Type I acquired cutis laxa: Report of a unique progressive case and short review.

Cutis laxa is a rare connective tissue disorder, characterized by a reduced number and abnormal properties of elastic fibers throughout the dermis, creating a clinical appearance of premature aging. It can be subdivided into inherited and acquired, the latter rarer than the former, and skin involvement may be localized or generalized. The etiology of acquired cutis laxa (ACL) remains unknown and there is no definitive treatment. We present the case of a 30-year-old man diagnosed with type I ACL with progressive systemic involvement at the renal, pulmonary, and digestive levels. Histological analysis of the skin revealed reduction and fragmentation of elastic fibers. Immunosuppressive treatment was started with prednisone, cyclophosphamide, and rituximab, with which a complete response to proteinuria was achieved and the progression of lung damage was limited. Autoimmune, infectious, and neoplastic diseases were ruled out.

Influence of visceral adiposity on cardiovascular risk in patients with systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic inflammation, endothelial dysfunction, generalized fibrosis and high cardiovascular mortality. The evaluation of cardiovascular risk through the visceral adiposity index (VAI) has been helpful due to its direct relationship to the body and visceral fat percentage. We evaluated the influence of body composition and anthropometrics on cardiovascular risk as measured by VAI in healthy controls (HC) and SSc. An analytical cross-sectional study of 66 participants (33 SSc and 33 HC), mean age 52.7 ± 10, 95% women, was conducted from August 2020 to January 2021. Inclusion criteria in cases were consecutive patients with SSc (ACR/EULAR 2013), 63.6% were diffuse cutaneous (dcSS) subtype, and 36.4 were limited cutaneous (lcSS) subtype. HC was matched by age and gender. Serum lipid profiles and InBody anthropometrics were analyzed and compared. We performed descriptive statistics, bivariate analysis with Student's t, or Mann-Whitney U, correlation and chi-square according to the variable type and distribution. Total cholesterol was significantly higher in SSc than HC (345 vs 194, p = < 0.001). The BMI was higher in HC (26.2 vs 28.9, p < 0.001). Kilograms of muscle (19.8 vs 28.9, p < 0.001) and total fat (23.4 vs 28.9, p < 0.001) were lower in SSc patients compared to HC. VAI was similar when BMI < 25, but significantly higher when BMI > 25 in SSc than in HC (3 vs 1.9, p = 0.030). The increase in BMI at overweight or obese in SSc is associated with a significant increase in cardiovascular risk.

[Giant tongue leading to dysphagia in light chain amyloidosis patient]. / Lengua gigante condicionante de disfagia en paciente con amiloidosis de cadenas ligeras.

Introduction: Macroglossia as a clinical manifestation of systemic amyloidosis is a rare condition, occurring in less than 9% of all types of amyloidosis. The aim of this report is to present the diagnostic approach of a patient with macroglossia, providing a systematic approach and considering relevant diagnostic possibilities during their evaluation. Clinical case: We present the case of a 60-year-old man who presented with a progressively enlarging giant tongue for six months, causing dysphagia and reduced oral opening. A tongue biopsy was taken, which histopathologically exhibited homogenous eosinophilic amyloid-like material. Congo red staining showed amyloid material with red dye under light microscopy and apple-green birefringence under polarized light. Bone marrow biopsy showed 30% plasma cells, allowing for a definitive diagnosis of soft tissue amyloidosis. Although it is a benign lesion, localized amyloidosis should be differentiated from systemic forms. Conclusions: The approach of patients with macroglossia is complex due to the diagnostic possibilities, from endocrinological causes, neoplastic, and even by deposit; Being an isolated sign in a patient is a challenge in its approach, because the involvement of the airway is the main complication to avoid in these patients.

Introducción: la macroglosia, como manifestación clínica de la amiloidosis sistémica, es una condición poco frecuente, ya que ocurre en menos del 9% de todos los tipos de amiloidosis. El objetivo de este informe es presentar el abordaje diagnóstico de un paciente con macroglosia, proporcionando un enfoque sistemático y considerando las posibilidades diagnósticas relevantes durante su evaluación. Caso clínico: presentamos el caso de un hombre de 60 años que presentó una lengua gigante de crecimiento progresivo durante seis meses, lo que causó disfagia y reducción en la apertura oral. Se realizó una biopsia de la lengua, la cual histopatológicamente exhibió material homogéneo similar a un amiloide eosinofílico. La tinción de rojo Congo mostró material amiloide con colorante rojo bajo microscopía óptica y con birrefringencia verde manzana bajo luz polarizada. La biopsia de médula ósea mostró un 30% de células plasmáticas, lo que permitió realizar un diagnóstico definitivo de amiloidosis de tejidos blandos. Aunque se trata de una lesión benigna, la amiloidosis localizada debe diferenciarse de las formas sistémicas. Conclusiones: el abordaje de pacientes con macroglosia es complejo, debido a las diversas posibilidades diagnósticas, que incluyen causas endocrinológicas, neoplásicas e, incluso, por deposición de sustancias. Cuando se presenta como un signo aislado en un paciente, se convierte en un reto en su abordaje, ya que la principal complicación a evitar en estos pacientes es el compromiso de la vía aérea.

[Ethiology, risk factors and Whole blood viscosity index in thromboembolic venous disease]. / Etiología, factores de riesgo e Índice de viscosidad sanguíneo total en enfermedad tromboembólica venosa.

