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Objective To phenotype SLE based on symptom burden (disease damage, system involvement and patient reported outcomes), with a specific focus on objective and subjective cognitive function. Methods SLE patients ages 18–65 years underwent objective cognitive assessment using the ACR Neuropsychological Battery (ACR-NB) and data were collected on demographic and clinical variables, disease burden/activity, health-related quality of life (HRQoL), depression, anxiety, fatigue and perceived cognitive deficits. Similarity network fusion (SNF) was used to identify patient subtypes. Differences between the subtypes were evaluated using Kruskal–Wallis and χ2 tests. Results Of the 238 patients, 90% were female, with a mean age of 41 years (s.d. 12) and a disease duration of 14 years (s.d. 10) at the study visit. The SNF analysis defined two subtypes (A and B) with distinct patterns in objective and subjective cognitive function, disease burden/damage, HRQoL, anxiety and depression. Subtype A performed worst on all significantly different tests of objective cognitive function (P Conclusion This study demonstrates the complexity of cognitive impairment (CI) in SLE and that individual, multifactorial phenotypes exist. Those with greater disease burden, from SLE-specific factors or other factors associated with chronic conditions, report poorer cognitive functioning and perform worse on objective cognitive measures. By exploring different ways of phenotyping SLE we may better define CI in SLE. Ultimately this will aid our understanding of personalized CI trajectories and identification of appropriate treatments.

Objective We investigated the autoantibody (autoAb) profiles in ANA+ individuals lacking systemic autoimmune rheumatic disease (SARD) and early SARD patients to determine the key differences between these groups and identify factors that are associated with an increased risk of symptomatic progression within the next 2 years in ANA+ individuals. Methods Using custom antigen (Ag) microarrays, 144 IgM and IgG autoAbs were surveyed in 84 asymptomatic and 123 symptomatic (48 UCTD and 75 SARD patients) ANA+ individuals. AutoAbs were compared in ANA+ individuals lacking a SARD diagnosis with ≥2 years follow-up (n = 52), including all those who demonstrated progression (n = 14) during this period, with changes over time assessed in a representative subset. Results We show that ANA+ individuals have autoAb to many self-Ags that are not being captured by current screening techniques and very high levels of these autoAbs are predominantly restricted to early SARD patients, with SLE patients displaying reactivity to many more autoAgs than the other groups. In general, the symptoms that developed in progressors mirrored those seen in SARD patients with similar patterns of autoAbs. Only anti-Ro52 Abs were found to predict progression (positive predictive value 46%, negative predictive value 89%). Surprisingly, over 2 years of follow-up the levels of autoAbs remained remarkably stable regardless of whether individuals progressed or not. Conclusion Our findings strongly argue that development of assays with an expanded set of auto-Ags and enhanced dynamic range would improve the diagnostic and prognostic ability of autoAb testing.

espanolIntroduccion. La enfermedad de Still del adulto (ESA) es una condicion medica poco frecuente que puede presentar complicaciones graves; sin embargo, aun no se conocen con claridad sus factores de riesgo, sus factores pronosticos, ni los aspectos asociados a las recaidas y a la refractariedad a esteroides en individuos con esta condicion. Objetivos. Describir el comportamiento clinico de la ESA y determinar los factores asociados a la refractariedad a esteroides, a recaidas y a complicaciones en pacientes con esta enfermedad. Materiales y metodos. Estudio retrospectivo de cohortes que incluyo 45 pacientes diagnosticados con ESA entre enero de 2007 y enero de 2017 en 2 hospitales de referencia. Se presentan medidas de resumen. Se realizaron dos analisis de regresion logistica para identificar posibles factores que expliquen la refractariedad a esteroides, las recaidas y el desarrollo de complicaciones en la poblacion de estudio. Resultados. La edad promedio de los participantes fue 42.13±15.8 anos. De los 45 pacientes incluidos, 23 (51.1%) eran mujeres, a 42 (93.3%) se le prescribieron esteroides, y, de estos, 13 (32%) se consideraron refractarios a esteroides. Se presentaron complicaciones en 12 (26.7%) individuos y su frecuencia fue mayor en pacientes con temperatura maxima >39°C. Finalmente, 33 (73.3%) pacientes tuvieron un seguimiento mayor a 1 ano, de los cuales, 17 (37.8%) presentaron recaidas, las cuales fueron mas frecuentes en individuos refractarios a esteroides o con esplenomegalia. Conclusion. Los pacientes que recibieron cualquier tipo de terapia biologica fueron mas refractarios a esteroides; por su parte, la refractariedad a esteroides y la esplenomegalia se asociaron a un mayor numero de recaidas, y la temperatura >39°C se asocio a al desarrollo de complicaciones. EnglishIntroduction: Adult Still disease (ASD) is a rare disorder that can lead to serious complications; however, risk factors associated with the development of this disease, its prognostic factors, and aspects related to relapse and steroids refractoriness in individuals with this condition are not yet clearly known. Objectives: To describe the clinical behavior of ASD and determine factors associated with steroid refractoriness, relapse, and complications in patients with this condition. Materials and methods: A retrospective cohort study was conducted in 45 patients diagnosed with ASD between January 2007 and January 2017 in 2 reference hospitals. Summary statistics are presented. In addition, two logistic regression analysis were conducted in order to identify possible factors explaining steroid refractoriness, relapses, and the development of complications in the study population. Results: Participants’ average age was 42.13 ± 15.8 years. Out of the 45 patients, 23 (51.1%) were women, 42 (93.3%) were prescribed steroids, and of these, 13 (32%) were considered as steroid refractory. Complications were observed in 12 individuals (26.7%), and their frequency was higher in patients whose maximum body temperature was higher than 39°C. Finally, 33 (73.3% patients had a follow-up greater than 1 year, of which 17 (37.8%) suffered relapses; in addition, relapses were more frequent in patients who were steroid refractory or those with splenomegaly. Conclusion: Patients who were prescribed any type of biological therapy were more steroid refractory. On the other hand, steroid refractoriness and splenomegaly were associated with a higher frequency of relapses, while having a body temperature >39°C was associated with developing complications.

