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1.
Clin Rheumatol ; 42(3): 949-958, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36454342

RESUMEN

Among myositis-specific antibodies, anti-melanoma differentiation-associated gene 5 (anti-MDA5) is one of the antibodies with a unique spectrum that is expressed principally in clinically amyopathic dermatomyositis (CADM) and, to a lesser extent, in dermatomyositis (DM). In addition to muscle and classical skin involvement, patients with anti-MDA5 DM/CADM are characterized by the expression of rapidly progressive interstitial lung diseases, vasculopathic lesions, and non-erosive arthritis. Although cardiac involvement has been described in other inflammatory myopathies, such as myocarditis, pericarditis, and conduction disorders, in anti-MDA5 DM/CADM patients, heart disease is infrequent. We report a case of a young male presenting with constitutional symptoms, polyarthritis, skin ulcers, and mild muscle weakness who developed an episode of high ventricular rate atrial fibrillation during his hospitalization. The anti-MDA5 DM diagnosis was supported by increased muscular enzymes, positive anti-MDA5 and anti-Ro52 antibodies, and the presence of organizing pneumonia. He was treated with high-dose glucocorticoids, rituximab, and beta-blocker drugs and received pharmacological cardioversion, which improved his myopathy symptoms and stabilized his heart rhythm. Here, we describe eight similar cases of anti-MDA5 DM/CADM with cardiac involvement. The case presented and the literature reviewed reveal that although rare, physicians must be aware of cardiac disease in patients with suggestive symptoms to guarantee early assessment and treatment, thereby reducing life-treating consequences.


Asunto(s)
Dermatomiositis , Miositis , Humanos , Masculino , Autoanticuerpos , Dermatomiositis/diagnóstico , Helicasa Inducida por Interferón IFIH1
2.
Clin Rheumatol ; 41(11): 3565-3572, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35986821

RESUMEN

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS syndrome) is a recently described genetic disorder that gathers autoinflammatory symptoms and myeloid dysplasia. The first description was reported in 2020, and subsequently, a growing number of cases have been described worldwide. Herein, we describe a case of a 72-year-old male patient with VEXAS syndrome with p.Met41Val mutation of the UBA1 gene, prominent supraglottic larynx involvement, and costochondritis. To our knowledge, this is the first report of VEXAS syndrome in Colombia and South America. This disease could present features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. Supraglottic larynx chondritis and costochondritis are atypical manifestations. These features were proposed previously to differentiate relapsing polychondritis from VEXAS syndrome but are not entirely reliable like in the case described. A diagnosis of VEXAS should be considered in male patients with incomplete or complete features of the previously described conditions, refractory to treatment, requiring high-dose glucocorticoids, and associated progressive hematologic abnormalities. Key Points • VEXAS syndrome is a recently described genetic (somatic mutations in UBA1 gene) disorder that gathers autoinflammatory and hematologic manifestations. • VEXAS syndrome should be considered in male patients with incomplete or complete features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, refractory to treatment, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. • Glucocorticoids ameliorate symptoms effectively. However, other treatment options are limited due to a lack of evidence. Traditional immunosuppressants and biological therapy have been used empirically with limited efficacy and a transient effect. Bone marrow transplant offers a curative approach, but it has high morbidity and mortality.


Asunto(s)
Arteritis de Células Gigantes , Laringe , Síndromes Mielodisplásicos , Poliarteritis Nudosa , Policondritis Recurrente , Síndrome de Sweet , Anciano , Arteritis de Células Gigantes/complicaciones , Arteritis de Células Gigantes/diagnóstico , Arteritis de Células Gigantes/genética , Humanos , Inmunosupresores/uso terapéutico , Masculino , Síndromes Mielodisplásicos/complicaciones , Policondritis Recurrente/complicaciones , Policondritis Recurrente/diagnóstico , Policondritis Recurrente/genética , Síndrome de Sweet/complicaciones , Vacuolas
3.
Iatreia ; 35(2): 108-116, abr.-jun. 2022. tab
Artículo en Español | LILACS-Express | LILACS | ID: biblio-1421625