Backround: Venous thromboembolic disease (VTED) is a frequent cause of hospitalization and mortality. Whole blood viscosity (WBV) participates in the pathogenesis of thrombosis. Objective: To identify the most frequent etiologies and their association with WBV index (WBVI) in hospitalized patients with VTED. Material and methods: Observational, cross-sectional, retrospective, analytical study, Group 1: cases (patients diagnosed with VTED) and Group 2: controls without thrombosis. Risk factors for VTED were described and WBVI was calculated from total proteins and hematocrit. Descriptive and inferential statistics were used with Chi-squared test, Fisher's exact test, Mann Whitney U test, bivariate and multivariate logistic regression analysis. Results: We included 146 patients and 148 controls, age 46.3 ±17.7 vs. 58 ± 18.2 years, of both sexes (female, 65.1%). The most frequent etiology was neoplastic (23.3%), followed by diseases with cardiovascular risk (17.8%). Independent risk factors for VTED were age, chronic kidney disease, presence of liver disease or solid neoplasia. WBVI was similar in patients with VTED as in those without thrombosis. We found an association of the presence of deep vein thrombosis and diseases with cardiovascular risk (p = 0.040). Conclusions: The presence of chronic kidney disease, liver disease, and solid neoplasia are independent risk factors for VTED. The WBVI is a simple and rapid diagnostic tool in the evaluation of patients with VTED.

Introducción: la enfermedad tromboembólica venosa (ETEV) es causa frecuente de hospitalización y mortalidad. La viscosidad sanguínea participa en la patogénesis de la trombosis. Objetivo: analizar los factores de riesgo y el índice de viscosidad sanguíneo total (IVTS) en pacientes con ETEV. Material y métodos: estudio observacional, transversal, retrospectivo, analítico. Grupo 1: casos (pacientes con diagnóstico de ETEV), y grupo 2: controles sin trombosis. Se describieron los factores de riesgo para ETEV y se calculó el IVTS a partir de proteínas totales y hematocrito. Se utilizó estadística descriptiva e inferencial con prueba de Chi cuadrada, prueba exacta de Fisher, U de Mann Whitney, análisis de regresión logística bivariado y multivariado. Resultados: incluimos 146 pacientes y 148 controles, edad 46.3 ± 17.7 frente a 58 ± 18.2 años, de ambos sexos, femenino (65.1%). La etiología más frecuente fue la neoplásica (23.3%), seguida de la enfermedad con riesgo cardiovascular (17.8%). Los factores de riesgo independientes para ETEV fueron: edad, enfermedad renal crónica, presencia de hepatopatía o neoplasia sólida. El IVTS fue similar en los pacientes con ETEV que en aquellos sin trombosis. Se encontró asociación de la presencia de trombosis venosa profunda y enfermedades con riesgo cardiovascular (p = 0.040). Conclusiones: la presencia de ERC, hepatopatía y neoplasia sólida son factores de riesgo independientes para ETEV. El IVTS es un instrumento diagnóstico sencillo y rápido en la evaluación de los pacientes con ETEV.

[The impact of COVID-19 in a cohort of patients with systemic sclerosis]. / El impacto de COVID-19 en una cohorte de pacientes con esclerosis sistémica.

Background: COVID-19 brought with it unknowns related to systemic sclerosis. Objective: To know the clinical evolution and prognosis of COVID-19 in a cohort of patients with systemic sclerosis. Methods: During the pandemic, we had digital contact with a cohort of 197 patients with SSc. If they had any condition that met the suspected definition of COVID-19, they underwent polymerase chain reaction testing for SARS-CoV-2; they were treated on an outpatient or hospital basis without interfering with their treatment. They followed their evolution every 24 hours until they became asymptomatic or died. Results: Thirteen patients (6.6%), nine diffuse cutaneous (dcSSc), and four limited cutaneous (lcSSc) developed COVID-19 during nine months of follow-up. The immunosuppressants used at the time of the disease were: mycophenolate mofetil, methotrexate, and prednisone, in low doses. Seven patients had interstitial lung disease (ILD). The main symptoms were chest pain, cough, dyspnea, dysgeusia, and anosmia, 1 with mild symptoms without pneumonia, 11 with mild pneumonia, and one with severe pneumonia that required hospital management. Only one (7.7%) presented severe pneumonia, was hospitalized, and died. Conclusions: COVID-19 disease in patients with SSc can be overcome in most cases, even when they are ILD and were using immunosuppressants at the time of infection with the SARS-CoV-2 virus.

Introducción: la COVID-19 trajo consigo incógnitas relacionadas con la esclerosis sistémica, enfermedad de baja prevalencia asociada a neumopatía intersticial difusa (NID). Objetivo: conocer la evolución clínica y el pronóstico de la COVID-19 en una cohorte de pacientes con esclerosis sistémica (ES). Métodos: se analizó una serie de 13 casos procedentes de una cohorte de 197 pacientes con ES en seguimiento vía digital. Cuando los pacientes cumplieron con la definición sospechosa de COVID-19 se solicitó prueba de reacción en cadena de polimerasa para SARS-CoV-2. Todos los pacientes recibieron seguimiento durante su atención ambulatoria u hospitalaria, sin interferir con su tratamiento cada 24 horas hasta quedar asintomáticos o fallecer. Resultados: de 197 pacientes, trece (6.6%) enfermaron de COVID-19 de edad 57 años (RIC: 52-63), cutáneos difusa (ESD) y 4 limitada (ESL) en lapso de 9 meses. Once presentaron neumonía leve (84%), una neumonía grave con fallecimiento intrahospitalario (7.7%). La oximetría media al ambiente se mantuvo en SO2 90% (88-92%). Casi todos usaban inmunosupresores (84%) al momento de enfermar: micofenolato de mofetilo, metotrexato, prednisona en dosis bajas. Siete (53%) tenían enfermedad pulmonar intersticial (EPI) previa. Principalmente manifestaron disnea (67.5%), dolor torácico, tos, disgeusia y anosmia. Conclusiones: es posible que al momento del contagio con el virus SARS-CoV-2 los inmunosupresores permitieran una menor respuesta inflamatoria sistémica, evitando un peor pronóstico, incluso en quienes tienen enfermedad intersticial previa.