In this article, we describe three cases of lupic patients that developed microangiopathy. Those cases represent the principal etilogies of microangiopathy in Lupus such as, microangiopathic haemolytic anaemia and antiphospholipid syndrome with and without castastrofic manifestations. The clinicians must know deeply about this condition to recognize it in a timely manner.

Resumen Aunque las vasculitis asociadas a ANCA tienen manifestaciones clinicas diversas, la afeccion valvular es infrecuente, especialmente en el contexto de una poliangeitis microscopica. Reportamos el caso de una mujer de 38 anos con vasculitis ANCA con caracteristicas clinicas de poliangeitis microscopica y anticuerpos anti-mieloperoxidasa positivos (MPO), que presento una masa vegetante en la valvula mitral que resolvio con inmunosupresion.

Se reporta el caso de un hombre de 47 arios con intoxicación crónica por plomo, secundaria a exposición laboral de 5 años, con sintomatología típica de saturnismo. Consultó por debilidad muscular generalizada, disfagia y parestesias en extremidades. Se documentaron altos niveles de plomo en sangre, asociados a neuropatía periférica, confirmada por electromiografía, y disminución de la fuerza muscular en cintura escapular y pélvica (deltoides y vasto medial), así como atrofia de músculos del cuello (flexores y extensores) manifestada como cefaloparesia. Adicional al cuadro de saturnismo se diagnosticó miopatía inflamatoria con base en la elevación de enzimas musculares, miositis por resonancia magnética nuclear y biopsia muscular compatible, siendo, hasta donde se sabe, el primer reporte conocido de la coexistencia de estas 2 enfermedades. A case is presented on a 47-year-old man with chronic lead poisoning with typical symptoms after 5 years of occupational exposure. He consulted for generalised muscle weakness, early dysphagia, cephaloparesia, and paresthesias in upper and lower limbs. He also had atrophy and decreased proximal muscle strength (deltoid and medial vast) and in both flexor and extensor muscles of the neck. He had a history of high blood lead levels and peripheral neuropathy documented by electromyography. In addition to the diagnosis of lead poisoning, inflammatory myopathy was confirmed based on muscle enzyme elevation, muscular inflammation in magnetic resonance imaging, and typical findings in a muscle biopsy. To our knowledge, this is the first report where both conditions are documented in one patient.