RESUMEN

Resumen Objetivo: describir las principales características demográficas y clínicas de los pacientes pediátricos con poliarteritis nodosa (PAN). Metodología: estudio descriptivo retrospectivo. Resultados: se obtuvieron las historias clínicas de menores de 18 años con diagnóstico de vasculitis o PAN atendidos entre el 2010 y 2019 en dos hospitales de Medellín. Se incluyeron 19 pacientes, 11 clasificados como PAN cutánea (PANC) según la definición de la Liga Europea contra el Reumatismo y la Sociedad Europea de Reumatología Pediátrica, y 8 como PAN sistémica (PANS) acorde con los criterios de Ankara 2008. El 57,9 % fueron varones (n = 11). La mediana de la edad en el diagnóstico fue 10 años (3-17 años) y del tiempo de seguimiento: 15 meses (0-105 meses). Las manifestaciones más frecuentes fueron nódulos subcutáneos (84,2 %), fiebre (57,9 %), artralgias (57,9 %) y dolor en las pantorrillas (52,6 %). Los pacientes con PANS presentaron más pérdida de peso y hemoglobina baja. 5 niños cursaron con úlceras (26,3 %) cuatro con PANC; 2 exhibieron necrosis lingual (10,5 %) y 2 necrosis digital (10,5 %), todos con PANC. El promedio de la PCR fue de 10,2 mg/dl (0,33-45,3) y, de la eritrosedimentación: 74 mm/hora (15-127 mm/hora). En las imágenes hubo un realce meníngeo, aneurismas intrahepáticas y mesentéricos, entre otros hallazgos. Todos recibieron esteroides sistémicos. Ninguno falleció. Conclusión: en esta serie, la PAN pediátrica fue más común en niños de edad escolar. La PANC fue más frecuente que la PANS. Las manifestaciones más descritas fueron los nódulos subcutáneos, las mialgias, la fiebre, las artralgias y el dolor en las pantorrillas. Aunque se ha considerado la PANC como una enfermedad benigna, estos pacientes pueden requerir tratamiento con glucocorticoides e inmunosupresores.


Summary Objective: To describe the main demographic and clinical characteristics of patients who were diagnosed with childhood polyarteritis nodosa (PAN). Methods: A descriptive study was conducted using retrospective data Results: The clinical registries from patients under 18th years with vasculitis or PAN diagnosis attended between 2010 and 2019 in two Medical Centers from Medellín were obtained. Nineteen patients were included: eleven classified as cutaneous PAN (PANC) according to the definition of the European League Against Rheumatism and the European Society of Pediatric Rheumatology and eight as systemic PAN according to Ankara 2008 Criteria. 57.9% were male. The median age at diagnosis was ten years (3-17), and the median follow-up period was 15 months (0-105). The most common clinical characteristics were subcutaneous nodules (84.2%), fever (57.9%), arthralgias (57.9%), and calf pain (52.6%). SPAN patients had more frequent weight loss and lower hemoglobin. Five patients had ulcers (26.3%), four of them with CPAN; two patients had lingual necrosis (10.5%), and two digital necrosis (10.5%), all of them also with CPAN. CRP mean was 10.2 mg/dl (0.33-45.3) and mean erythrocyte sedimentation rate (ESR) was 74 mm/h (15-127 mm/h). Meningeal enhancing, intrahepatic, and mesenteric aneurism, among others, were reported in diagnostic images. All patients required treatment with systemic glucocorticoid. None of the patients died during the follow-up period. Conclusions: In this pediatric series from Medellín-Colombia, PAN was more common in boys of school age. CPAN was more frequent than SPAN. The clinical characteristics most reported were subcutaneous nodules, myalgias, arthralgias, and calf pain. Although CPAN has been considered a benign disease, these patients may require systemic glucocorticoid and immunosuppressive therapy.