High mortality at the beginning of the COVID-19 pandemic in a referral center in the metropolitan area of Mexico City.

INTRODUCTION: There is limited information about the coronavirus disease 2019 (COVID-19) disease in Latin-American countries. Our objective was to describe the clinical characteristics and outcomes of COVID-19 patients in Mexico. METHODOLOGY: We conducted a retrospective cohort study with 333 consecutive patients who were admitted to Hospital de Especialidades "Dr. Antonio Fraga Mouret" in Mexico City with COVID-19 between April 1, 2020, and June 30, 2020. Demographic, clinical, laboratory data, treatment details and 30-day outcomes were analyzed. RESULTS: The patients studied included 52% men (172/233) and the median age was 45 years. Up to 75% (250/333) of patients were classified as overweight or obese. There were 185 (56%) inpatients; 85% (158/185) were hospitalized in the general ward, and 15% (27/185) in the Intensive Care Unit (ICU). Laboratory measurements showed significant differences between inpatients and outpatients such as lymphocyte-count (median 0.8 vs 1.2×109/L, p < 0.001), LDH (median 650 vs 294 U/L, p < 0.001), CRP (median 147 vs 5 mg/L, p = 0.007), CK-MB (median, 15 vs 10 U/L, p = 0.008), ferritin (median, 860 vs 392 ng/mL, p = 0.02), and D-dimer (median, 780 vs 600 ng/mL, p = 0.15). These differences were seen between survivor and non-survivor patients as well. The rate of death in mechanically ventilated patients was 94% (67/71). Mortality at 30-day follow-up was 57% (105/185). CONCLUSIONS: We observed that majority of the non-survivors were obese and young. Complications leading to death was observed in majority of the cases.

Protective HLA alleles against severe COVID-19: HLA-A*68 as an ancestral protection allele in Tapachula-Chiapas, Mexico.

HLA is a polymorphic antigen presenter which has provided valuable information on the susceptibility of populations to viruses. Therefore, the study of HLA can reveal specific susceptibility or resistance alleles to severe COVID-19 in an ethnically dependent manner. This pilot study investigated HLA alleles associated with COVID-19 severity in Tapachula, Chiapas, Mexico. A total of 146 Mexican Mestizos were typed for HLA class I and II using PCR-SSP. The patients were classified according to the outcome (death or improvement) and the infection's severity (mild or severe). In addition, a group of exposed uninfected individuals was included. HLA-A*68 was found to be a protective allele against the severe infection and fatal outcome; pC = 0.03, OR = 0.4, 95% CI =0.20-0.86, and pC =0.009, OR = 0.3, 95% CI =0.13-0.71 respectively. HLA-DRB1*03 also appears to be a protective factor against fatal outcome pC = 0.009, OR = 0.1, 95%IC = 0.01-0.66; however, the low frequency of this allele in the studied population limits the statistical power. The severity and fatal outcome of COVID-19 patients in Tapachula, Chiapas depend more on the lack of resistance than susceptibility HLA alleles.

Kikuchi-Fujimoto Disease: Report of a Case with Progression to Lupus Nephritis.

BACKGROUND Kikuchi-Fujimoto disease (KFD) is an enigmatic disease, with a distinctive histopathology and a benign and self-limited course. It is more frequent in young Asian women. Autoimmune diseases are identified as one of its triggers; primarily SLE, which may precede, be concomitant with, or develop after the diagnosis of KFD. Patients with KFD should receive periodic follow-up for several years to detect possible evolution of SLE. The main feature of KFD is lymphadenopathy, and cervical lymph nodes are involved in 50% to 98% of cases. Other symptoms such as fever, fatigue, weight loss, and arthralgias are also reported. Differential diagnosis between KFD and SLE is a challenge. When KFD and SLE coexist, a lymph node biopsy may be diagnostic. Treatment should be symptomatic with analgesics and anti-inflammatories, with complete resolution in 3 to 4 months. Corticosteroids and immunosuppressive therapy are justified only in cases concomitant with SLE. CASE REPORT We report a case of KFD in a 28-year-old woman who was initially negative for anti-nuclear antibodies (ANA) and anti-double-stranded deoxyribonucleic acid antibodies (anti-dsDNA), but who became antibody-positive and presented with lupus nephritis 2 months later. CONCLUSIONS We present a case of a patient with KFD who developed SLE 2 months later; highlighting the importance of recognizing its association and its possible progression to monitor for future development of SLE and provide timely treatment to avoid complications. We also compared the clinical, laboratory, and histological similarities between the 2 entities.

[Pulmonary alveolar proteinosis. Report of a case]. / Proteinosis alveolar pulmonar. Reporte de un caso.

BACKGROUND: The pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of lipoprotein material in the alveoli. It is classified as primary, secondary and congenital. The primary form, of autoimmune origin, is characterized by antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF). The aim of this article is to present a case of PAP with adequate response to treatment with bronchoalveolar lavage (BAL) and GM-CSF. CLINICAL CASE: A 28-year-old female patient who started with mild to moderate effort dyspnea, distal cyanosis and lower respiratory tract infection. We found restrictive pattern respiratory function tests, chest X-ray with bilateral nodular reticulum pattern and high-resolution tomography with ground glass image and bronchiectasis, besides video bronchoscopy with inflammatory process. The open biopsy revealed data of alveolar proteinaceous material and mononuclear infiltrate. Treatment was given with BAL and GM-CSF which was suspended by dermal reaction. The patient had a satisfactory evolution and is currently asymptomatic. CONCLUSION: The present case had clinical, imaging and histological manifestations for the diagnosis of autoimmune PAP with a satisfactory response to treatment. Although PAP is a low prevalence entity, the diagnosis and therapeutic options must be taken into account, including BAL and GM-CSF, since this factor is required for surfactant factor homeostasis.

