Performance of the 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease in a Latin American Cohort.

BACKGROUND/OBJECTIVE: The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients. METHODS: A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied. RESULTS: We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes. CONCLUSIONS: The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria.

[Creatine phosphokinase enzyme elevation in two third-level hospitals of Córdoba]. / Estudio de los pacientes con elevación de enzima Creatinfosfokinasa en dos hospitales de tercer nivel de la ciudad de córdoba.

Introduction: HyperCKemia is defined as the elevation of creatine phosphokinase (CK) levels greater than 1.5 times the upper limit (CK>285 U/L), being produced by multiple causes, which vary according different populations. The main objective of the study was to know the frequency of hyperCKemia in two hospitals in Córdoba and its main causes. Methods: Retrospective analytical study in two hospitals in Córdoba, Argentina, where all patients over 18 years of age who presented CK values ​​greater than 285 U/L on at least 2 occasions in a period between 1 and 4 weeks were identified, between the years 2015 and 2017. Results: 254 patients with hyperCKemia were identified, the majority were male (n=181, 71.3%) and their median age was 65 years (interquartile range 25-75%=50-73 years). The main causes of hyperCKemia were ischemic myopathy in 99 (39%) patients and drug-induced myopathies in 45 (17.7%), with statins being the most frequently involved drugs in 31 cases. In only 3.1% of the cases the final diagnosis was not reached. Drug-induced hyperCKemias, when compared to other causes, occurred more frequently in patients with a history of chronic kidney disease (9/45 [20%] vs 18/209 [8.6%], p = 0.025) and in non-smokers (41/45 [91.1%] vs. 157/209 [75.1%], p=0.019). Main conclusion: 254 cases of hyperCKemia were observed, the main causes being of ischemic origin and secondary to drug use, especially statins.

Introducción: Se denomina hiperCKemia a la elevación de niveles de creatinfosfoquinasa (CK) mayor a 1,5 veces el límite superior(CK>285 U/L), siendo producida por múltiples causas, que varían según las poblaciones de estudio. El objetivo principal del estudio fue conocer la frecuencia de hiperCKemia en dos hospitales de la Ciudad de Córdoba y sus principales causas. Metodología: Estudio analítico retrospectivo en dos hospitales de la ciudad de Córdoba en Argentina, donde se identificaron todos los pacientes mayores de 18 años que presentaron valores de CK mayores a 285 U/L en al menos 2 oportunidades en un período entre 1 y 4 semanas, entre los años 2015 y 2017. Resultados: Se identificaron 254 pacientes con hiperCKemia, la mayoría eran de sexo masculino (n=181, 71.3%) y su mediana de edad fue 65 años (rango intercuartil 25-75%=50-73 años). Las principales causas de hiperCKemia fueron la miopatía isquémica en 99 (39%) pacientes y las miopatías inducidas por drogas en 45 (17.7%), siendo las estatinas las drogas más frecuentemente involucradas en 31 casos. En sólo el 3.1% de los casos no se arribó al diagnóstico final. Las hiperCKemias inducidas por drogas al compararse con las otras causas, se presentaron más frecuentemente en pacientes con antecedente de enfermedad renal crónica (9/45[20%] vs 18/209[8.6%], p=0.025) y en no tabaquistas(41/45[91.1%] vs 157/209[75.1%], p=0.019) . Conclusión: Se observaron 254 casos de hiperCKemia, siendo sus principales causas de origen isquémico y secundario a consumo de fármacos, especialmente por estatinas.

Clinical and Serological Features in Latin American IgG4-Related Disease Patients Differ According to Sex, Ethnicity, and Clinical Phenotype.

BACKGROUND/OBJECTIVE: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD. METHODS: We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation. RESULTS: The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance. CONCLUSIONS: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype.

IgG4-Related Disease: Mimickers and Diagnostic Pitfalls.

BACKGROUND: The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered. SUMMARY OF THE LITERATURE: The main differential diagnoses for the pancreato-hepato-biliary phenotype are pancreatic adenocarcinoma and cholangiocarcinoma. Other differential diagnoses include type 2 autoimmune pancreatitis and primary sclerosing cholangitis. In patients with retroperitoneal/aortic phenotype, inflammatory conditions such as idiopathic retroperitoneal fibrosis and large vessel vasculitides should be ruled out, and most of the time, a biopsy will be needed to exclude malignancies. In head and neck limited phenotype, autoimmune conditions (eg, granulomatosis with polyangiitis, Graves orbitopathy, sarcoidosis), malignancies, and histiocytosis should be ruled out, whereas the main differential diagnoses of the Mikulicz/systemic phenotype are Sjögren syndrome, granulomatosis with polyangiitis, and multicentric Castleman disease. CONCLUSIONS: Approaching a patient with probable IgG4-RD through a clinical phenotype framework will ease the diagnostic algorithm and facilitate the prompt recognition of the disease. There are certain clinical, serological, radiological, and histological features in each clinical phenotype that, if present, increase the likelihood that a patient may have IgG4-RD instead of the mimicker condition. Those clues that point toward IgG4-RD diagnosis should be actively sought in the workup of patients.

