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.

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.

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.

[Multicentric hyaline vascular Castleman's disease. A POEMS type variant]. / Enfermedad de Castleman multicéntrica hialina vascular. Una variante de POEMS.

BACKGROUND: Castleman's disease is an atypical lymphoproliferative disorder which may be compatible with paraneoplastic manifestations of POEMS syndrome. CLINICAL CASE: a 53 year old man with a history of type 2 diabetes, hypothyroidism and Addison's disease presented with numbness and weakness in limbs, dyspnea, skin hardening, Raynaud's phenomenon, weight loss and fatigue. A physical exam showed tachypnea, generalized cutaneous hyperpigmentation and skin hardening of extremities, muscle weakness, hypoesthesia and hyporeflexia. Laboratory showed hyperprolactinemia, low testosterone, hypothyroidism and Addison's disease. Electrophoresis of proteins showed polyclonal hypergammaglobulinemia. Somatosensory evoked potentials reported peripheral neuropathy and severe axonal polyneuropathy by electromyography. Chest X-rays showed bilateral reticular infiltrates and mediastinal widening. An echocardiogram displayed moderate pulmonary hypertension. Skin biopsy had no evidence of scleroderma. CT reported axillar, mediastinal and retroperitoneal nodes. The mediastinal lesion biopsy reported hyaline vascular Castleman's disease, multicentric variety. He was treated with rituximab. CONCLUSIONS: the case meet criteria for multicentric hyaline vascular Castleman's disease, POEMS variant, treated with rituximab.

Introducción: la enfermedad de Castleman es un trastorno linfoproliferativo atípico en el que pueden existir manifestaciones compatibles con síndrome POEMS. Caso clínico: hombre de 53 años de edad con antecedente de diabetes mellitus tipo 2, hipotiroidismo y enfermedad de Addison. Se iniciaron parestesias y debilidad en las extremidades y, posteriormente, disnea, endurecimiento cutáneo, fenómeno de Raynaud y pérdida de peso. Se identificó taquipnea, hiperpigmentación cutánea generalizada y extremidades con endurecimiento cutáneo, debilidad muscular, hipoestesia e hiporreflexia; así como hiperprolactinemia, testosterona baja, hipotiroidismo y enfermedad de Addison; los anticuerpos antinucleares y antiScl-70 fueron negativos. Los potenciales evocados somatosensoriales indicaron neuropatía periférica y la electromiografía, olineuropatía axonal severa. Radiografía torácica: infiltrado reticular bilateral y ensanchamiento mediastinal. Electrocardiograma: hipertensión arterial pulmonar moderada. Tomografía toracoabdominal: ganglios axilares, mediastinales y retroperitoneales. Con la biopsia se identificó enfermedad de Castleman multicéntrica hialina vascular. El paciente recibió rituximab. Conclusiones: si bien la experiencia con el rituximab aún es limitada, en el caso descrito se observó buena respuesta.

[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.

[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.

[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.

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.

[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.

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.

[Clinical, endoscopic and manometric findings in the gastroesophageal tract of patients with systemic sclerosis]. / Manifestaciones de esclerosis sistémica en el aparato digestivo. Clínicas, endoscópicas y por manometría.

BACKGROUND: In the systemic sclerosis (SSc) gastrointestinal manifestations are present up to 90 % and the esophagus is the most affected organ. The purpose was to investigate clinical, endoscopic and manometric upper gastroesophageal manifestations in patients with SSc. METHODS: We studied 60 patients with SSc, mean age of 48 +/- 11 years and a mean disease evolution of 11 +/- 8 years. An endoscopy of gastro-esophageal tract and esophageal manometry were performed. RESULTS: The clinical manifestations were: dysphagia 80 %, pirosis and regurgitations 78 %. Endoscopic findings were: esophagitis 60 %, hiatal hernia 65 %, loose hiatus 15 % and Barrett's esophagus 18 %. Manometric findings were hypotensive lower esophageal sphincter (LES) 95 %. Aperistalsis (41 %), slight hypomotility (30 %), severe hypomotility (27 %) were found. Gastric manifestations were: early satiety 45 %, abdominal pain 35 %, nausea 20 % and vomiting 10 %, and by endoscopy we observed: Gastric disturbances in 80 %, of these 40 % corresponds to non erosive gastropathy, 30 % to erosive gastropathy and 10 % to nodular gastropathy. CONCLUSIONS: The most frequents endoscopic finding were esophagitis and hiatal hernia, and by manometry were aperistalsis and hypotensive LES. These alterations imply disorders of motility and dysfunction of LES. Barrett's esophagus was always associated with hiatal hernia.