INTRODUCCIÓN: la proteinosis alveolar pulmonar (PAP) se caracteriza por la acumulación de material lipoproteináceo en los alveolos, y se clasifica en primaria, secundaria y congénita. La primaria, de origen autoinmune, se caracteriza por anticuerpos contra el factor estimulante de colonia de granulocitos y macrófagos (GM-CSF). El objetivo del presente trabajo es presentar un caso de PAP con buena respuesta al tratamiento con lavado broncoalveolar (LBA) y GM-CSF. CASO CLÍNICO: Caso clínico: distal e infección de vías respiratorias bajas. Se efectuaron pruebas de función respiratoria con patrón restrictivo, radiografía de tórax con patrón retículo nodular bilateral y tomografía de alta resolución con imagen de vidrio despulido y bronquiectasias, así como video broncoscopía con proceso inflamatorio. La biopsia a cielo abierto evidenció datos de material proteináceo alveolar e infiltrado mononuclear. Se dio tratamiento con LBA y GM-CSF, el cual fue suspendido por reacción dérmica. Tuvo evolución satisfactoria y actualmente se encuentra asintomática. CONCLUSIÓN: el presente caso tuvo manifestaciones clínicas, de imagen e histológicas para el diagnóstico de PAP autoinmune con respuesta satisfactoria. Para el tratamiento de la PAP, aunque es una entidad de baja prevalencia, se debe tener en cuenta el diagnóstico y las opciones terapéuticas, entre ellas, el LBA y el GM-CSF, puesto que este factor se requiere para la homeostasis del factor surfactante.

Púrpura de Henoch-Schönlein como manifestación inicial de mieloma múltiple / Henoch-Schonlein purpura as initial manifestation of multiple myeloma

Resumen La púrpura de Henoch-Schönlein como vasculitis paraneoplásica de tumores sólidos se encuentra en 9 a 11% de los casos reportados en adultos con carcinoma de estómago, mama, pulmón, próstata o riñón; es poco frecuente como paraneoplásico de mieloma múltiple. Se comunica el caso de una paciente que padeció púrpura de Henoch-Schönlein como manifestación inicial de mieloma múltiple.

Abstract Henoch-Schonlein purpura as paraneoplasic vasculitis of solid tumors is found in 9-11% of cases reported in adults with stomach, breast, lung, prostate and kidney carcinomas; it is little frequent as paraneoplastic of multiple myeloma. This paper reports the case of a patient that suffered from Henoch-Schonlein purpura as initial manifestation of multiple myeloma.

[IgG4-related disease: a case report]. / Enfermedad asociada a IgG4: reporte de un caso.

IgG4-related disease is an inflammatory condition characterized by high levels of IgG4. It affects salivary and lacrimal glands, pancreas, lymph nodes, lungs or kidney. The diagnosis is based on identifying a histological pattern with a dense lymphocyte and plasmacyte infiltration, focal fibrosis or phlebitis, finding more than 10 IgG4 positive cells per high power field and/or IgG4/IgG ratio in plasma higher than 40%. We present a patient with Mikulicz's disease who meets histological findings required for the diagnosis of IgG4 related disease.

La enfermedad relacionada con IgG4 es una condición fibroinflamatoria en la que existe elevación de IgG4, afección a nivel de glándulas salivares, lacrimales, páncreas, ganglios linfáticos y pulmón. Para su diagnóstico se requiere la identificación de un patrón histológico sugestivo que muestre infiltrado linfoplasmocitario denso, fibrosis focal o flebitis a nivel de una glándula, más de 10 células positivas para IgG4 por campo de gran aumento y relación de IgG4/IgG arriba de 40% en plasma. Describimos el caso de una paciente que presentó enfermedad de Mikulicz y cumplió con los datos histológicos para diagnóstico de enfermedad relacionada con IgG4.

Clinical, biochemical, and radiological characterization of the calcinosis in a cohort of Mexican patients with systemic sclerosis.

Calcinosis is a frequent complication of systemic sclerosis (SSc) that is usually located in extremities but may occur across the board. The aim of our study was to identify and quantify the distribution of calcinosis in a cohort of Mexican patients with SSc and its association with clinical features and autoantibodies. A cohort of patients with SSc (2013 ACR/EULAR criteria), classified in diffuse cutaneous (dcSSc) and limited cutaneous (lcSSc) (Le Roy criteria), was studied. For their analysis, patients were allocated into those with and without calcinosis (clinical and/or radiological). The evaluation included the modified Rodnan scale for skin and Medsger disease severity scale (DSS). Calcium, phosphorus, vitamin D, and parathyroid hormone (PTH) and antinuclear antibodies and extractable nuclear antigens were determined in serum. A total of 109 patients were included, 41 (37 %) with and 68 (63 %) without calcinosis. Calcinosis was more frequent in patients with dcSSc (55 vs 27 %). In total, we identified 354 sites with calcinosis and mean per patient of 12.0 ± 9.1; the most common sites affected were the hands (83 %), proximal upper extremity (27 %), and proximal lower extremity (22 %). Patients with calcinosis had a higher score of Rodnan scale, Mesdger DSS, and frequency of anti-nucleolar and anti-Scl-70 antibodies compared to those without calcinosis. Abnormal PTH elevation was found in 35 % of patients with calcinosis and 23 % without it. The prevalence of calcinosis is high in Mexican patients with SSc, especially in diffuse variety, and is associated with increased severity of disease.

Detection of autoantibodies to DSF70/LEDGFp75 in Mexican Hispanics using multiple complementary assay platforms.