RESUMEN El daño irreversible de órgano es predictor de morbilidad, mortalidad, mayor acúmulo de daño y mala calidad de vida en los pacientes con lupus eritematoso sistémico. Objetivos: Caracterizar el daño y los factores que mejor lo explican, en una población de pacientes colombianos con lupus eritematoso sistémico. Métodos: Estudio retrospectivo de seguimiento a una cohorte. El daño se midió con el SLICC/ACR (índice de Systemic Lupus International Collaborating Clinics y del American College of Rheumatology) y la actividad de la enfermedad por SELENA SLEDAI. La caracterización del daño se hizo mediante estadística descriptiva, los factores asociados con el desenlace se evaluaron con Chi2 de Pearson o Fisher, t de Student o U de Mann-Whitney; la proporción de pacientes que acumularon daño se evaluó con el test de Friedman y el puntaje acumulado con el test de Wilcoxon. La determinación de los factores asociados independientemente con el desenlace se hizo con una regresión logística. Resultados: Se incluyeron 161 pacientes con diagnóstico de novo y seguimiento mínimo de un año; el 28,9% sufrió daño. Los dominios más representados fueron el neuropsiquiátrico, renal y vascular. Los anticuerpos antifosfolípido, las dosis promedio de prednisolona mayores a 12,5 mg/día y presentar 2 o más recaídas se asociaron independientemente al daño orgánico. Conclusiones: Los anticuerpos antifosfolípido, la dosis de esteroides y la frecuencia de recaídas se asocian al daño orgánico en una población colombiana de pacientes con lupus eritematoso sistémico. ABSTRACT Irreversible organ damage is a predictive factor of morbidity, mortality, increased accumulation of damage, and poor quality of life in patients with systemic lupus erythematosus. Objectives: To describe the damage, and the factors that best explain it, in a population of Colombian patients. Methods: A retrospective follow-up study of a patient cohort. The damage was measured using the Systemic Lupus International Collaborating Clinics (SLICC) and the American College of Rheumatology ACR index, and disease activity by SELENA SLEDAI. Descriptive statistics were used to describe the damage. The factors associated with the outcome were evaluated with Pearson's or Fisher's Chi2, Student's t or Mann-Whitney's U. The proportion of patients that accumulated damage was evaluated with the Friedman test, and the cumulative score with the Wilcoxon test. The determination of the factors independently associated with the outcome was performed using logistic regression. Results: A total of 161 patients with recent diagnosis, and followed for one year or more, were included, 28.9% of whom had suffered damage. The most represented domains were neuropsychiatric, renal and vascular. Anti-phospholipid antibodies, mean doses of prednisolone greater than 12.5 mg/day, and suffering two or more relapses were independently associated with organ damage. Conclusions: Anti-phospholipid antibodies, steroid doses and frequency of relapses are associated with organ damage in a Colombian population of patients with SLE.

RESUMEN Introducción: El síndrome antifosfolípido se caracteriza por la presencia de anticuerpos contra fosfolípidos de membrana y manifestaciones clínicas, principalmente trombóticas y obstétricas. Su tratamiento se basa en la anticoagulación indefinida, generalmente con warfarina, la cual, por diversos factores, no siempre es factible por lo que es necesario el uso de terapias alternativas. Objetivo: Describirla experiencia con rivaroxabán en pacientes con síndrome antifosfolípido. Materiales y métodos: Estudio descriptivo en el que se evaluaron pacientes que cumplieron los criterios de Sydney de 2006 para síndrome antifosfolípido y que recibieron anticoagulación con rivaroxabán a dosis de 20 mg día en 2 hospitales de referencia en Medellín (Colombia), entre enero de 2012 y abril de 2015. Resultados: Se incluyeron 7 pacientes con una media de edad de 36,6 ± 10,8 arios (rango: 2355). De estos, 4 individuos tenían trombosis venosa, 5 trombosis arteriales, 5 anticuerpos anticardiolipinas positivos, 3 anticoagulante lúpico positivo, 2 pacientes tenían anti-f32 glicoproteína positivo y un paciente triple positividad de anticuerpos. La mediana de utilización de la warfarina fue de 15 meses (rango: 1-36). Las razones para el inicio de rivaroxabán fueron: sangrado (n = 2), rango subterapéutico de anticoagulación (n = 2), toxicodermia (n = 1), intolerancia gastrointestinal (n = 1) y retrombosis (n = 1). El tiempo de uso fue 17,9 ± 13,4 meses (rango: 3-34) y durante el periodo de seguimiento no se presentaron eventos adversos, pero sí 2 episodios nuevos de trombosis. Conclusión: El uso de inhibidores del factor Xa en una serie de pacientes con síndrome antifosfolípido e imposibilidad para el uso de warfarina mostró un adecuado perfil de seguridad; no obstante, hubo 2 episodios recurrentes de trombosis. Background: Antiphospholipid syndrome is an autoimmune disease with antibodies against membrane phospholipids with mainly thrombotic and/or obstetric manifestations. Its treatment is generally based on indefinite anticoagulation, usually with warfarin, and which, for various factors, is not always feasible, making it necessary to use alternative therapies. Objective: To describe the experience with rivaroxaban in patients with antiphospholipid syndrome. Materials and methods: A descriptive study was conducted on subjects that met the 2006 Sydney criteria for antiphospholipid antibodies syndrome and received anticoagulation with rivaroxaban at 20mg daily dose in 2 reference hospitals in Medellin, Colombia, between January 2012 and April 2015. Results: The study included 7 patients, with a mean age of 36±10.8 years (range 23-55). Four patients had venous thrombosis, 5 arterial, 5were positive for anticardiolipin antibodies, 3 reactive to lupus anticoagulant, 2 anti-β2 glycoprotein positive subjects, and one patient had triple antiphospholipid antibody positivity. The median time of warfarin use was 15 months (RIQ 1-36). The reasons for starting rivaroxaban were: bleeding (n = 2), sub-therapeutic coagulation ranges (n = 2), toxicoderma, gastrointestinal intolerance, and re-thrombosis (n = 1, each). The time of use was 17.9±13.4 months (range: 3-34). There were 2 recurrent cases of thrombosis during follow-up, and no adverse events. Conclusion: The use of factor Xa inhibitors in a series of patients with antiphospholipid syndrome and unable to use warfarin showed an adequate safety profile; however, 2 recurrent episodes of venous thrombosis occurred.