5.
Rev. colomb. reumatol ; 28(3): 203-212, jul.-set. 2021. tab, graf
Artículo en Español | LILACS | ID: biblio-1357272

RESUMEN

RESUMEN La arteritis de células gigantes es una vasculitis que afecta de manera predominante a vasos de gran calibre y aparece en personas mayores de 50 arios. Su presentación clínica incluye cefalea, alteraciones auditivas o síntomas similares a polimialgia reumática. En su forma más grave puede causar pérdida de visión uni- o bilateral, secundaria a neuropatía óptica isquémica de tipo arterítico. En la actualidad, el estándar de referencia para su diagnóstico es la biopsia de arterias temporales, procedimiento que no es inocuo y que puede tener como complicaciones infección, lesión nerviosa o sangrado, entre otras. Entre las técnicas no invasivas de diagnóstico, el ultrasonido y el Doppler de arterias temporales han tomado un rol cada vez más importante en el diagnóstico de esta entidad, dado que son pruebas benignas, con nulos efectos adversos y, a través de estrategias como las clínicas fast-track apoyadas en este método diagnóstico, se ha logrado reducir las complicaciones isquémicas de la enfermedad.


ABSTRACT Giant cell arteritis is a vasculitis that predominantly affects large calibre vessels, and usually appears in people over 50 years-old. Its clinical presentation includes headache, hearing impairment, or polymyalgia rheumatica-like symptoms. In its most severe form, it can cause uni- or bilateral vision loss secondary to arteritic ischaemic optic neuropathy. Currently, the gold standard for its diagnosis is the temporal artery biopsy, a procedure that is not harmless and may have complications such as infection, nerve injury, bleeding, among others. Among non-invasive diagnostic methods, the ultrasound and temporal artery Doppler have gained a predominant role in the diagnosis of giant cell arteritis, as it is a benign test with no adverse effects. Through strategies such as «fast-track¼ clinics, supported by this diagnostic method, a reduction has been achieved in ischaemic complications of the disease.


Asunto(s)
Humanos , Persona de Mediana Edad , Anciano , Disciplinas de las Ciencias Naturales , Arteritis de Células Gigantes , Ultrasonido , Vasculitis , Acústica , Enfermedades Cardiovasculares
6.
Rev. colomb. reumatol ; 28(3): 213-217, jul.-set. 2021. tab, graf
Artículo en Español | LILACS | ID: biblio-1357273

RESUMEN

RESUMEN Objetivo: La actividad y el riesgo de recaída de la arteritis de Takayasu son bajos durante la gestación. Hasta el 40% de las pacientes puede tener desenlaces obstétricos desfavorables, por lo que es importante conocer su comportamiento clínico. Describimos las características clínicas y el desenlace obstétrico de gestantes con arteritis de Takayasu atendidas en un hospital de alta complejidad. Materiales y métodos: Evaluación retrospectiva de historias clínicas de gestantes con arteritis de Takayasu atendidas en el Hospital Universitario San Vicente Fundación de Medellín, Colombia, entre 2011 y 2018. Resultados: Se incluyó en el estudio a 6 pacientes con mediana de edad al diagnóstico de 17,5 arios (RI 9,25), al parto de 24 arios (RI 8,25) y con una duración de la enfermedad de 5,5 arios (RI 10,5). Del total, 3 pacientes tenían compromiso aórtico extenso; al parto, 3 pacientes esta ban activas y requirieron inmunosupresores, 5 tenían hipertensión arterial, una desarrolló preeclampsia en el segundo trimestre, una tenía insuficiencia mitral y tricuspídea grave con disminución de la fracción de eyección del ventrículo izquierdo; 2 tenían aneurismas (arte ria subclavia izquierda y aorta ascendente). Ningún embarazo resultó en aborto ni parto pretérmino; hubo 2 óbitos fetales, uno por restricción del crecimiento intrauterino e insuficiencia placentaria, y otro de etiología desconocida; ambas pacientes con actividad de la enfermedad, afección aórtica extensa e hipertensión arterial. Fueron por cesárea 5 par tos por indicación materna; no hubo disección aórtica, rotura aneurismática ni hemorragia cerebral. Conclusión: Las pacientes con enfermedad activa y afección aórtica extensa presentaron resultados obstétricos desfavorables, lo que indica que el inadecuado control de la vasculitis genera mayores complicaciones materno-fetales.


