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.

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.

Epidemiological characterization of patients with sarcoidosis in a high complexity hospital in southwestern Colombia

Introduction: Epidemiological studies on sarcoidosis in Colombia are scarce, and although recent reports from the north of the country have been published, clinical-epidemiological associations are not clear. Our aim was to characterize patients with sarcoidosis diagnosed at Fundación Valle del Lili in Cali, Colombia. Methods: A retrospective study of a series of sarcoidosis cases was conducted between 2011 and 2019. Demographic, clinical, laboratory, imaging, histopathological, and treatment variables were analyzed. Results: A total of 34 patients with a diagnosis of sarcoidosis were found. The majority were women (n = 25; 73%), and the mean age was 50 years. The main symptoms of onset were erythema nodosum (n = 11; 33%), arthritis (n = 10; 30%), and cough (n = 9; 27%). In 64% of the cases, there was pulmonary involvement, with pulmonary nodules, mediastinal adenopathy, and interstitial lung disease found in 54%, 50%, and 36% of cases, respectively. In 85% of cases, there were extrapulmonary manifestations, mainly cutaneous (50%). Angiotensin-converting enzyme (ACE) was elevated in 62% of the cases in which it was measured (n = 16; 47%). During the diagnostic process, 23 biopsies were performed, of which 95% showed granulomas with noncaseating necrosis. Most of the patients (76%) were controlled with prednisolone, at an average dose of 20 mg (7.5-50 mg) per day. Conclusions: Sarcoidosis was more frequent in women and mestizos, and it presented earlier in men. Elevated ACE was not associated with extrapulmonary involvement. Calcium-phosphorus profile and antinuclear antibodies were not useful to establish the diagnosis.

Introducción: En Colombia son escasos los estudios epidemiológicos sobre la sarcoidosis; aunque recientemente se han publicado reportes del norte del país, son grupos muestrales pequeños, por lo que no quedan claras las asociaciones clínico-epidemiológicas. Nuestro objetivo fue caracterizar pacientes con sarcoidosis en la Fundación Valle del Lili, en Cali, Colombia. Métodos: Se realizó un estudio retrospectivo de una serie de casos de sarcoidosis entre el 2011 y el 2019. Se analizaron variables demográficas, clínicas, de laboratorio, imagenológicas, histopatológicas y de tratamiento. Resultados: Se encontraron 34 pacientes con diagnóstico de sarcoidosis, la mayoría fueron mujeres (n = 25; 73%), y la edad promedio fue 50 anos. Los principales síntomas de inicio fueron eritema nudoso (n = 11; 33%), artritis (n = 10; 30%) y tos (n = 9; 27%). En el 64% de los casos hubo compromiso pulmonar, y se encontraron nódulos pulmonares, adenopatías mediastinales y enfermedad pulmonar intersticial en un 54, 50 y 36% de los casos, respectivamente. En el 85% de los casos hubo manifestaciones extrapulmonares, principalmente cutáneas (50%). Los niveles de enzima conversora de angiotensina estuvieron elevados en el 62% de los casos en los que fue medida (n = 16; 47%). Durante el proceso diagnóstico se realizaron 23 biopsias, de las cuales el 95% evidenció granulomas con necrosis no caseificante. La mayoría de los pacientes (76%) fueron controlados con prednisolona, a una dosis promedio de 20 mg (7,5-50 mg) por día. Conclusiones: La sarcoidosis fue más frecuente en mujeres y mestizos. La presentación fue más temprana en hombres. La enzima conversora de angiotensina no se relacionó con compromiso extrapulmonar. Ni el perfil fósforo-calcio ni los anticuerpos antinucleares fueron útiles para establecer el diagnóstico.

From ocular immune privilege to primary autoimmune diseases of the eye.

After the discovery of ocular immune privilege, exhaustive research has been performed, and advances have been made in the field of ocular immunology. Currently, it is clear that local and systemic pathways are involved in maintaining a well-preserved environment to guarantee normal vision. The development of autoimmunity in the eye is still a subject of research; however, it has been suggested that microglial cells could act as a gateway for initiating autoimmunity. Moreover, based on the fact that ocular involvement in systemic autoimmune diseases is well described, we aimed to collect and describe ocular diseases with a proposed primary autoimmune pathogenic mechanism. It should be noted that the autoimmune classification in several entities is a topic of discussion among authors.

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.

Increase in direct costs for health systems due to lupus nephritis: the case of Colombia.