Differential diagnosis between pancreatic involvement in IgG4-related disease and pancreatic cancer. / Diagnóstico diferencial entre compromiso pancreático en enfermedad relacionada con IgG4 y cáncer de páncreas.

IgG4-related disease is a systemic disorder characterised by diffuse or tumoural inflammatory lesions. It can mimic pancreatic cancer, leading to errors in diagnosis and treatment increasing rates of morbidity and mortality in patients. The aim of this review is to take a differential diagnostic approach to these two entities using epidemiology, clinical and laboratory findings, imaging and histopathology.

Síndrome de pulmón encogido y derrame pleural como manifestación inicial de síndrome de Sjögren primario / Shrinking lung syndrome and pleural effusion as an initial manifestation of primary Sjögren's syndrome

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Posterior reversible leukoencephalopathy syndrome: Case series and review of the literature. / Síndrome de leucoencefalopatía posterior reversible: serie de casos y revisión de la literatura.

OBJECTIVE: To describe clinical manifestations, antecedents, comorbidities and associated treatments, imaging findings, and follow-up in patients with posterior reversible encephalopathy syndrome. METHODS: A retrospective, descriptive analysis of admitted patients was performed between June 2009 and May 2014 in a third-level care hospital. We evaluated age, sex, comorbidities, symptoms, values of blood pressure at admission, renal function, medication and time elapsed until the disappearance of symptoms. RESULTS: Thirteen patients were included. In all, 77% of them had a history of hypertension at baseline and 85% had impaired renal function. The most prevalent comorbidity was renal transplantation, and 85% had deterioration of renal function. Five of the patients had undergone renal transplantation. The most common clinical manifestation was seizures. All had subcortical lesions and bilateral parietooccipital involvement was the finding most frequently observed. CONCLUSION: This syndrome should be taken into account in the differential diagnoses of patients presenting with acute neurological syndromes and the abovementioned risk factors.

[Characteristics of bloodstream infections in adult patients of two third level centers of Córdoba, Argentina.] / Características de las infecciones del torrente sanguíneo en pacientes adultos de dos centros de tercer nivel de Córdoba, Argentina.

Background: Bloodstream infections (BI) are associated with high morbidity and mortality. Objective: To determine epidemiological, microbiological and clinical features of community (CA-BI) and nosocomial bloodstream infections (N-BI). Methods: Bacteremia and fungemia events were retrospectively analyzed in two third-level hospitals between April 2009 and August 2013. Results: We identified 1150 events of bloodstream infections, 53.2% were CA-BI. Gram negative microorganisms were isolated in 61%. The most frequent pathogens were Escherichia coli in CA-BI and Klebsiella pneumoniae in N-BI. Staphylococcus aureus was the most frequent gram positive organism. The main comorbidities were renal disease (39%) and malignancy (38%). There were 26.8% of primary bloodstream infections, and the main infection foci included respiratory (17.04%) and urinary tract (16.86%). A high percentage of gram-negative bacteria of CA-BI and N-BI were resistance to ampicillin sulbactam (40.2% and 57.5%), cephalothin (36.7% and 46.8%), trimethoprim-sulfamethoxazole 32.8% vs 35.5%) and ciprofloxacin (24.6% and 35.3%). Methicillin-resistant Staphylococcus aureus were more frequently into ITS-IH (31.4% vs 11.8%, p = 0.007). Conclusions: Clinical and epidemiological characteristics of CA-BI and N-BI were similar to those reported by other Latin-American studies. We observed some differences in antimicrobial resistance profiles. We emphasize the importance of local epidemiological surveillance to choose appropriate empirical treatments. Conclusions: Clinical and epidemiological characteristics of CA-BI and N-BI were similar to those reported by other Latin-American studies. We observed some differences in antimicrobial resistance profiles. We emphasize the importance of local epidemiological surveillance to choose appropriate empirical treatments.

Introducción: Las infecciones del torrente sanguíneo (ITS) generan elevada morbimortalidad. Objetivo: Determinar características epidemiológicas, microbiológicas y clínicas de ITS adquiridas en la comunidad (ITS-AC) e intrahospitalarias (ITS-IH). Métodos: Se analizaron retrospectivamente eventos de bacteriemia y fungemia en dos hospitales de tercer nivel entre abril de 2009 y agosto de 2013. Resultados: Se identificaron 1150 eventos de ITS, 53% ITS-AC. El 61% de microorganismos aislados fueron gram negativos. Los patógenos más frecuentes fueron Escherichia coli en ITS-AC y Klebsiella pneumoniae en ITS-IH. Staphylococcus aureus fue el gram positivo más frecuente. Las principales comorbilidades fueron enfermedad renal (39%) y neoplasias (38%). El 26,8% de ITS fueron primarias. Los focos infecciosos más frecuentes fueron respiratorio (17%) y urinario (16,9%). Un elevado porcentaje de gram negativos en ITS-AC e ITS-IH fueron resistentes a ampicilina sulbactam (40,2% y 57,5%), cefalotina (36,7% y 46,8%), trimetoprima-sulfametoxazol (32,8% vs 35,5%) y ciprofloxacina (24,6% y 35,3%). Staphylococcus aureus meticilino resistente fue más frecuente en ITS-IH (31,4% vs 11,8%, p=0,007). Conclusiones: Las características clínicas y epidemiológicas de ITS fueron similares a las reportadas por otros estudios latinoamericanos. Pero observamos algunas diferencias en los perfiles de susceptibilidad antimicrobiana. Resaltamos la importancia de la vigilancia epidemiológica local para elegir tratamientos empíricos apropiados.

