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La principal complicación del síndrome antifosfolipídico (SAF) es la diátesis trombótica, tanto arterial como venosa, que suele manifestarse como trombosis venosa profunda o diversos tipos de eventos cerebrovasculares; sin embargo, puede ocurrir en cualquier vaso sanguíneo, incluyendo muy ocasionalmente los vasos adrenales. Puede ser incluso la primera manifestación del SAF, y suele ser bilateral, con el consecuente desarrollo de insuficiencia adrenal (enfermedad de Addison). Se ha propuesto que el fenómeno inicial es una trombosis de las venas adrenales que condiciona un infarto. Con frecuencia, el compromiso bilateral es irreversible y requiere terapia de suplencia hormonal de forma permanente. La afectación unilateral es más rara y parece tener mejor pronóstico. Se reporta el caso de una paciente de 35 años con antecedente de SAF (abortos a repetición, livedo reticularis,presencia de anticuerpos antifosfolipídicos y anticoagulante lúpico), que presentó un infarto adrenal derecho durante el puerperio y cuya manifestación clínica fue dolor lumbar súbito e intenso. El diagnóstico se realizó con escanografía contrastada. La paciente ha evolucionado favorablemente, sin que se evidencie insuficiencia adrenal.

Lupus nephritis (LN) is one of the most prevalent and severe complications of systemic lupus erythematosus (SLE), requiring reliable urine and serum biomarkers to evaluate it. Anti-nucleosome and anti-C1q antibodies are associated with LN in several geographic regions. Also, southwest Colombia has a heterogeneous ethnicity, which motivated the evaluation of the frequency and relationship of such markers with LN in this region.

Lupus nephritis (LN) is one of the most prevalent and severe complications of systemic lupus erythematosus (SLE), requiring reliable urine and serum biomarkers to evaluate it. Anti-nucleosome and anti-C1q antibodies are associated with LN in several geographic regions. Also, southwest Colombia has a heterogeneous ethnicity, which motivated the evaluation of the frequency and relationship of such markers with LN in this region.

Malta is a small island state in the Mediterranean, with one of the highest population densities and per capita car ownership rates in the European Union. University campuses located in urban areas have a substantial role to play in transport planning and policies since they are amongst the largest generators and attractors of commuters. However, at the University of Malta, mobility to and from the work and study place is still characterised by high levels of private car use, resulting in traffic congestion, parking constraint and land use uptake. The current transport system is discriminatory towards those outside the car, who cannot or do not want to drive. These people find themselves struggling for space to walk and cycle, whilst bus users have to withstand long journey times due to car traffic and limited priority. A participatory Active Travel workshop was organised at the university in order to better understand the determinants of pedestrian and cyclist mobility to and from the university campus. Participants included people from the university community as well as other interested stakeholders. Four different guided walks or cycles led participants along predefined paths in the surroundings of the university, to assess the environment from the perspective of a pedestrian or cyclist. Information on the active travelers’ experiences allows the assessment and spatial distribution of perceived walkability and bikeability around campus. These outputs are then presented in a participatory web map that has evident potential to assist in the design and improvement of public space for promoting active travel. Contributions from the workshop are used to identify barriers and opportunities for walking and cycling and to construct tangible policy recommendations for promoting active travel in the vicinity of the university and the wider urban environment in Malta.

La infección por Salmonella enteritidis(ISE) es rara en pacientes con lupus eritematoso sistémico (LES), y comúnmente se confunde con exacerbaciones del LES. ISE en pacientes con LES se ha relacionado con malos desenlaces. Este artículo describe los hallazgos clínicos y desenlaces de 5 pacientes con LES y diagnóstico de ISE, en una cohorte de 325 pacientes (1,53%). Todos los pacientes fueron mujeres, con una edad promedio de 28,2 (14 a 37 años). La duración promedio del LES antes de la ISE clínica fue de 3,2 años (1 a 7 años). No hubo evidencia de infección recurrente. Los pacientes fueron tratados con ciprofloxacino o ceftriaxona, con resolución de las manifestaciones clínicas en 4 pacientes. Un paciente murió debido a falla orgánica multisistémica. Nuestro reporte remarca las manifestaciones clínicas inespecíficas de la ISE que asemeja actividad lúpica. Un adecuado diagnóstico y una terapia antibiótica temprana son esenciales para mejorar los desenlaces de estos pacientes.