ABSTRACT Objective: The activity and risk of relapse of Takayasu's arteritis are low during pregnancy. Up to 40% of patients may have unfavorable obstetric outcomes therefore it is important to know their clinical behavior. We describe the clinical characteristics and obstetric outcome of pregnant women with Takayasu arteritis treated in a hospital of high complexity. Materials and methods: A retrospective evaluation of medical records of pregnant patients with Takayasu's arteritis treated at Hospital Universitario San Vicente Fundación in Medellin, Colombia between 2011 and 2018. Results: Six patients with a median age at diagnosis 17.5 (RI 9.25) years, at delivery 24 (RI 8.25) years, disease duration 5.5 (RI 10.5) years. Three patients had extensive aortic involvement; at delivery, 3 patients were active and required immunosuppressants, 5 had high blood pres sure, one developed preeclampsia in the second trimester, one had severe mitral and tricus pid insufficiency with a decreased ejection fraction of the left ventricle; 2 had aneurysms (left subclavian artery and ascending aorta). No pregnancy resulted in abortion or preterm birth; there were 2 fetal deaths, one due to intrauterine growth restriction and placental insuffi ciency and another of unknown etiology; both patients with disease activity, extensive aortic condition, and arterial hypertension. Five deliveries were by cesarean section by maternal indication; there was no aortic dissection, aneurysmal rupture or cerebral hemorrhage. Conclusion: Patients with active disease and extensive aortic compromise presented unfa vorable obstetric results, suggesting that inadequate control of vasculitis leads to greater maternal-fetal complications.


Asunto(s)
Humanos , Femenino , Adolescente , Adulto , Varicocele , Preeclampsia , Complicaciones del Embarazo , Vasculitis , Enfermedades Cardiovasculares , Arteritis de Takayasu
7.
Clin Rheumatol ; 40(1): 181-191, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32529420

RESUMEN

OBJECTIVE: To identify factors associated with active tuberculosis (TB) in patients with systemic lupus erythematosus (SLE). METHODS: We performed a retrospective case-control study in two tertiary care teaching hospitals in Medellín, Colombia. From January 2007 to December 2017, a total of 268 patients with SLE were included. SLE patients with TB (cases) were matched 1:3 with SLE patients without TB (controls) by disease duration and the date of the hospitalization in which the diagnosis of TB was made (index date of cases) to the nearest available rheumatology hospitalization in the matched controls (± 2 years). Conditional univariable and multivariable logistic regression analyses were performed. RESULTS: Sixty-seven cases and 201 controls were assessed. Only pulmonary TB occurred in 46.3%, only extrapulmonary TB in 16.4% and disseminated TB in 37.3% of cases. Multivariable logistic regression analysis showed that lymphopenia (OR, 2.91; 95% CI 1.41-6.03; P = 0.004), 12-month cumulative glucocorticoid dose ≥ 1830 mg (OR, 2.74; 95% CI 1.26-5.98; P = 0.011), and having been treated with ≥ 2 immunosuppressants during the last 12 months (OR, 2.81; 95% CI 1.16-6.82; P = 0.022) were associated with TB after adjusting for age, sex, ethnicity, disease duration, disease activity, and comorbidity index. A trend towards an association of kidney transplantation with TB was also found (OR, 3.77; 95% CI 0.99-14.30; P = 0.051). CONCLUSION: Among SLE patients, cumulative glucocorticoid dose, lymphopenia, and the use of ≥ 2 immunosuppressants during the last 12 months were associated with active TB infection. Key Points • Among SLE patients, a cumulative dose of glucocorticoids equivalent to 5 mg/day of prednisone during the last 12 months is independently associated with the development of TB. • The use of two or more immunosuppressants during the last 12 months is also a risk factor for TB infection development is SLE patients. • Lymphopenia is predominant in SLE patients with TB, being especially profound in those with disseminated TB. • Renal transplant recipients with SLE also have an elevated risk of TB.