OBJECTIVE: Lupus nephritis is one of the most severe complications of systemic lupus erythematosus and it has been estimated that can occur in up to 60% of patients. Direct costs of lupus nephritis have not been studied in developing countries. This study aimed to describe lupus nephritis direct costs in Colombia. METHODS: Administrative data from two Colombian health maintenance organizations for 2014 and 2015 was obtained. An algorithm based on the International Statistical Classification of Diseases and Related Health Problems 10th revision codes was developed to identify patients with lupus nephritis and lupus nephritis under study. RESULTS: The average annual per-patient, all-claims, all-cause direct cost for lupus nephritis was US$ 12,624, 7.5 times higher than the average lupus patient without lupus nephritis. For lupus nephritis cases under study, estimated direct cost was US$ 3,664, 2 times higher than average lupus patient in Colombia. Difference in lupus nephritis patients is mainly accounted for the cost and frequency of procedures, exceeding by a factor of 5 the cost for durable medical equipment and the cost for drugs, respectively. CONCLUSION: Lupus patients who progress to lupus nephritis stage increased seven-fold the average annual per-patient, all-claims, and all-cause direct cost for the Colombian health system.

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.

Increase in direct costs for health systems due to lupus nephritis: the case of Colombia

ABSTRACT Objective Lupus nephritis is one of the most severe complications of systemic lupus erythematosus and it has been estimated that can occur in up to 60% of patients. Direct costs of lupus nephritis have not been studied in developing countries. This study aimed to describe lupus nephritis direct costs in Colombia. Methods Administrative data from two Colombian health maintenance organizations for 2014 and 2015 was obtained. An algorithm based on the International Statistical Classification of Diseases and Related Health Problems 10th revision codes was developed to identify patients with lupus nephritis and lupus nephritis under study. Results The average annual per-patient, all-claims, all-cause direct cost for lupus nephritis was US$ 12,624, 7.5 times higher than the average lupus patient without lupus nephritis. For lupus nephritis cases under study, estimated direct cost was US$ 3,664, 2 times higher than average lupus patient in Colombia. Difference in lupus nephritis patients is mainly accounted for the cost and frequency of procedures, exceeding by a factor of 5 the cost for durable medical equipment and the cost for drugs, respectively. Conclusion Lupus patients who progress to lupus nephritis stage increased seven-fold the average annual per-patient, all-claims, and all-cause direct cost for the Colombian health system.

Caracterización epidemiológica de pacientes con uveítis en el suroccidente colombiano / Epidemiological characterization of patients with uveitis in southwestern Colombia

Introducción: La etnicidad y la geografía se han descrito como determinantes de la presentación clínica de la uveítis y su etiología. Objetivo: Caracterizara los pacientes del suroccidente colombiano con uveítis. Material y métodos: Estudio retrospectivo, del 2011 al 2019, en Cali, Colombia. Se evaluaron características demográficas, clínicas, etiológicas y desenlaces de pacientes con uveítis. Resultados: Se incluyeron 144 pacientes. La edad fue 51.5 (35.2-61.7) años, 80 (55.5%) fueron mujeres. El 66% (n = 95) presentó uveítis anterior. De las manifestaciones clínicas, el ojo rojo fue la más frecuente, seguido de dolor ocular. La mayoría tuvo síntomas < 12 semanas, presentó agudeza visual de 20/20-20/40 y rangos de presión intraocular entre 10 y 22 mmHg. De las etiologías, 20 (17.9%) continuaban en estudio y 7 (6.3%) fueron no definidas/idiopáticas. Trauma se vio en 12 (10.7%), la asociada a HLA-B27 en 10 (8.9%) y toxoplasmosis en 8 (7.1%). Conclusión: Es la primera caracterización de la uveítis en el suroccidente colombiano

Background: Ethnicity and geography have been described as determinants of the clinical presentation of uveitis and its etiology. Objective: To characterize patients from southwestern Colombia with uveitis. Material and methods: Retrospective study, from 2011 to 2019, in Cali, Colombia. Demographic, clinical, etiological characteristics and outcomes of patients with uveitis were evaluated. Results: 144 patients were included. The age was 51.5 (35.2-61.7) years, 80 (55.5%) were women. 66% (n = 95) presented anterior uveitis. Of the clinical manifestations, the red eye was the most frequent, followed by ocular pain. Most had symptoms < 12 weeks, visual acuity of 20/20-20/40 and intraocular pressure ranges between 10-22 mmHg. Of the etiologies, 20 (17.9%) were still under study and 7 (6.3%) were undefined/idiopathic. Trauma was seen in 12 (10.7%), that associated with HLA-B27 in 10 (8.9%) and toxoplasmosis in 8 (7.1%). Conclusion: It is the first characterization of uveitis in southwestern Colombia.

Systemic erythematosus lupus and pregnancy outcomes in a Colombian cohort.