The BAFF complex (B cell activator factor) composed by the BAFF cytokine, APRIL and their receptors – BAFF-R (BR3), TACI, BCMA – influences B-lymphocyte maturation, peripheral survival and immunoglobulins class isotype switching, with multiple potential clinical implications. In this review we discuss BAFF biologic functions and it relevance in several clinical disorders – autoimmune, neoplastic, infectious and BAFF therapies. BAFF/APRIL serum levels are increased in autoimmune diseases where their concentrations are related with disease antibodies titles, activity, progression and inclusive organic compromise, making its inhibition an important therapeutic target.

El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune, cuyo compromiso se puede ver reflejado en la calidad de vida, incapacidad y progresión a complicaciones en los pacientes. Con el fin de evaluar estos componentes, diversas escalas clinimétricas se han utilizado en el SSp.

Primary Sjögren's syndrome (pSS) is an autoimmune disease that can affect quality of life, cause disability, including progression to systemic complications in patients. In order to evaluate these components, several clinimetric scales have been used in pSS.

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis, inflammation, and microcirculatory alterations.

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis, inflammation, and microcirculatory alterations.

Salmonella enteritidisinfection (SEI) is rare in systemic lupus erythematosus (SLE) patients, and it is often mistaken with SLE flares. SEI in SLE patients has been associated with a poor outcome. A description is presented of the clinical features and outcomes of five patients with an SEI from a cohort of 325 patients with SLE (1.53%). All patients were women, with a mean age of 28.2 years (14 to 37 years). Mean duration of SLE before clinical SEI was 3.2 years (1 to 7 years). There was no evidence of recurrent infection. The patients were treated with ciprofloxacin and/or ceftriaxone, with resolution of clinical manifestations in four of them. One patient died due to multisystem organ failure. Our report highlights the unspecific clinical manifestations of SEI that resembles lupus flare-up. An accurate diagnosis and early antibiotic treatment are essential to improve their outcomes.

La enfermedad relacionada con IgG4 es una condición inflamatoria sistémica, caracterizada por la infiltración de diversos órganos por complejos formados por células plasmáticas IgG4 positivas, asociadas con niveles elevados de IgG4 en el suero. El diagnóstico de esta enfermedad es complejo y los hallazgos clínicos no son patognomónicos. En esta serie de casos, describimos un amplio espectro clínico en 4 pacientes, en quienes, después de descartar otros diagnósticos, se confirmó la enfermedad relacionada con IgG4. A pesar de que esta enfermedad no es común, se debe considerar entre los diagnósticos diferenciales de enfermedades con afectación de múltiples órganos. Este reporte de pacientes con enfermedad relacionada con IgG4, en Colombia, resalta un amplio espectro de presentaciones clínicas, incluyendo estenosis subglótica, pancreatitis autoinmune, fibrosis retroperitoneal y compromiso sistémico.

IgG4-related disease is an inflammatory systemic condition noted by the infiltration of different organs by IgG4-bearing plasma cells, as well as elevated serum IgG4 levels. Diagnosis of this condition is complex, and clinical findings are not particularly indicative. In this case series, a description is presented on 4 patients with a wide spectrum of clinical manifestations, in whom, after ruling out different options, a diagnosis of IgG4 related disease was confirmed. Despite this disease not being common, it should be considered among the options whenever multiple organs are affected. This report of patients with IgG4-related disease in Colombia highlights a wide spectrum of clinical presentations, including subglottic stenosis, autoimmune pancreatitis, retroperitoneal fibrosis, and systemic compromise.

Belimumab, a monoclonal type Ig G1 antibody that binds and inhibits the soluble form of the Blys (B lymphocyte stimulator) has shown to be effective in the management of systemic lupus erythematosus (SLE). However, its effectiveness is unknown in an ethnically variable population, such as in Colombia.

El belimumab es un anticuerpo monoclonal tipo IgG1 que se une e inhibe la forma soluble de Blys (estimulador de linfocitos B) y ha mostrado efectividad en el manejo del lupus eritematoso sistémico (LES). Sin embargo, se desconoce su efectividad en una población tan variable étnicamente como la colombiana.

El compromiso gastrointestinal en pacientes con lupus eritematoso sistémico (LES) es muy diverso. Su frecuencia y ubicación a lo largo del tracto digestivo varían ampliamente. Los procesos inflamatorios secundarios a los depósitos de complejos inmunes o eventos vasculares pueden ser los causantes de este compromiso. Una de las manifestaciones gastrointestinales características en los pacientes con LES es la pseudoobstrucción intestinal, que se define como la propulsión intestinal ineficaz que se produce en ausencia de factores mecánicos u obstructivos. Esta es, sin embargo, una complicación rara y poco entendida del LES. En este artículo, reportamos el caso de un paciente masculino con diagnóstico de LES y pseudoobstrucción intestinal, que fue tratado exitosamente con esteroides e inmunoglobulinas intravenosas. Se presenta una revisión completa de la literatura y una propuesta de la fisiopatología de la manifestación.

