[Recurrent chronic Vogt-Koyanagi-Harada syndrome or sarcoidosis? A Gloomy diagnosis]. / ¿Síndrome de Vogt-Koyanagi-Harada crónico o sarcoidosis? Un diagnóstico sombrío.

Background: Vogt-Koyanagi-Harada syndrome (VKH) is a systemic disease that affects organs profuse in melanocytes, presenting with a chronic and diffuse bilateral granulomatous panuveitis, as well as neurological, auditory, and cutaneous manifestations. In this article, a systematic approach is presented for the diagnostic management of VKH syndrome, considering relevant diagnostic possibilities to rule out other entities that manifest similar symptoms. Clinical case: 71-year-old man with a long-standing history of vitiligo, who experienced visual loss in his right eye 6 months before his admission, along with bilateral hearing loss predominantly in the right ear. During his hospitalization, he presented with chronic headache, fever, and significant involuntary weight loss. Ophthalmological examination revealed that his right eye only perceived light and had hyperemic bulbar conjunctiva, while the left eye had a visual acuity of 20/200. The right fundus had scattered pigmentation, while the left had an edematous optic disc and right optic atrophy. Conclusions: The diagnosis of VKH syndrome is established by the presence of the 5 diagnostic criteria for complete disease, including retinal depigmentation, neurological alterations, and dermatological manifestations. Since patients can present with a wide variety of symptoms, initial differential diagnosis should be considered, which represents a diagnostic challenge.

Introducción: el síndrome de Vogt-Koyanagi-Harada (VKH) es una enfermedad sistémica que afecta a órganos ricos en melanocitos y se manifiesta con una panuveítis granulomatosa bilateral, crónica y difusa, así como con manifestaciones neurológicas, auditivas y cutáneas. En este artículo se presenta un enfoque sistemático para el abordaje diagnóstico del síndrome de VKH y se consideran las posibilidades diagnósticas relevantes para descartar otras entidades que se presentan con síntomas similares. Caso clínico: hombre de 71 años con antecedentes de vitiligo de larga data, quien experimentó una pérdida visual en su ojo derecho seis meses antes de su ingreso, junto con hipoacusia bilateral, predominantemente en el oído derecho. Durante su hospitalización, presentó cefalea crónica, fiebre y una significativa pérdida involuntaria de peso. En la exploración oftalmológica, el ojo derecho solo percibía luz y presentaba conjuntiva bulbar hiperémica, mientras que el ojo izquierdo tenía una agudeza visual de 20/200. El fondo del ojo derecho presentaba pigmentación dispersa, mientras que el izquierdo tenía una papila edematosa y atrofia óptica derecha. Conclusiones: el diagnóstico del síndrome de VKH se establece mediante la presencia de los 5 criterios diagnósticos para la enfermedad completa, incluida la hipopigmentación retiniana, las alteraciones neurológicas y las manifestaciones dermatológicas. Dado que los pacientes pueden presentar una amplia variedad de síntomas, el diagnóstico diferencial debe considerarse inicialmente, lo que representa un desafío diagnóstico.

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.

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome.

The Sweet's syndrome, is an inflammatory skin disorder characterized by extensive infiltration of neutrophils in the dermis with extension to the subcutis, known as acute febrile neutrophilic dermatosis. It may occur as a paraneoplastic syndrome. To our knowledge, there are currently few reports about transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing Sweet syndrome in the literature. Herein we describe an unusual case in a young patient with these characteristics that evolved to a fatal outcome.

[Intestinal endometriosis: a diagnostic challenge for the internist?] / Endometriosis intestinal: ¿un reto diagnóstico para el médico internista?

Background: Within the wide variety of clinical skills distinctive of the internist the diagnostic approach of abdominal pain is paramount in everyday clinical practice. It is well known that no physician can diagnose what they don't know: classically considered as one of the ''great simulators'', intestinal endometriosis is a rare yet potentially fatal cause of abdominal pain if misdiagnosed, thus requiring a comprehensive medical evaluation. Case report: We present the case of a 33-year-old woman evaluated for a bowel obstruction, in the first instance associated with a probable abdominal tumor, subsequently concluding the definitive diagnosis of intestinal endometriosis. Conclusions: Although endometriosis is a frequent pathology, the location and clinical presentation presented in this case is not. However, the lack of information on this, like any other pathology, can delay the diagnosis or carry the risk of offering inappropriate treatments for an incorrect diagnosis. This is the importance of its knowledge and dissemination among first-contact doctors as well as clinical and surgical specialists.

