquartile range (IQR) = 25-48 years). Infection was the leading cause of admission in 77 (27.60%) cases, followed by lupus flare. The average length of hospitalization was 5 days (IQR 3-11 days), and the SLE Disease Activity Index 2000, Acute Physiology, Age and Chronic Health Evaluation (APACHE II), and Sequential Organ Failure Assessment (SOFA) scores were 9 (IQR 2-17), 14 (IQR 10-17), and 3 (IQR 2-5), respectively. Non-survivors presented with higher APACHE II and SOFA scores. Infection was the leading cause of mortality (n = 38; 20.21%), and predictors of mortality included invasive mechanical ventilation, vasoactive medication requirement, higher SOFA scores, and antiphospholipid syndrome comorbidity. CONCLUSIONS: We found that infection was the leading cause of ICU admissions and mortality in patients with SLE. Factors identified here as predictors of mortality should be accurately identified at admission for the prompt treatment of SLE patients.

Systemic lupus erythematosus in the intensive care unit: a systematic review.

Systemic lupus erythematosus (SLE) is an autoimmune disease with heterogeneous pathophysiologic mechanisms and diverse clinical manifestations. SLE is a frequent cause of intensive care unit (ICU) admissions. Multiple studies with controversial findings on the causes, evolution and outcomes of ICU-admitted patients with SLE have been published. The aim of this paper is to review the literature reporting the clinical characteristics and outcomes, such as mortality and associated factors, in such patients. Among the main causes of ICU admissions are SLE disease activity, respiratory failure, multi-organ failure and infections. The main factors associated with mortality are a high Acute Physiology and Chronic Health Evaluation (APACHE) score, the need for mechanical ventilation, and vasoactive and inotropic agent use. Reported mortality rates are 18.4%-78.5%. Therefore, it is important to evaluate SLE disease severity for optimizing clinical management and patient outcomes.

Sjögren Syndrome in the Intensive Care Unit: An Observational Study.

BACKGROUND/OBJECTIVE: Studies on the clinical characteristics, prognosis, and factors associated with mortality in patients with Sjögren syndrome (SS), particularly those in the intensive care unit (ICU), are limited. The present study aimed to describe clinical and immunological variables associated with mortality in patients with SS admitted to ICU at a single center in Cali, Colombia. METHODS: An observational, medical records review study was performed between 2011 and 2019 by reviewing the clinical records of patients with SS admitted to ICU at a high-complexity center. RESULTS: Seventy-two patients were included with a total of 117 ICU admissions (17 cases required readmission and 1 case required 17 readmissions): 103 (86.32%) were attributable to medical issues, and 14 corresponded to surgical admissions. Major causes of ICU medical admission were infection (44/103) followed by organ involvement. Only 5 admissions were related to SS due to neurological involvement. The APACHE (Acute Physiology, Age, and Chronic Health Evaluation) score was 10 (interquartile range [IQR], 7-16), the SOFA (Sequential Organ Failure Assessment) score was 2 (IQR, 0-14), and the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) score was 0 (IQR, 0-12) with higher values in the nonsurvivor group. Intensive care unit mortality was 12/72 (16.67%). CONCLUSIONS: The main cause of ICU admission was infection. Patients with increased medical requirements, such as mechanical ventilation and vasopressor support, and with higher APACHE, SOFA, and ESSDAI scores were more susceptible to poor outcomes. Moreover, 50% of deaths were attributable to SS and 25% to infection.

Description of criteria for near miss in high-complexity obstetric population with sickle cell anemia: an observational study.

Introduction: Sickle cell disease (SCD) is one of the most common monogenic diseases worldwide, affecting approximately 30 million people across the globe. It is one of the most common hemoglobinopathies affecting pregnancy, particularly in the black population. Pregnant women with SCD, in conjunction with the physiological changes of pregnancy, have an increased risk of developing severe maternal and fetal complications.Materials and methods: A descriptive, observational study was conducted retrospectively to determine the maternal and neonatal disease burden of pregnant women with SCD between 2011 and 2016. The study included pregnant patients with SCD admitted to the hospital, with confirmed diagnosis through hemoglobin electrophoresis. Patients having no confirmatory testing of the disease were excluded.Results: In all, 54% (34 patients) had no previous diagnosis of sickle cell anemia or presence of sickle cell trait, and so an in-hospital diagnosis was carried out through hemoglobin gel electrophoresis, whereas the remaining 46% (28 patients) had already a peripheral diagnosis. In total, the following profiles were obtained: Hb AS 62.9% (39 patients), Hb SC 17.7% (11 patients), Hb SS 16.1% (10 patients), Hb AC 3.2% (2 patients), and Hb CC 0% (0 patients). Vaso-occlusive pain crises constituted the most common cause of hospital admission antepartum (54.8% of the general population), occurring in 100% of the patients with Hb SS and Hb AC, in 45% of the Hb SC group and in 43% of those with the Hb AS trait. The second cause of hospital admission encompassed infectious processes, which affected 45% of the general population, 30% of the Hb AS group, 60% of the Hb SS group, 77% of the Hb SC group, and 100% of the Hb AC group. The method of termination of pregnancy of 44% (27 patients) was C-section. The most common perinatal complication was preterm birth, occurring in 26% (16 patients), with the highest incidence in the Hb AC group. The second most common complication was the intrauterine growth restriction, reported in 6% (4 patients) of all patients, being most frequently in the Hb SS group.Conclusion: Pregnant women having SCD should be assessed and managed by a multidisciplinary team composed of hematology, perinatology, and intensive care. Management should be conducted preferably in high-quality centers experienced in treating this entity, thereby decreasing the great impact of morbidity and mortality associated with SCD in the pregnant population.