[Systemic sclerosis and hypothyroidism]. / Prevalencia de hipotiroidismo y anticuerpos antitiroglobulina en pacientes mexicanos con esclerosis sistémica.

OBJECTIVE: Assess the prevalence of hypothyroidism and anti-thyroglobuline antibodies (AbATg) among Mexican patients with systemic sclerosis (SSc). MATERIAL AND METHODS: Thyroid function was studied in SSs patients and controls. Triiodothyronine, thyroxine, thyroid stimulant hormone (TSH) and AbATg were measured. RESULTS: 110 SSc patients (106 women and 4 men), mean age 48.1 +/- 28.5 yrs versus 80 healthy controls (76 women and 4 men) with mean age 47.5 +/- 28.8 yrs were included. Hypothyroidism was diagnosed in 19% patients compared with 1.3% in the control group. The following results were found; triyodotironina: 82 ng/dl versus 160 ng/dl, p < 0.000; tiroxina: 5.4 ng/dl versus 7ng/dl, p < 0.01; TSH: 8.2 uUl/ mL versus 1.1 +/- 2 uUl/mL, p < 0.000. Subclinical hypothyroidism was observed in 35% patients versus 0% controls, TSH: 7.2 uUl/ml versus 1.2 +/- 1.4 uUl/ml, p < 0. 000; triyodotironina: 116 ng/dl compared with 160 ng/dl, p = ns; tiroxina: 7.0 ng/dl vs. 7.0 ng/dl, p ns; AbATg were positive in patients 54% and 2.5%, p < 0.001 in the control group. CONCLUSIONS: Our study reports a high prevalence of hypothyroidism among SSc Mexican patients, especially of the subclinical type. We need to consider hypothyroidism as a clinical entity often found among SSc patients, and start hormone replacment treatment accordingly.

[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.

[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.

[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.

[Clinical and angiographic manifestations inpatients without previous diagnosis of Takayasu´s arteritis]. / Manifestaciones clínicas y angiográficas en pacientes sin diagnóstico previo de arteritis de Takayasu.

INTRODUCTION: Takayasu's arteritis is a systemic vasculitis that affects the aorta and its main branches mainly seen in young women and it is characterized by decrease or absence of pulses. The arteriography is necessary to corroborate the diagnosis. The aim of this article is to analyze the clinical manifestations and radiological findings in patients with no previous diagnosis of TA. METHODS: The patients were sent to the Department of Radiodiagnosis and Imaging for the performance of an angiography for cerebrovascular disease (CVD) in young patients, renovascular hypertension (RVH) and arterial insufficiency (IA) of limbs. Clinical manifestations were used to investigate the diagnosis of AT according to the criteria of the American College of Rheumatology 1990. A digital subtraction angiography (DSA) was performed and the type of AT was evaluated according to Numano classification. RESULTS: There were 10 women with an average age of 32.4±10 years. The reasons for the study of these patients were stroke in young patients 4, RVH 3 and AI of upper extremities 3 patients. The main types of TA were I: 50%, V: 40%, IV 10%. The main arterial involvement were the supraaortic trunk and both carotids 80%, right subclavian 80%, left subclavian 70%, left vertebral 40%, right vertebral 30%, thoracic aortic 40%, abdominal aortic 40%, right renal 40% and left renal 10%. CONCLUSIONS: The most frequent clinical manifestations were stroke in young patients, RVH and AI. The AT type I was the most common, followed by V and IV respectively, which explains the clinical manifestations.

Introducción: la arteritis de Takayasu (AT) es una vasculitis sistémica que afecta a la aorta y sus ramas principales, se distingue por disminución o ausencia de pulsos. La arteriografía corrobora el diagnóstico de AT. El objetivo de este estudio es analizar las manifestaciones clínicas y hallazgos arteriográficos en pacientes sin diagnóstico previo de AT.Métodos: se estudiaron 10 pacientes enviadas al Departamento de Radiodiagnóstico para la realización de panangiografía por enfermedad cerebrovascular (EVC) en pacientes jóvenes, hipertensión renovascular (HRV) e insuficiencia arterial (IA) de alguna extremidad. Se investigaron manifestaciones clínicas orientadas al diagnóstico de AT y se realizó arteriografía con sustracción digital y ultrasonido Doppler color. A las pacientes se les realizó el diagnóstico de AT y por arteriografía se usó la clasificación de Numano.Resultados: la EVC en paciente joven se encontró en 4 casos, HRV en 3, e IA de las extremidades superiores en 3. Por arteriografía los tipos de AT fueron I: 50 %, V: 40 % y IV 10 %. La afección principal se demostró en los tronco supraaórticos y de estos las carótidas 80 %, subclavia derecha 80 %, subclavia izquierda 70 %, vertebral izquierda 40 %, vertebral derecha 30 %, aorta abdominal 40 %, renal derecha 40 % y renal izquierda 10 %.Conclusión: las manifestaciones clínicas más comunes fueron: EVC, HRV e IA de las extremidades superior. La AT tipo I fue la más frecuente y explica las manifestaciones de EVC, seguida de la tipo V.