Introducción: Dentro del gran abanico de competencias características del médico clínico se encuentra el abordaje diagnóstico del síndrome doloroso abdominal. Es bien sabido que el médico no diagnostica lo que no conoce. Considerada como una gran simuladora en la patología abdominal, la endometriosis intestinal es una causa de dolor abdominal poco frecuente, pero potencialmente mortal, siendo necesario un abordaje diagnóstico detallado. Caso clínico: Presentamos el caso de una mujer de 33 años evaluada por un cuadro de obstrucción intestinal, en primera instancia asociada a un probable tumor abdominal, concluyendo posteriormente el diagnóstico definitivo de endometriosis intestinal. Conclusiones: Si bien la endometriosis es una patología frecuente, la localización y la presentación clínica de este caso no lo son. Sin embargo, la falta de información de esta, al igual que de cualquier otra patología, puede retrasar el diagnóstico o conllevar el riesgo de ofrecer tratamientos no adecuados por un diagnóstico incorrecto. He aquí la importancia de su conocimiento y difusión entre médicos de primer contacto, así como entre especialistas clínicos y quirúrgicos.

Parry-Romberg, a neurocutaneous syndrome: presentation of three cases. / Parry-Romberg, un síndrome neurocutáneo: presentación de tres casos.

BACKGROUND: Parry-Romberg syndrome is characterized by progressive hemiatrophy of the skin, subcutaneous tissue, muscle and bones of the skull. Its incidence is low, with a progressive and slow course. Its etiology is unknown, but it has been associated with several factors. Its clinical presentation involves dermatological, musculoskeletal and neuropsychiatric manifestations. The treatment consists of medical and surgical strategies. The use of steroids, alone or in combination with immunomodulators, has the objective of slowing down progression. The surgical treatment lies in facial reconstruction or volumetric regeneration, to correct the appearance and function of facial structures. The objective is to show three cases of Parry-Romberg syndrome with the representative characteristics of the disease. CLINICAL CASEs: 1) A 41-year-old woman with atrophy and right supraciliary hypochromia associated with seizures and headache. 2) A 43-year-old woman with parietal deformation and right supraciliary atrophy, associated with facial paralysis, depression and headache. 3) A 36-year-old woman with right hemifacial atrophy associated with ocular involvement and headache. The indicated treatment was based on a systemic steroid plus a cytostatic agent. The surgical treatment was evaluated according to the affection of facial structures. CONCLUSIONS: Parry-Romberg syndrome is a rare disease, characterized by progressive atrophy. Clinical manifestations determine the diagnosis and early start of medical and surgical treatment.

INTRODUCCIÓN: El síndrome de Parry-Romberg se caracteriza por hemiatrofia progresiva de la piel, el tejido subcutáneo, los músculos y los huesos del cráneo. Su incidencia es baja, y su curso es progresivo y lento. Se desconoce su etiología, pero se ha asociado a diversos factores. Su presentación clínica involucra manifestaciones dermatológicas, musculoesqueléticas y neuropsiquiátricas. El tratamiento consiste en estrategias médicas y quirúrgicas. El uso de esteroides, solos o en combinación con inmunomoduladores, tiene el objetivo de frenar la progresión. El tratamiento quirúrgico consiste en reconstrucción facial o regeneración volumétrica, a fin de corregir la apariencia y la función de las estructuras faciales. Se presentan tres casos de síndrome de Parry-Romberg con las características representativas de la enfermedad. CASOS CLÍNICOS: 1) Mujer de 41 años, con atrofia e hipocromía supraciliar derecha, asociadas a crisis convulsivas y cefalea. 2) Mujer de 43 años, con deformación parietal y atrofia supraciliar derecha, asociadas a parálisis facial, depresión y cefalea. 3) Mujer de 36 años, con atrofia hemifacial derecha asociada a afección ocular y cefalea. El tratamiento se basó en esteroide sistémico más un agente citostático. El tratamiento quirúrgico se valoró de acuerdo con la afectación de la estructuras faciales. CONCLUSIONES: El síndrome de Parry-Romberg es una enfermedad rara caracterizada por una atrofia progresiva. Las manifestaciones clínicas determinan el diagnóstico y el inicio temprano del tratamiento médico y quirúrgico.

Plasmablastic lymphoma: case report.