Gastrointestinal involvement in patients with systemic lupus erythematosus (SLE) is very diverse, and the frequency of occurrence and location along the digestive tract varies widely. Inflammatory processes secondary to immune complex deposits or vascular events may cause this involvement. One of the most characteristic gastrointestinal manifestations in these patients is the intestinal pseudo-obstruction, which is defined as the ineffective intestinal propulsion that occurs in the absence of mechanical or obstructive factors. This is, however, a rare and poorly understood complication of SLE. The case is presented of a male SLE patient presenting with intestinal pseudo-obstruction, and was successfully treated with steroids and intravenous immunoglobulin. A complete review of the literature and a proposal for the pathophysiology of intestinal pseudo-obstruction are presented.

The leading complication of antiphospholipid syndrome (APS) is arterial and venous thrombotic diathesis, which usually manifests as deep vein thrombosis or cerebrovascular events. However, it can occur in any blood vessel, rarely including the adrenal vessels. This can be the first manifestation of APS, and it is frequently bilateral and triggers adrenal insufficiency (Addison’s disease). It has been proposed that the initial phenomenon is thrombosis of the adrenal veins leading to infarction. Bilateral involvement is usually irreversible and requires permanent hormone replacement therapy. Unilateral involvement is even more infrequent and appears to have a better prognosis. We report the case of a 35-year-old female patient with a history of APS (repeated spontaneous abortions, livedo reticularis, presence of antiphospholipid antibodies and lupus anticoagulant), who presented with right adrenal infarction during the puerperium and whose clinical manifestation was sudden and severe lumbar pain. The diagnosis was made with contrast enhanced tomography. The patient made satisfactory progress, with no evidence of adrenal insufficiency.

El complejo BAFF (factor activador de células B) compuesto por la citocina BAFF, APRIL y sus receptores —BAFF-R (BR3), TACI y BCMA— influyen en la sobrevida periférica, en la maduración de los linfocitos B y en el cambio de clase de las inmunoglobulinas, con múltiples implicaciones clínicas potenciales. Las funciones biológicas de BAFF y su relevancia en varios desórdenes clínicos —autoinmunes, neoplásicos, infecciosos, incluyendo las terapias BAFF dirigidas— son revisadas y discutidas en el presente artículo. Los niveles séricos de BAFF/APRIL se encuentran incrementados en las enfermedades autoinmunes en las que sus concentraciones se relacionan con los títulos de anticuerpos, actividad, progresión de la enfermedad e incluso compromiso orgánico, haciendo de su inhibición un blanco terapéutico atractivo.

The aim of this study was to describe the efficacy and safety of anti–interleukin 6 receptor antibody (tocilizumab TCZ) in patients with severe or refractory Takayasu arteritis (TA).

Bariatric procedures are an effective option for weight loss and control of comorbidities in obese patients. Obesity is a proinflammatory condition in which some cytokines such as leptin, a proinflammatory protein, is elevated and adiponectin, an anti-inflammatory protein, is decreased. In patients undergoing weight reduction surgeries, these hormone levels behave paradoxically. It is not known whether bariatric surgery protects against development of autoinflammatory or autoimmune conditions; nevertheless, changes occurring in the immune system are incompletely understood. In this case series, we describe 4 patients undergoing bariatric surgery, who subsequently developed systemic autoimmune diseases. Patients in our case series were asymptomatic before surgery and developed an autoimmune disease within 11.2 months. Two women fulfilled criteria for systemic lupus erythematosus (one associated with antiphospholipid syndrome), and 2 men developed rheumatoid arthritis. A causal relationship is difficult to establish because factors that could trigger these diseases are multiple, including genetic susceptibility, time elapsed until achievement of ideal weight, and vitamin deficiencies, among others. However, clinicians must be attentive to this possible association.