PURPOSE: Antinuclear autoantibodies (ANA) targeting the dense fine speckled antigen DFS70, also known as lens epithelium-derived growth factor p75 (LEDGF/p75), are attracting attention due to their low frequency in systemic rheumatic diseases but increased frequency in clinical laboratory referrals and healthy individuals (HI). These ANA specifically recognize the stress protein DFS70/LEDGFp75, implicated in cancer, HIV-AIDS, and inflammation. While their frequency has been investigated in various ethnic populations, there is little information on their frequency among Hispanics/Latinos. In this study, we determined the frequency of anti-DFS70/LEDGFp75 autoantibodies in Mexican Hispanics using multiple detection platforms. METHODS: The frequency of anti-DFS70/LEDGFp75 antibodies was determined in 171 individuals, including 71 dermatomyositis (DM) patients, 47 rheumatoid arthritis (RA) patients, 30 obesity (OB) patients, and 23 HI. Antibody detection was achieved using four complementary assay platforms: indirect immunofluorescence, Western blotting, ELISA, and chemiluminescent immunoassay. RESULTS: We detected relatively low frequencies of anti-DFS70/LEDGFp75 antibodies in patients with DM (1.4%), RA (4.3%), and OB (6.6%), and elevated frequency (17.4%) in HI. A strong concordance between the different antibody detection platforms was observed. CONCLUSIONS: The low frequency of anti-DFS70/LEDGFp75 antibodies in Mexican patients with rheumatic diseases, but relatively higher frequency in HI, is consistent with previous observations with non-Hispanic populations, suggesting that geographic differences or ethnicity do not influence the frequency of these autoantibodies. Our results also highlight the importance of confirmatory assays for the accurate detection of these autoantibodies. Future studies with larger cohorts of healthy Hispanics/Latinos are needed to confirm if their anti-DFS70/LEDGFp75 antibody frequencies are significantly higher than in non-Hispanics.

Guías de práctica clínica para la atención del embarazo en mujeres con enfermedades reumáticas autoinmunes del Colegio Mexicano de Reumatología. Parte I / Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part I

Antecedentes. El embarazo en mujeres con enfermedades reumáticas autoinmunes se asocia a diversas complicaciones maternofetales. El desarrollo de guías de práctica clínica con la mejor evidencia científica disponible puede ayudar a homogeneizar la atención en estas pacientes. Objetivos. Proporcionar recomendaciones respecto al control prenatal, el tratamiento y el seguimiento más efectivo de la mujer embarazada con lupus eritematoso (LES), artritis reumatoide (AR) y síndrome por anticuerpos antifosfolípidos (SAF). Metodología. Para la elaboración de las recomendaciones se conformaron grupos nominales de expertos y se realizaron consensos formales, búsqueda sistematizada de la información, elaboración de preguntas clínicas, elaboración y calificación de las recomendaciones, fase de validación interna por pares y validación externa del documento final teniendo en cuenta los criterios de calidad del instrumento AGREE II. Resultados. Los grupos de trabajo contestaron las 37 preguntas relacionadas con la atención maternofetal en LES, AR y SAF, así como de fármacos antirreumáticos durante el embarazo y la lactancia. Las recomendaciones fueron discutidas e integradas en un manuscrito final y se elaboraron los algoritmos correspondientes. En esta primera parte se presentan las recomendaciones para mujeres embarazadas con LES. Conclusiones. La guía mexicana de práctica clínica para la atención del embarazo en mujeres con LES proporciona recomendaciones e integra la mejor evidencia disponible para el tratamiento y el seguimiento de estas pacientes (AU)

Background. Pregnancy in women with autoimmune rheumatic diseases is associated with several maternal and fetal complications. The development of clinical practice guidelines with the best available scientific evidence may help standardize the care of these patients. Objectives. To provide recommendations regarding prenatal care, treatment, and a more effective monitoring of pregnancy in women with lupus erythematosus (SLE), rheumatoid arthritis (RA) and antiphospholipid antibody syndrome (APS). Methodology. Nominal panels were formed for consensus, systematic search of information, development of clinical questions, processing and grading of recommendations, internal validation by peers, and external validation of the final document. The quality criteria of the AGREE II instrument were followed. Results. The various panels answered the 37 questions related to maternal and fetal care in SLE, RA, and APS, as well as to the use of antirheumatic drugs during pregnancy and lactation. The recommendations were discussed and integrated into a final manuscript. Finally, the corresponding algorithms were developed. We present the recommendations for pregnant women with SLE in this first part. Conclusions. We believe that the Mexican clinical practice guidelines for the management of pregnancy in women with SLE integrate the best available evidence for the treatment and follow-up of patients with these conditions (AU)

Guías de práctica clínica para la atención del embarazo en mujeres con enfermedades reumáticas autoinmunes del Colegio Mexicano de Reumatología. Parte II / Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part II

Antecedentes. El embarazo en mujeres con enfermedades reumáticas autoinmunes se asocia a diversas complicaciones materno-fetales. El desarrollo de guías de práctica clínica con la mejor evidencia científica disponible puede ayudar a homogeneizar la atención en estas pacientes. Objetivos. Proporcionar recomendaciones respecto al control prenatal, el tratamiento y el seguimiento más efectivo de la mujer embarazada con lupus eritematoso sistémico, artritis reumatoide (AR) y síndrome por anticuerpos antifosfolípidos (SAF). Metodología. Para la elaboración de las recomendaciones se conformaron grupos nominales de expertos y se realizaron consensos formales, búsqueda sistematizada de la información, elaboración de preguntas clínicas, elaboración y calificación de las recomendaciones, fase de validación interna por pares y validación externa del documento final teniendo en cuenta los criterios de calidad del instrumento AGREE II. Resultados. Los grupos de trabajo contestaron las 37 preguntas relacionadas con la atención materno-fetal en lupus eritematoso sistémico, AR y SAF, así como de fármacos antirreumáticos durante el embarazo y lactancia. Las recomendaciones fueron discutidas e integradas en un manuscrito final y se elaboraron los algoritmos correspondientes. En esta segunda parte se presentan las recomendaciones para mujeres embarazas con AR, SAF y el uso de fármacos antirreumáticos durante el embarazo y lactancia. Conclusiones. La guía mexicana de práctica clínica para la atención del embarazo en mujeres con AR y SAF integra la mejor evidencia disponible para el tratamiento y el seguimiento de estas pacientes (AU)