INTRODUCCIÓN: El linfoma plasmablástico es un linfoma no Hodgkin de alto grado. Se describe en pacientes con infección por el virus de la inmunodeficiencia humana (VIH), en receptores de trasplantes y en pacientes de edad avanzada. Se presenta en la cuarta década de la vida, siendo la cavidad oral el sitio más común de afección. Sus células neoplásicas expresan marcadores de células plasmáticas como CD138 y CD38. En su patogenia se ha implicado al virus de Epstein-Barr. La consideración más desafiante en el diagnóstico diferencial es el mieloma múltiple, ya que las características morfológicas e inmunofenotípicas de estas dos enfermedades son muy similares. El régimen EPOCH (etopósido, epirubicina, vincristina, ciclofosfamida y prednisona) asociado a tratamiento antirretroviral es la opción más eficaz, mejorando la supervivencia a 17 meses. CASO CLÍNICO: Varón de 35 años con VIH y síntomas de disfunción orgánica asociada a mieloma. Se realizó electroforesis de proteínas y aspirado de médula ósea, descartándose mieloma múltiple. Continuando con el abordaje diagnóstico y con la sospecha de neoplasia ósea, se realizó una biopsia ósea que reportó linfoma plasmablástico CD138+ y Ki-67 80%, Epstein-Barr positivo. El paciente recibió quimioterapia con EPOCH y tuvo una sobrevida de 6 meses. CONCLUSIONES: La presencia de disfunción orgánica relacionada con mieloma motivó la búsqueda de dicha patología y se determinó la presencia de linfoma plasmablástico. La correlación clínica, bioquímica, inmunohistoquímica y radiográfica es esencial para el correcto diagnóstico. BACKGROUND: Plasmablastic lymphoma is a high grade non-Hodgkin lymphoma. It is described in patients with HIV infection, post-transplant and advanced age. It appears in the fourth decade of life and the oral cavity is the most common site of affection. Its neoplastic cells express markers of plasma cells such as CD138, CD38. In its pathogenesis the Epstein-Barr virus has been implicated. The most challenging consideration in the differential diagnosis is with multiple myeloma, since the morphological and immunophenotypic characteristics of these two entities are very similar. Treatment with EPOCH (etoposide, epirubicin, vincristine, cyclophosphamide and prednisone) associated with antiretroviral treatment is the most effective option, improving survival to 17 months. CASE REPORT: Male of 35 years with HIV and symptoms of organic dysfunction associated with myeloma. Protein electrophoresis and bone marrow aspiration were performed, ruling out multiple myeloma. Continuing with the diagnostic approach and with suspicion of bone neoplasia, a bone biopsy was performed with report of plasmablastic lymphoma CD 138+ and Ki-67 80%, Epstein-Barr positive. He received chemotherapy with EPOCH, with a six-month survival. CONCLUSIONS: The presence of organic dysfunction related to myeloma motivated the search for said pathology and the presence of plasmablastic lymphoma was determined; clinical, biochemical, immunohistochemical, and radiographic correlation are essential for correct diagnosis.

Still's disease: a rare condition, in a patient of unusual age.

INTRODUCCIÓN: Cuando inicia en el adulto, la enfermedad de Still es de mayor prevalencia en caucásicos y entre los 16 y 35 años. De etiología desconocida, se asocia a HLA-II, DR2, 4 y 7, y Bw35. El comienzo de los síntomas es agudo, con fiebre en agujas, asociada a exantema en las extremidades y el tronco, maculopapular, eritematoso y evanescente, pruriginoso, con fenómeno de Koebner. Presenta artralgias y artritis con un patrón poliarticular, simétrico y migratorio, mialgias y adenopatías. El 90% de los pacientes presentan anemia, leucocitosis con neutrofilia y trombocitosis asociada a la actividad, así como elevación de transaminasas y ferritina > 2000. El factor reumatoide y los anticuerpos antinucleares son negativos. El objetivo del presente trabajo es presentar un caso de enfermedad de Still que se sale del patrón habitual de manifestación. CASO CLÍNICO: Mujer de 56 años, inicia con exantema -maculopapular, eritematoso, pruriginoso, en zona periorbitaria -bilateral, tórax anterior, región glútea bilateral y zonas de extensión de codos y rodillas, que respetan el abdomen. Fiebre vespertina de 39 °C, con artralgias de codos, muñecas y rodillas, y mialgias, con faringe hiperémica. Después de descartar procesos infecciosos, neoplásicos y autoinmunitarios, y de acuerdo con los criterios de Yamaguchi y Fautrel, se diagnosticó enfermedad de Still. CONCLUSIONES: Este caso se presenta por la baja prevalencia de la enfermedad de Still y porque en el grupo etario de nuestra paciente no es habitual su presentación. Los -antecedentes familiares y el cuadro clínico sugestivo obligaron a descartar la presencia de otros procesos mórbidos, toda vez que el diagnóstico de enfermedad de Still es de exclusión. BACKGROUND: Adult-onset on Still's disease is common in Caucasians, between 16 and 35 years. Its cause is unknown, but it is associated with HLA-II, DR2, 4, 7 and Bw35. First symptoms are acute; fever in needles associated with exanthema in extremities and maculopapular trunk, erythematous and evanescent, pruritic with Koebner phenomenon. Patients present arthralgias and arthritis with a polyarticular, symmetrical and migratory pattern; myalgias and adenopathies. 90% of patients have anemia, leukocytosis with neutrophilia and thrombocytosis associated with the activity. Elevation of transaminases and ferritin greater than 2000. The rheumatoid factor and antinuclear antibodies are negative. The aim of this article is to present a case report of Still's Disease whose pattern of appearance is uncommon. CASE REPORT: A 56-year-old woman presented papular macular, erythematous, pruritic exanthema, in the bilateral peri-orbital area, anterior thorax, bilateral gluteal region, and elbow and knee extensions, while respecting abdomen. In addition, evening fever of 39 °C with arthralgias in elbows, wrists, and knees, myalgias, and hyperemic pharynx were manifested. According to the criteria of Yamaguchi and Fautrel, and after ruling out infectious, neoplastic, autoimmune processes, Still's disease was concluded. CONCLUSIONS: This case is presented due to the low prevalence of Still's disease and its presentation is not usual in the age group of our patient. The family history and very ­indicative clinical pictures forced us to rule out the presence of other morbid processes, while reinforcing the diagnosis of Still's disease, since it is by exclusion.