[Nephrocalcinosis and proximal tubulopathy in Sjögren's Syndrome.] / Nefrocalcinosis y acidosis tubular renal proximal en síndrome de Sjögren.

Primary Sjögren's syndrome is a systemic and chronic autoimmune disease. Renal involvement may occur in up to 30% of patients. The incidence of tubulopathies ranges from 2.6 to 33%. They are manifested by defects in the urine concentration and hydroelectrolyte alterations, mainly distal tubular acidosis and exceptionally proximal tubular acidosis. These disorders can be associated with nephrocalcinosis and renal lithiasis. We report the case of a patient with primary Sjögren who presented proximal renal tubular acidosis associated with recurrent renal colic due to renal lithiasis and nephrocalcinosis. We highlight the importance of diagnosing renal tubular acidosis in patients with Sjögren's syndrome that present alterations in urinary sediment and electrolyte disorders to avoid nephrocalcinosis and nephrolithiasis. Acidosis correction treatment aims to prevent the progression of the disorder and preserve renal function.

El síndrome de Sjögren primario es una enfermedad autoinmune sistémica de evolución crónica. Puede presentar compromiso renal hasta en un 30% de los pacientes.La incidencia de tubulopatías varía de 2.6 a 33%. Se manifiestan por defectos en la concentración de la orina y alteraciones hidroelectrolíticas, principalmente acidosis tubular distal y de manera excepcional acidosis tubular proximal. Estos trastornos pueden asociarse a Nefrocalcinosis y litiasis renal. Reportamos el caso de una paciente con Sjögren primario que presentó acidosis tubular renal proximal asociada a cólicos renales recurrentes por litiasis renal y nefrocalcinosis. Destacamos la importancia de diagnosticar acidosis tubular renal en pacientes con síndrome de Sjögren que presenten alteraciones en el sedimento urinario y desórdenes electrolíticos para evitar la nefrocalcinosis medular y las nefrolitiasis asociadas. La terapia para la corrección de la acidosis tiene como objetivo evitar la progresión del trastorno y preservar la función renal.

[Uncomplicated urinary tract infection in women: etiology and antimicrobial resistance]. / Infección urinaria no complicada en mujeres: etiología y resistencia a antimicrobianos.

Our objectives were to determine the etiology and analyze the antibiotic resistance profiles of microorganisms causing uncomplicated urinary tract infections in our setting. An analytical cross-sectional study was conducted. In vitro antimicrobial resistance of urine cultures was analyzed. 580 urine cultures of women over age fifteen were included. 82.6 % of urine cultures corresponded to cystitis and the remaining 17.4 % corresponded to pyelonephritis. 353 urine cultures of women <50 years old (60.9%) and 227 of women = 50 years old (39.1%) were obtained. The most common pathogens were Escherichia coli (85.5 %) and Klebsiella pneumoniae (4.7 %). For Escherichia coli, there was a resistance of 28.6% to trimethoprim-sulfamethoxazole,7.9% to ciprofloxacin and 0.4% to nitrofurantoin. Significant difference (p = 0.005) was seen in the resistance to ciprofloxacin in women = 50 years old. Our data show there is a low in vitro resistance to nitrofurantoin

Nuestros objetivos fueron determinar la etiología y analizar los perfiles de resistencia antimicrobiana de los microorganismos causantes de infecciones urinarias no complicadas en nuestro medio. Se realizó un estudio analítico de corte transversal. Se analizó la resistencia antimicrobiana in vitro de los urocultivos. Se incluyeron 580 urocultivos de mujeres mayores de 15 años. Un 82.6% de urocultivos correspondieron a cistitis y el 17.4% a pielonefritis.Se obtuvieron 353 urocultivos de mujeres < 50 años (60.9%) y 227 a ? 50 años (39.1%).Los patógenos más frecuentes fueron: Escherichia coli (85.5%) y Klebsiella pneumoniae (4.7%). Se encontró una resistencia de E coli a trimetoprima-sulfametoxazol del 28.6%, a ciprofloxacina de 7.9% y a nitrofurantoína de 0.4%. Se evidenció diferencia significativa (p=0.005) en la resistencia de E coli a ciprofloxacina en las mujeres ?50 años de edad. Nuestros datos muestran que existe una baja resistencia in vitro a nitrofurantoína.