Bacillus Calmette-Guerin (BCG) is a vaccine derived from attenuated strains of Mycobacterium bovis. Mycobacterium bovis BCG-based therapy is considered an effective alternative for bladder carcinoma in situ when exclusive transurethral resection is inadequate. Among the complications associated with therapy, there are few reports in the literature on reactive arthritis. We report the case of a 56-year-old woman who develops predominantly peripheral spondyloarthritis, following instillation of BCG, as preventive treatment of relapse onset high-grade urothelial carcinoma after surgical resection.

Behcet's disease, is an inflammatory disease of unknown etiology with multisystem involvement and chronic course. In addition to the triad (recurrent oral and genital ulcers, and uveitis), it may exhibit involvement of other organs, but it is a rare hematologic commitment. Behcet's disease association with myelodysplastic processes, although uncommon, has been previously described. We report the case of a patient with Behcet's disease who developed thrombocytopenia and monocytosis. Trisomy 8 was found in karyotypic ana­lysis and findings of myelodysplastic/myeloproliferative disease in the bone marrow. We discuss the relationship between trisomy 8, myelodysplastic syndrome and Behcet's disease.

Reportamos el caso de una paciente con unas lesiones en piel que fueron diagnosticadas inicialmente como morfea por lo cual fue remitida al servicio de reumatología. Las características clínicas e histológicas nos permitieron descartar morfea clásica y clasificarla mejor como una atrofoderma de Pasini y Pierini. Aunque algunos la consideran una variante localizada y benigna de la morfea para otros se trata de una entidad nosológica distinta. Esta enfermedad debe ser considerada en el diagnóstico diferencial de la esclerodermia localizada y todos aquellos desórdenes que semejan esclerodermia para lograr un diagnóstico correcto y evitar tratamientos y exámenes innecesarios. Informamos la ausencia de respuesta de sus lesiones con el uso de hidroxicloroquina. We present the case of a patient with skin lesions diagnosed with morphea that was sent by her dermatologist to the outpatient clinic of our Rheumatology Department. We made a diagnosis of atrophoderma of Pasini and Pierini (APP). It is often considered a variant or an incomplete form of morphea. There is disagreement within the medical community as to whether APP should be classified as a type of morphea or as a separate individual disorder. The disease should be included in the differential diagnosis of localized scleroderma and scleroderma-like disorders to spare patients unnecessary therapies. In our patient, the lesions did not resolve despite an 8-month course of hydroxychloroquine.

El síndrome de Gardner-Diamond o púrpura psicógena es una vasculopatía de presunto origen autoinmune que se caracteriza por una reacción cutánea localizada, asociada a situaciones de estrés emocional. Se presenta el caso de una paciente con lesiones equimóticas, dolorosas y de aparición intermitente, relacionadas con diversos eventos estresores, que habían sido manejadas como manifestación de lupus. Gardner-Diamond syndrome or psychogenic purpura is an vasculopathy characterized by a localized cutaneous reaction, associated with episodes of emotional stress or mental illness as trigger factors. A case of a female patient with multiple, intermittent, nodular, ecchymotic and painful lesions related to various stressing events that was treated as lupus is reported below.

The development of autoimmune disorders requires a combination of genetic, immunological, and environmental factors. Infectious agents, such as viruses and bacteria, can trigger autoimmunity through different mechanisms, and for systemic vasculitis in particular, microbial agents have been suggested to be involved in its pathogenesis. Although the exact mechanisms have not been fully elucidated, different theories have been postulated. This review considers the role of infections in the etiology of primary vasculitis, emphasizing their related immunological events.

La granulomatosis con poliangitis como causa del síndrome pulmón-riñón es infrecuente en niños. Presentamos el caso de un paciente de 13 años con hemorragia alveolar, glomerulonefritis rápidamente progresiva, escleritis y anticuerpos anti-PR3 (proteinasa 3). Hacemos referencia a las características de esta vasculitis asociada a anticuerpos contra el citoplasma de los neutrófilos (ANCA) en los niños y a su enfoque terapéutico.