Acquired hemophilia: clinical case of hemorrhage caused by inhibitors against factor VIII. / Hemofilia adquirida: caso clínico de hemorragia por inhibidores contra el factor VIII.

BACKGROUND: Acquired hemophilia is caused by antibodies against coagulation factors, especially against factor VIII. As it is a low prevalence disease, our objective is to present an idiopathic case of acquired hemophilia to facilitate decision-making in clinical practice for others, exposing the therapeutic modality used. CASE REPORT: 65 years old woman, started with non-trauma hematomas, with asthenia, adynamia and dyspnea. Laboratory studies showed anemia (hemoglobin 7.1 g/dl, hematocrit 21%, mean corpuscular volume 91 fl, mean corpuscular hemoglobin 29.6 pg) and long clotting times (activated partial thromboplastin time 100 seconds) and with prothrombin time and International Normalized Index (17 seconds and 1.2 seconds respectively). So, plasma dilution tests were performed with plasma, without aPTT correction (1:2, 76.6 seconds; 1: 4, 60 seconds; 1: 8, 45 seconds) evidencing the presence of the inhibitor. The diagnosis was confirmed by the decrease in factor VIII (1%) and the presence of the factor VIII inhibitor (256 UB). The treatment started with prednisone and cyclophosphamide. Due to the presence of severe bleeding, treatment with activated prothrombin complex concentrate (CCpa) was required. Subsequently, Rituximab was prescribed, with clinical improvement and increased levels of hgb and reduced aPTT from the first week of treatment. CONCLUSIONS: The approach of acquired hemophilia should include control of the bleeding, the search for the etiology ruling out of the main causes of malignant neoplasms according to age and gender, and the immediate initiation of immunosuppression to decrease inhibitor levels.

INTRODUCCIÓN: La hemofilia adquirida es ocasionada por anticuerpos contra los factores de la coagulación, en especial contra el factor VIII. Por ser de baja prevalencia, presentamos un caso de hemofilia adquirida idiopática exponiendo la modalidad terapéutica utilizada para facilitar a otros la toma de decisiones en la práctica clínica. CASO CLÍNICO: Mujer de 65 años que inició con hematomas no asociados a traumatismo, con astenia, adinamia y disnea. Los estudios de laboratorio mostraron anemia (hemoglobina 7.1 g/dl, hematocrito 21%, volumen corpuscular medio 91 fl, hemoglobina corpuscular media 29.6 pg) y tiempos de coagulación prolongados (tiempo de tromboplastina parcial activado [TTPa] 100 segundos), con tiempo de protrombina e INR (International Normalized Ratio) dentro de parámetros normales (17 y 1.2 segundos, respectivamente), por lo que se realizaron pruebas de dilución con plasma sin corrección del TTPa (1:2, 76.6 segundos; 1:4, 60 segundos; 1:8, 45 segundos), evidenciando la presencia del inhibidor. El diagnóstico se confirmó con la disminución del factor VIII (1%) y la presencia de inhibidor del factor VIII (256 UB). Se inició tratamiento con prednisona y ciclofosfamida. Por presentar hemorragia grave requirió tratamiento con concentrado de complejo de protrombina activado y posteriormente se indicó rituximab, con mejoría clínica, incremento en los niveles de hemoglobina y reducción del TTPa desde la primera semana de tratamiento. CONCLUSIONES: El abordaje de la hemofilia adquirida debe incluir el control de la hemorragia, la búsqueda de la etiología descartando las principales causas de neoplasias malignas de acuerdo con la edad y el sexo, y el inicio inmediato de inmunosupresión para disminuir los niveles de inhibidor.