Asunto(s)
Lupus Eritematoso Sistémico , Tuberculosis , Estudios de Casos y Controles , Colombia/epidemiología , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Tuberculosis/complicaciones , Tuberculosis/epidemiología
9.
Reumatol Clin (Engl Ed) ; 16(3): 242-244, 2020.
Artículo en Inglés, Español | MEDLINE | ID: mdl-29789229

RESUMEN

We report the case of a 47-year-old man with a 9-year history of psoriatic arthritis (PsA) in whom we detected renal involvement, hypocomplementemia, peripheral neuropathy, acral necrotic lesions and positive cryoglobulins. The results of the diagnosis led us to conclude that the clinical picture corresponded to cryoglobulinemic vasculitis concomitant with PsA. In addition, we present a review of the literature on the presence of these two diseases in a single patient.


Asunto(s)
Artritis Psoriásica/complicaciones , Crioglobulinemia/complicaciones , Vasculitis/complicaciones , Humanos , Masculino , Persona de Mediana Edad
10.
J Clin Rheumatol ; 25(3): e16-e26, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-29782427

RESUMEN

OBJECTIVES: The aims of this study were to describe clinical and laboratory manifestations of patients with levamisole-adulterated cocaine-induced vasculitis/vasculopathy and to propose a skin classification according to the distribution and severity of lesions. METHODS: We report the characteristics of 30 patients admitted with levamisole-adulterated cocaine-induced vasculitis/vasculopathy in 4 high-complexity institutions in Colombia, from December 2010 to May 2017. We compare our findings with the main published series. RESULTS: Median age was 31 years (interquartile range, 27-38 years) with a male-to-female ratio of 5:1. Eighty-three percent of the patients had retiform purpura affecting the limbs, buttocks, face, or abdomen; 73% had ear necrosis, 50% cutaneous ulcers, 17% genital necrosis, 13% oral ulcers, and 10% digital necrosis. Cutaneous involvement was classified according to the frequency of the compromised corporal area, and purpuric lesions were stratified in 4 grades of severity. Anti-neutrophil cytoplasmic autoantibodies were positive in 85% of the cases, lupus anticoagulant in 73%, and antinuclear autoantibodies in 57%; rheumatoid factor was negative in all cases. We found nephritis in 17 cases (57%). Prednisolone was used in most of the patients (70%), with other immunosuppressive agents being used in a lower percentage. Improvement was observed in 93% of the patients, but symptoms recurred in 40%, attributed to relapses in consumption. End-stage chronic renal disease developed in 10% of the cases, and 1 patient died. CONCLUSIONS: Because of rising cocaine consumption and levamisole adulteration frequency, levamisole-adulterated cocaine-induced vasculitis/vasculopathy is becoming more common. Detailed characterization of skin involvement coupled with multiple antibody positivity is essential for a diagnosis. Renal involvement is frequent, clinically and histologically heterogeneous, and potentially serious.


Asunto(s)
Trastornos Relacionados con Cocaína/complicaciones , Cocaína , Glomerulonefritis , Levamisol , Púrpura , Vasculitis , Adyuvantes Farmacéuticos/efectos adversos , Adyuvantes Farmacéuticos/farmacología , Adulto , Autoanticuerpos/sangre , Cocaína/farmacología , Colombia , Inhibidores de Captación de Dopamina/farmacología , Contaminación de Medicamentos , Femenino , Glomerulonefritis/inducido químicamente , Glomerulonefritis/diagnóstico , Glomerulonefritis/inmunología , Glomerulonefritis/terapia , Humanos , Levamisol/efectos adversos , Levamisol/farmacología , Masculino , Necrosis , Manejo de Atención al Paciente/métodos , Púrpura/inducido químicamente , Púrpura/diagnóstico , Púrpura/inmunología , Púrpura/terapia , Piel/patología , Resultado del Tratamiento , Vasculitis/inducido químicamente , Vasculitis/diagnóstico , Vasculitis/inmunología , Vasculitis/terapia
11.
Rev. colomb. reumatol ; 23(2): 73-78, Apr.-June 2016. tab
Artículo en Inglés | LILACS | ID: biblio-830395