OBJECTIVE: Pregnant women with SLE have higher probabilities of maternal complications. SLE during pregnancy has alternating patterns of remission and flare-ups; however, most pregnant SLE patients tend to worsen with associated poor obstetric and perinatal outcomes. This study aimed to describe obstetric outcomes in pregnant women with SLE. METHODS: This retrospective study was performed between 2011 and 2020 at a highly complex referral health center in Cali, Colombia. Pregnant women with a diagnosis of SLE were included. Demographic, clinical, and laboratory features and obstetric and fetal outcomes, including intensive care unit (ICU) characteristics, were evaluated. RESULTS: Forty-eight pregnant women with SLE were included. The median age was 29 (25-33.7) years. The SLE diagnosis was made before pregnancy in 38 (79.1%) patients, with a median disease duration of 46 (12-84) months. Thirteen (27.1%) patients had lupus nephritis. Preterm labor (34, 70.8%), preeclampsia (25, 52%), and preterm rupture of membranes (10, 20.8%) were the most common obstetric complications. A relationship between a greater systemic lupus erythematosus pregnancy disease activity index (SLEPDAI) and the development of hypertensive disorders during pregnancy was established (preeclampsia = p < 0.0366; eclampsia = p < 0.0153). A relationship was identified between lupus nephritis (LN) and eclampsia (p < 0.01), preterm labor (p < 0.045), and placental abruption (p < 0.01). Seventeen (35.4%) patients required ICU admission; 52.9% of them were due to AID activity, 17.6% for cardiovascular damage, 11.7% for septic shock, and 5.8% for acute kidney failure. Fetal survival was 89.5% (N = 43/48). Among the live births, two (4.2%) newborns were diagnosed with neonatal lupus, and two (4.2%) were diagnosed with congenital heart block. One maternal death was registered due to preeclampsia and intraventricular hemorrhage. CONCLUSIONS: This study is the first to describe SLE during pregnancy in Colombia. SLE was the most prevalent AID in this cohort, and complications included preterm labor, preeclampsia, and postpartum hemorrhage. A higher SLEPDAI and lupus nephritis predicted adverse maternal outcomes.

Validation and adaptation to Spanish of the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI).

INTRODUCTION AND OBJECTIVES: Sjögren's Syndrome (SS) is an autoimmune disease with a wide spectrum of clinical manifestations that can have an important impact on the patient's quality of life. To make an objective evaluation of the components of the disease, clinimetric tools such as the ESSPRI have been designed. The objective of this study is to adapt this scale to the Spanish language. MATERIALS AND METHODS: This is a cross-sectional study to validate clinimetric scales, carried out in Cali, Colombia. A translation of the original English version of ESSPRI into Spanish was made and applied to patients with SS, as well as PROFAD and ESSDAI, as an activity marker. The reliability index of the questionnaire in Spanish with Cronbach's alpha coefficient and Spearman's correlation coefficient were calculated to compare the scales. Demographic, clinical and laboratory characteristics were also evaluated. RESULTS: ESSPRI, PROFAD and ESSDAI were applied to 42 patients with SS, 97.62% were women. The average result of the ESSPRI was 5.8 (± 4.6), with a reliability coefficient of .8034 and a correlation with PROFAD of .5800 (p=.0001), and of -.0848 (p=.593) with ESSDAI. DISCUSSION AND CONCLUSIONS: Reliability with the applied version of ESSPRI in Spanish was adequate. A discrepancy was found between this scale and ESSDAI, which highlights the importance of applying both tools to ensure objective monitoring of disease control and its impact on the quality of life of patients with SS.

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.

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.

Therapeutic Plasma Exchange as a Treatment for Autoimmune Neurological Disease.

INTRODUCTION: Therapeutic plasma exchange (TPE) is commonly used as treatment of certain autoimmune neurological diseases (ANDs), and its main objective is the removal of pathogenic autoantibodies. Our aim was to describe the clinical profile and the experience with the usage of TPE in patients with ANDs at our institution. METHODS: This is an observational retrospective study, including medical records of patients with diagnosis of ANDs who received TPE, between 2011 and 2018. Characteristics of TPE, such as number of cycles, type of replacement solution, and adverse effects, were evaluated. The modified Rankin Scale (mRS) was applied to measure the clinical response after the therapy. RESULTS: 187 patients were included with the following diagnoses: myasthenia gravis (MG), n = 70 (37%); Guillain-Barré syndrome (GBS), n = 53 (28.3%), neuromyelitis optica spectrum disorders (NMOSD), n = 35 (18.7%); chronic inflammatory demyelinating polyneuropathy (CIDP), n = 23 (12.2%); and autoimmune encephalitis (AE), n = 6 (3.2%). The most used types of replacement solution were albumin (n = 131, 70%) and succinylated gelatin (n = 45, 24%). All patients received a median of five cycles (IQR 5-5). Hypotension and hydroelectrolytic disorders were the main complications. After TPE, 99 patients (52.9%) showed improvement in the mRS scores and a statistical significance (p < 0.05) was seen between the admission score and after TPE for every diagnosis except for CIDP. CONCLUSION: TPE has an adequate safety profile, and improvement in functionality in treated patients reflects its effectiveness.