Background. Pregnancy in women with autoimmune rheumatic diseases is associated with several maternal and fetal complications. The development of clinical practice guidelines with the best available scientific evidence may help standardize the care of these patients. Objectives. To provide recommendations regarding prenatal care, treatment, and a more effective monitoring of pregnancy in women with lupus erythematosus, rheumatoid arthritis (RA) and antiphospholipid syndrome (APS). Methodology. Nominal panels were formed for consensus, systematic search of information, development of clinical questions, processing and staging of recommendations, internal validation by peers and external validation of the final document. The quality criteria of the AGREE II instrument were followed. Results. The panels answered 37 questions related to maternal and fetal care in lupus erythematosus, RA and APS, as well as for use of antirheumatic drugs during pregnancy and lactation. The recommendations were discussed and integrated into a final manuscript. Finally, the corresponding algorithms were developed. In this second part, the recommendations for pregnant women with RA, APS and the use of antirheumatic drugs during pregnancy and lactation are presented. Conclusions. We believe that the Mexican clinical practice guidelines for the management of pregnancy in women with RA and APS integrate the best available evidence for the treatment and follow-up of patients with these conditions (AU)

[Prevalence of metabolic syndrome and insulin resistance in system sclerosis]. / Prevalencia de síndrome metabólico y resistencia a la insulina en esclerosis sistémica.

BACKGROUND: The metabolic syndrome (MS) is a group of cardiovascular risk factors whose prevalence is increased in rheumatic diseases. The aim of this study was to estimate de prevalence of MS and insulin resistance (IR) in systemic sclerosis (SSc). METHODS: Fifty five patients with SSc were included. The World Health Organization criteria were used to define MS. Demographic, anthropometric and blood pressure data were recorded. Blood glucose, high density lipoprotein cholesterol (HDL-c), triglycerides and insulin were measured. Oral glucose tolerance curve was performed to identify impaired glucose tolerance and diabetes mellitus in those patients with normal fasting blood glucose. The HOMA index was calculated as well as a quantified proteinuria in urine of 24 hours. RESULTS: The prevalence of MS was 36.4 % (20 of 55 patients). Seventy percent of the patients with MS had limited SSc and 30 % diffuse SSc. An increased IR was observed in the limited SSc in comparison with the diffuse SSc (2.948 vs. 1.817 ± 0.3844 ± 0.2771, p = 0.03). An association between IR and MS was found in the limited SSc (p = 0.0001). Regarding the rest of the MS criteria, hypertriglyceridemia and an abnormal waist/hip ratio were the variables most often encountered, 95 % and 85 % respectively. Fifty percent of MS patients had low levels HDL-c and 40 % of them were hypertensive. None of the patients had proteinuria. CONCLUSION: The prevalence of MS in SSc was 36.4 %, similar to the found in other rheumatic diseases, but higher compared to the found in the Mexican population.

Introducción: el síndrome metabólico (SM) es un conjunto de factores de riesgo cardiovascular cuya prevalencia se encuentra incrementada en enfermedades reumáticas. El objetivo de este estudio fue estimar la prevalencia de SM y resistencia a la insulina (RI) en esclerosis sistémica (ES). Métodos: se incluyeron 55 pacientes con ES. Se evaluó SM utilizando los criterios de la OMS. Se registraron datos demográficos, antropométricos y presión arterial. Se midieron valores de glucosa, colesterol de lipoproteínas de alta densidad (c-HDL), triglicéridos e insulina. Se realizó una curva de tolerancia oral a la glucosa para identificar intolerancia a la glucosa y diabetes mellitus en pacientes con glucosa normal en ayuno. Se calculó el índice HOMA y se cuantificó proteinuria en orina de 24 horas. Resultados: la prevalencia del SM fue del 36.4 %. El 70 % de los pacientes con SM correspondió a ES limitada y 30 % a ES difusa. Se observó mayor resistencia a la insulina (RI) en la ES limitada frente a ES difusa. Se encontró asociación entre RI y el SM en la forma limitada. Del resto de los criterios de SM, la hipertrigliceridemia y el índice cintura/cadera anormal fueron las variables más frecuentes, 95 % y 85 % respectivamente. El 50 % de los pacientes con SM tuvo niveles bajos de c-HDL, e hipertensión arterial sistémica 40 %. En ningún paciente hubo proteinuria. Conclusiones: la prevalencia de SM en ES fue del 36.4 %, similar a la encontrada en otras enfermedades reumáticas, pero mayor en comparación con la población mexicana.

[Overweigth, obesity, metabolic syndrome and waist/height index in health staff]. / Sobrepeso, obesidad, síndrome metabólico e índice cintura/talla en el personal de salud.