RESUMEN

INTRODUCTION: In patients with lupus nephritis it is necessary to define the need for immuno-suppressive therapy according to histological class observed in renal biopsy. OBJECTIVE: To evaluate the agreement between the opinions of six independent clinical rheumatologists regarding the need for immunosuppression and the result of renal biopsy in patients with lupus nephritis.MATERIALS AND METHODS: A cross-sectional study on the agreement between a diagnostic test in adult patients with systemic lupus erythematosus. Each rheumatologist predicted the out-come of the biopsy. In order to evaluate the agreement, a dichotomous qualitative outcome was defined and was considered zero if it was not necessary to add a cytotoxic (classes I, II and VI), and otherwise was (classes III, IV, V or combinations). The percentage agreement and kappa statistics with a confidence interval of 95% was measured. RESULTS: Information was collected on 34 patients, with a total of 204 predictions made by 6 rheumatology interns. Rheumatologists were correct in their clinical impression in 180 cases (88.2% concordance rate, overall kappa of 0.62 (95% CI = 0.48-0.76. Of the 204 scenarios generated, 162 corresponded to proliferative forms of lupus nephritis, for which the rheumatologists anticipated the need for immunosuppression in 153 and failed to treat in 9 cases (5.5%, or about 1 in 18). CONCLUSIONS: The clinical opinion of rheumatologist is quite successful in defining the need for immunosuppression. In general, expert opinion could eventually be offered as an alternative choice to renal biopsy for the patient.


INTRODUCCIÓN: En pacientes con nefritis lúpica es necesario definir la necesidad de inmunosupresores de acuerdo con la clase histológica observada en la biopsia renal. OBJETIVO: Evaluar la concordancia entre la opinión clínica de 6 reumatólogos independientes con respecto a la necesidad de inmunosupresión y el resultado de la biopsia renal en pacientes con nefritis lúpica. MATERIALES Y MÉTODOS: Estudio de corte transversal de concordancia de una prueba diagnóstica. Se incluyeron pacientes adultos con lupus eritematoso sistémico. Cada reumatólogo intentaba predecir el resultado de la biopsia. Para evaluar la concordancia se consideró un desenlace cualitativo dicotómico así: cero si no era necesario adicionar citotóxico (clases I, II o VI), y uno en caso contrario (clases III, IV, V o combinaciones). Se midió el porcentaje de acuerdo y el estadístico kappa con intervalo de confianza del 95%. RESULTADOS: Se recolectó información de 34 pacientes. Participaron 6 internistas reumatólogos para un total de 204 predicciones. Los reumatólogos acertaron en su impresión clínica en 180 ocasiones (porcentaje de concordancia 88.2%, kappa global de 0.62 (IC95% = 0.48-0.76]. De los 204 escenarios generados 162 correspondían a formas proliferativas, de los cuales los reumatólogos anticiparon la necesidad de inmunosupresión en 153 y dejaron de tratar en 9 ocasiones (5.5%, o uno de cada 18, aproximadamente). CONCLUSIONES: La opinión clínica del reumatólogo es bastante acertada para definir la necesidad de inmunosupresión. En general, la opinión del experto podría llegar a ofrecerse como una alternativa a la biopsia renal para el paciente que así lo escoja.


Asunto(s)
Humanos , Nefritis Lúpica , Terapia de Inmunosupresión , Consenso
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