[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.

[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.

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis.

The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid, pleocytosis and proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients. After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05). Neurological manifestations and neuroimaging findings were similar in both groups of vasculitides, but general symptoms, joint, musculoskeletal, and peripheral neuropathy were preponderant in SCNSV. After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.

[Wegener's granulomatosis: diagnosis and treatment]. / Granulomatosis de Wegener, abordaje diagnóstico y terapéutico.

Wegener's granulomatosis (WG) is a systemic, necrotizing and granulomatous vasculitis that affects the upper and lower respiratory tract and the kidney. It is the most common antineutrophil cytoplasmic antibodies (ANCA) vasculitis. The diagnosis of WG is based on clinical manifestations, histological findings and the presence of ANCA in serum. The American College of Rheumatology criteria includes: oral and nasal inflammation, abnormal chest radiography (nodules, fixed infiltrates, or cavities, urinary sediment (hematuria), and granulomatous inflammation on biopsy. Treatment of WG is divided into 2 phases, induction of remission (IR), followed by a maintenance phase (MP). In the IR it is necessary to use immunosuppressive intensive therapy (cyclophosphamide plus steroids) and the MP is a less intensive therapy in which immunosuppressors (IS) such as azathioprine, methotrexate, mycophenolate of mofetil, among others, may be employed. Their purpose is to keep remission and lower the adverse effects associated with IS. Etanercept has not been proven successfully for the MP. Rituximab and 15-dexopergualin constitute promising the rapies for refractory WG.

Granulomatosis de Wegener, abordaje diagnóstico y terapéutico / Wegener's granulomatosis: diagnosis and treatment

La granulomatosis de Wegener es una vasculitis sistémica, necrosante y granulomatosa que afecta el tracto respiratorio superior e inferior y el riñón; es la vasculitis anticuerpos anticitoplasma del neutrófilo (ANCA) positiva más frecuente. El diagnóstico se basa en las manifestaciones clínicas, la biopsia de los órganos afectados y la presencia de ANCA en el suero. Los criterios de clasificación según el Colegio Americano de Reumatología son inflamación oral o nasal, alteraciones en la radiografía de tórax (nódulos, infiltrado pulmonar fijo, cavitaciones), anormalidades urinarias (hematuria), biopsia con infiltrado granulomatoso. El tratamiento se divide en dos fases: de inducción a la remisión y de mantenimiento. En la primera es necesario el uso de terapia inmunosupresora intensiva: ciclofosfamida más esteroides para controlar la actividad de la enfermedad (tres a seis meses). En la fase de mantenimiento, menos intensa, se emplean inmunosupresores como azatioprina, metotrexate, micofenolato de mofetilo, entre otros; su objetivo es mantener la remisión y disminuir los efectos adversos asociados a la ciclofosfamida. El etanercept no ha sido útil para la fase de mantenimiento. El rituximab y la 15-desoxipergualina son terapias alternativas en casos refractarios.

Wegener's granulomatosis (WG) is a systemic, necrotizing and granulomatous vasculitis that affects the upper and lower respiratory tract and the kidney. It is the most common antineutrophil cytoplasmic antibodies (ANCA) vasculitis. The diagnosis of WG is based on clinical manifestations, histological findings and the presence of ANCA in serum. The American College of Rheumatology criteria includes: oral and nasal inflammation, abnormal chest radiography (nodules, fixed infiltrates, or cavities, urinary sediment (hematuria), and granulomatous inflammation on biopsy. Treatment of WG is divided into 2 phases, induction of remission (IR), followed by a maintenance phase (MP). In the IR it is necessary to use immunosuppressive intensive therapy (cyclophosphamide plus steroids) and the MP is a less intensive therapy in which immunosuppressors (IS) such as azathioprine, methotrexate, mycophenolate of mofetil, among others, may be employed. Their purpose is to keep remission and lower the adverse effects associated with IS. Etanercept has not been proven successfully for the MP. Rituximab and 15-dexopergualin constitute promising the rapies for refractory WG.