[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.

[Effect of pravastatine plus ezetimibe on carotid intima media thickness in patients with lupus erythematosus]. / Efecto de pravastatina más ezetimibe en el grosor intima-media carotídea en pacientes con lupus eritematoso.

BACKGROUND: Patients with systemic lupus erythematosus (SLE) have accelerated atherosclerosis that can be assessed by the carotid intima media thickness (CIMT) measurement. A prompt hypolipidemic treatment should be a part of the integral management. The aim of this study is to determine the effect of therapy with pravastatin plus ezetimibe on the CIMT in SLE patients. METHODS: Longitudinal, prospective, quasi-experimental trial. Out of 60 SLE patients in whom a carotid ultrasound was performed, we chose 22 with a CIMT>0.7 mm who were administered pravastatin plus ezetimibe during 6 months with determination of CIMT at the end of the study. We performed the following tests: total cholesterol (TC), HDL-cholesterol, LDL-cholesterol, tryglicerides, C-reactive protein (CRP), liver function, muscle enzimes and glucose, basal and at the end of treatment. STATISTICAL ANALYSIS: descriptive statistics and Wilcoxon test were used. RESULTS: There were 22 women with an age of 42±6.3 years, average disease evolution 7.5±6.6 years, of whom, 18 concluded the study. Right basal CIMT was 0.829±0.1448 vs. final 0.688±0.1453, p<0.003; left CIMT was 0.820±0.1312 vs. 0.724±0.1348, p<0.004. TC 208 mg/dl vs 168 mg/dl, LDL-C 125 mg/dl vs. 72 mg/dl, p=0.0004. CRP 3.12 vs. 2.25 p=0.004. In 2 cases there were gastrointestinal, skin and muscle adverse effects. CONCLUSIONS: Treatment with pravastatin plus ezetimibe decreases the CIMT with improvement in the concentration of total cholesterol, LDL-C and CRP levels with good toleration.

Introducción: Los pacientes con lupus eritematoso sistémico (LES) cursan con ateroesclerosis acelerada que puede ser evaluada mediante el grosor íntima-media carotídea (IMC). El uso de hipolipemiantes debe ser parte de su tratamiento. El objetivo de este estudio fue determinar el efecto de la terapia con pravastatina más ezetimibe en el grosor IMC en pacientes con LES.Métodos: estudio prospectivo, longitudinal, cuasi-experimental. De 60 paciente con LES a quienes se le realizó ultrasonido carotídeo, de los cuales se eligieron a 22 con grosor IMC > 0.7 mm y se les administró pravastatina más ezetimibe durante seis meses y posteriormente se determinó el grosor IMC. Se les determinó colesterol total (CT), colesterol HDL (c-HDL), colesterol LDL (c-LDL), triglicéridos, proteína C-reactiva (PCR), pruebas de funcionamiento hepático, enzimas musculares y glucosa basales y posterior al tratamiento. Análisis estadístico: estadística descriptiva y prueba de Wilcoxon.Resultados: se incluyeron 22 mujeres con edad 42 ± 6.3 años y evolución promedio 7.5± 6.6 años, de las cuales concluyeron el estudio 18 pacientes. El grosor IMC derecha basal fue de 0.829 ± 0.1448 frente a final 0.688 ± 0.1453, p < 0.003; el izquierdo 0.820±0.1312 frente a 0.724±0.1348, p < 0.004. El CT 208 mg/dl frente a 168 mg/dl, y el c-LDL 125 mg/dl frente a 72 mg/dl, p = 0.0004. Niveles de PCR 3.12 frente a 2.25 p = 0.004.Conclusiones: el tratamiento con pravastatina más ezetimibe disminuye el grosor IMC con mejoría en las concentraciones de colesterol total, colesterol LDL y niveles de PCR, así como buena tolerancia.