INTRODUCTION: Health staff self-applied strategies are insufficiently to reduce cardiovascular risk factors. The aim of this article is to investigate the prevalence of overweight, obesity and metabolic syndrome and waist/height index on health staff. METHODS: This is a cross-sectional study from January to April 2014 in 735 workers of the UMAE Specialties La Raza. A diagnosis of overweight and obesity was established according to WHO criteria and metabolic syndrome NCEP ATP-III. In 250 participants lipid profile and blood chemistry were obtained. Descriptive statistics was applied, chi square, Student t test or Kruskal-Wallis to compare groups. RESULTS: We included 496 women and 239 men (physicians, nurses and other workers). 38% were overweight; 22% had obesity. We found fasting hyperglycemia>126 mg/dl in 5.1%, between 100-126 mg/dl in 17.4%, hypertriglyceridemia in 40.4%; HDL cholesterol<40 mg/dl 66% in men and HDL cholesterol<50 mg/dl 51.4% in women. Waist measurement was 93±11 cm in men vs. 88±13 m in women. The waist/height index in the ideal weight group was 0.048±0.04, for overweight 0.55±0.04 and for obesity 0.65±0.064 (p<0.01). Metabolic syndrome prevalence was 30.6%, smoking 21.6% and alcohol consumption 35%. CONCLUSIONS: The prevalence of obesity-overweight was 60% and metabolic syndrome 30%, making it necessary to take immediate actions to modify the lifestyle of health staff.

Introducción: el personal de salud se autoaplica insuficientes estrategias para reducir sus propios factores de riesgo cardiovascular. El objetivo fue investigar la prevalencia de sobrepeso, obesidad y síndrome metabólico en personal de salud.Métodos: diseño transversal de enero a abril de 2014, en 735 trabajadores de la UMAE Especialidades La Raza. El diagnóstico de sobrepeso y obesidad se estableció acorde a criterios de la OMS y síndrome metabólico NCEP ATP-III. En 250 participantes se realizó perfil de lípidos y química sanguínea. Se aplicó estadística descriptiva, chi cuadrada, t de Student o Kruskal-Wallis al comparar los grupos, el valor de significancia fue p < 0.05.Resultados: incluimos 496 mujeres y 239 hombres (entre médicos, enfermeras y otros). Tuvieron sobrepeso 38 %, y obesidad 22 %. Encontramos hiperglucemia de ayuno > 126 mg/dl 5.1 %, entre 100-126 mg/dl 17.4 %, hipertrigliceridemia 40.4 %; colesterol-HDL < 40 mg/dl en 66 % de hombres y colesterol-HDL < 50 mg/dl en 51.4 % de mujeres. La cintura 93 ± 11cm en hombres frente a 88 ± 13 cm en mujeres. El índice cintura/talla en el grupo de peso ideal fue 0.048 ± 0.04, en el de sobrepeso 0.55 ± 0.04 y en el de obesidad 0.65 ± 0.064. Tuvieron síndrome metabólico 30.6 %, tabaquismo 21.6 % y consumo de alcohol 35 %. Solo 2 % presentaron presión arterial > 135/85.Conclusiones: la prevalencia de obesidad y sobrepeso del personal de salud es de 60 % y de síndrome metabólico 30 %, por lo que es necesario tomar acciones inmediatas que modifiquen el estilo de vida del personal de salud.

[Carotid and brachial intima-media thickness of the allelic variants G894T and T-789C of eNOS in systemic sclerosis patients]. / Grosor íntima-media de las variantes alélicas G894T y T-789C de eNOS en pacientes con esclerosis sistémica.

OBJECTIVE: To evaluate comparatively the carotid intima media thickness index (IMT) and brachial index with Doppler ultrasound, and pulmonary artery pressure with echocardiography in allelic variation of G894T and T-789C eNOS (endothelial nitric oxide synthase) in patients with systemic sclerosis. METHODS: This is a cross-sectional study in patients with scleroderma. The inclusion criteria were: age 18-70 years, scleroderma diagnosed patients with a prior informed consent. Polymorphism G894T and T-789C of eNOS was measured in blood, and IMT by carotid and brachial Doppler. We analyzed with descriptive statistics, Student's t test or chi square for association of variables and Spearman to correlate Doppler parameters. RESULTS: We found abnormally high IMT in carotid and brachial arteries. We also observed low correlation between both brachial arteries, good correlation between carotids and no correlation between carotid and brachial arteries. The left IMT>0.65 mm in brachial artery corresponds to pulmonary pressure>30 mmHg by echocardiography. G894T gene polymorphism was associated with increased IMT in right carotid. CONCLUSION: The G894T eNOS polymorphism was associated with increased IMT in right carotid. The one side carotid IMT is consistent with its contralateral. The left brachial IMT above to 0.65 mm, suggests the possibility of pulmonary arterial hypertension.

Introducción: el objetivo de este estudio es evaluar comparativamente el grosor íntima-media (IMT) carotídeo y braquial con ultrasonido Doppler, la presión arterial pulmonar ecocardiográfica y la variación alélica de los genes G894T y T-789C de la eNOS en pacientes con esclerodermia. Métodos: estudio transversal en pacientes con esclerodermia. Criterios de inclusión: edad 18-70 años, diagnosticado con esclerodermia, previo consentimiento informado. Se evaluó IMT, índice de resistencia (IR) e índice de pulsatilidad (IP) mediante Doppler carotídeo y braquial, perfil de lípidos, proteína C reactiva, polimorfismo G894T y T-789C de óxido nítrico sin tasa endotelial (eNOS). Analizamos con estadística descriptiva, prueba t de Student o chi cuadrada para asociación de variables y Spearman para correlacionar las variables cuantitativas. Resultados: encontramos IMT anormalmente alto en arterias carotídeas y braquiales. Observamos baja correlación entre ambas braquiales, buena correlación entre carótidas y ninguna correlación entre carótidas y braquiales. El IMT braquial izquierdo > 0.65 corresponde con presión arteria pulmonar > 30 mmHg ecocardiográfica. El polimorfismo del gen G894T se asoció con mayor IMT en carótida derecha.Conclusión: El polimorfismo G894T de eNOS se asocia a mayor IMT en carótida derecha. El IMT carotídeo concuerda con su IMT contralateral. El IMT braquial izquierdo mayor a 0.65 sugiere la posibilidad de hipertensión arterial pulmonar.

[Alterations in cerebral perfusion in patients with systemic sclerosis and cognitive impairment]. / Alteraciones en la perfusión cerebral en pacientes con esclerosis sistémica y deterioro cognitivo.

INTRODUCTION: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis, immunological and vascular abnormalities. Cerebral hypoperfusion can be caused by cerebral ischemia. Cognitive impairment (CI) are a major cause of morbidity in SSc The aim of this study is to estimate the frequency of alterations in cerebral perfusion (CP) in SSc patients with CI. METHODS: We studied 88 patients with SSc. The Montreal Test (MT) was given to all patients to evaluate CI. To 15 patients with CI and without systemic hypertension, diabetes mellitus, cerebrovascular disease, vasculitis, hypothyroidism, depression, and drugs that interfere with the cognitive assessment, the PC was measured by cerebral gammagram (CG). RESULTS: Of the 88 patients with ES, 58 had CI by MT. A decrease in CP was observed in following lobes: frontal in 9 of 15 patients, temporal in 7 of 15, and parietal in 3 of 15. Concordance between MT and CG was 60% for the frontal, 46% for the temporal and parietal 13%. CONCLUSIONS: The CI is common in SSc. A decrease in CP was more frequent in the frontal lobe, predominating in older patients and with longer duration of SSc.

Introducción: la esclerosis sistémica (ES) es una enfermedad autoinmune, sistémica, caracterizada por fibrosis, alteraciones inmunológicas y vasculares. La hipoperfusión cerebral puede ser causada por isquemia. Los trastornos cognitivos son causa importante de morbilidad. El objetivo de este estudio fue determinar la frecuencia de alteraciones en la perfusión cerebral (PC) en pacientes con ES y deterioro cognitivo (DC). Métodos: se estudiaron 88 pacientes con ES. A todos se les aplicó el Test de Montreal (TM) para evaluar el DC. A 15 pacientes con DC que cumplieron con los criterios (sin hipertensión arterial sistémica, diabetes mellitus, evento vascular cerebral, vasculitis, hipotiroidismo, depresión, sin ingesta de fármacos que interfieran con la evaluación cognitiva), se les midió la PC mediante Gamagrama Cerebral Perfusorio (GCP).Resultados: de los 88 pacientes con ES, 58 tuvieron DC con el TM. La PC se encontró disminuida en 12/15. La disminución de la PC se observó en los siguientes lóbulos (frontal en 9/15; temporal en 7/15, y parietal en 3/15. La concordancia entre el TM y GC fue 60 % para el lóbulo frontal, 46 % para el lóbulo temporal y 13 % parietal.Conclusiones: el DC es frecuente en la ES, la disminución de la PC fue más común en el lóbulo frontal, predominado en los pacientes de mayor edad y tiempo de evolución de la ES.

[Fever of unknown origin, comparing two series with 26 years of difference]. / Fiebre de origen oscuro, comparación de dos series con 26 años de diferencia.

INTRODUCTION: Fever of unknown origin (FUO) remains a syndrome with difficult approach and changing spectrum. Our aim was to compare two series of FUO patients seen at the Department of Internal Medicine, Hospital de Especialidades Centro Medico La Raza, Mexico City. METHODS: Data from FUO series from 1979-87 were compared with those from 2004-14 series. We analyzed demographic data, final diagnoses, and diagnostic tests used. We report median and range for numerical variables and frequencies for nominal data, bivariate analysis was done with chi-square or Fisher´s test as needed using SPSS version 17.0 for MAC and open-epi version 3.7. RESULTS: One hundred twenty seven patients were included in the 1979-87 series and 118 in the 2004-14 series. There were more non-infectious inflammatory diseases (p=0.0004) and less infectious diseases (p=0.024) in the 2004-14 series. We observed no significant differences in neoplastic diseases and undiagnosed cases between the two series. Laboratory tests and their diagnostic utility were similar in both series, but image studies were less useful in the 2004-14 series. Biopsy and laparotomy remained as frequent and useful tools in both series. CONCLUSIONS: The recent series had more non-infectious inflammatory diseases and less infectious causes of FUO. Invasive studies remain as useful diagnostic aids in a significant number of cases.

Introducción: la fiebre de origen oscuro (FOO) es un síndrome de abordaje difícil y espectro cambiante. El objetivo fue comparar dos series de FOO estudiadas en el departamento de Medicina Interna del Hospital de Especialidades del Centro Médico La Raza.Métodos: fue un estudio comparativo de datos secundarios: los datos de la serie 1979-87, que fueron publicados por Frati et al. y los de la serie 2004-14, registrados en la base de datos de FOO del departamento de Medicina Interna. Se compararon datos demográficos, diagnósticos finales y pruebas diagnósticas utilizadas. Se describen mediana y amplitud para las variables numéricas y distribución de frecuencias para las variables nominales. El análisis bivariado se hizo con chi cuadrada o prueba de Fisher con los programas SPSS versión 17.0 para MAC y open epi versión 3.7.Resultados: se incluyeron 127 pacientes en la serie 1979-87, y 118 en la 2004-14. La serie más reciente tuvo menos casos ocasionados por infecciones, (p = 0.024), y más enfermedades inflamatorias no infecciosas (p = 0.0004). Se mantuvieron constantes las enfermedades neoplásicas y los casos con diagnóstico desconocido. Variaron poco las pruebas de laboratorio realizadas y su utilidad diagnóstica; en cambio los estudios de imagen fueron menos útiles en la serie 2004-14 (p = 0.00003). Las biopsias y la laparotomía fueron frecuentemente realizadas y útiles en ambas series.Conclusiones: En la última serie hubo más enfermedades inflamatorias no infecciosas a expensas de un menor número de infecciones. Los estudios invasivos siguen siendo necesarios.