OBJECTIVE: Considering the significant morbidity and mortality of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) and the lack of precise information on disease in Latin America, we investigated the clinical and laboratory characteristics associated with PAH in Colombian patients with SSc and review the literature. METHODS: This multicenter study included patients followed at 5 rheumatology units that were systematically assessed using a pretested questionnaire on clinical and immunological variables, focusing on PAH. Conditional logistic regression was employed to assess association between PAH and specific clinical characteristics. A systematic review of the literature was performed through electronic databases. RESULTS: Of a total of 349 patients with SSc, 61 (17%) met the criteria for PAH. Pulmonary fibrosis [adjusted odds ratio (AOR) 7.37, 95% CI 3.67-14.81, p < 0.0001], microstomia (AOR 3.3, 95% CI 1.70-6.28, p < 0.0001), gastroesophageal reflux (AOR 2.41, 95% CI 1.31-4.43, p = 0.005), dysphagia (AOR 2.7, 95% CI 1.49-4.77, p = 0.001), hyperpigmentation (AOR 2.15, 95% CI 1.11-4.16, p = 0.02), and hypopigmentation (AOR 2.4, 95% CI 1.26-4.64, p = 0.008) were the most prevalent clinical characteristics associated with PAH, while anemia (AOR 5.4, 95% CI 1.98-14.93, p = 0.001) was observed as the unique laboratory risk factor. Association between subtypes of SSc and PAH was not observed. Significant differences in both clinical and laboratory data were observed among different series. CONCLUSION: PAH may be a frequent complication of SSc in the Colombian population regardless of disease subtype. The identified clinical and laboratory risk factors might assist earlier diagnosis and guide decisions on therapeutic interventions on this critical complication of SSc. The reasons underlying the reported divergences among patients from different ethnicities are not fully understood, but it is most likely that both genetic and environmental factors are responsible for them.

Panniculitis is caused by a heterogeneous group of clinical entities that tend to manifest themselves as erythematous-violaceous subcutaneous nodules on the legs. In addition to associations with rheumatic diseases including vasculitis, its causes may include various infections. The microorganisms most commonly involved are bacteria and fungi. Among the latter, Candida Sp,, Nocardia Sp., Sporotrichosis, cryptococcosis, histoplasmosis, cromomycosis, and inter alia, have been described. We present an immu-nocompetent patient with a persistent, neutrophilic, chronic septal panniculitis caused by Aureobasidium Pullulans, an infection-generating fungus belonging to the phaeohyphornycosis group. The patient was treated with amphotericin B 0.5 mg/kg/day, itraconazole 400 mg/day p.o., and 5 fluo-rocytosine 150 mg/kg/day for 4 weeks with excellent response.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with various clinical manifestations, including, rarely, a form of interstitial cystitis (lupus cystitis, LC). LC can be asymptomatic and usually has discrete symptoms that improve with conventional therapies available for SLE and/or typical interstitial cystitis. A very severe and refractory form is rarely described. In this study, we present four patients with SLE and a very severe form of noninfectious cystitis refractory to the different forms of treatment described. The clinical descriptions of the cases, demographic factors, manifestations associated with SLE, and clinical and paraclinical manifestations related to cystitis, treatments, and outcomes are provided. A proposal for the pathogenesis of this condition is based on the common findings of these patients, including the fact that three were in SLE remission and all four receiving rituximab as induction and/or maintenance therapy.

Diabetic muscle infarction is a rare complication of diabetes. We describe 2 cases of diabetic muscle infarction, each one of them with a particular form of clinical presentation: recurrence, bilateral engagement, and unilateral compromise. Both cases had history of poorly controlled diabetes mellitus and diabetic nephropathy. The diagnosis was based on clinical, imaging, and anatomopathological features. The treatment was with a close control of diabetes mellitus, analgesics, short-term immobilization, and physical therapy.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with high morbidity if untreated. Sometimes, despite aggressive treatments, the disease remains active with cumulative organic damage. We conducted a retrospective and descriptive observational study of patients with SLE refractory to conventional treatment who were treated with rituximab (RTX) as remission induction therapy and maintenance. There was a significant reduction in the conventional immunosuppressive drug dose and the number of relapses of disease. RTX appeared to be effective and safe for the induction and maintenance of remission in patient with SLE refractory to conventional treatment.

In the recent years, it has been of great interest to study the binding mechanism between the innate and adaptive immune responses as interrelated processes for the development of multiple autoimmune diseases. Infection has been a well-known trigger of autoimmunity and trauma has been related as well too. Cryptogenic antigens release, recognition of pathogenic structure, and metabolic changes generated by both stimuli begin an inflammatory process which in turn activates the immune system amplifying T and B cell responses. The development of relapsing polychondritis after trauma may have a direct association with these events and in turn probably trigger autoimmune phenomena.

It is an inherent part of living to be in constant modification, which are due to answers resulting from environmental changes. The different systems make adaptations based on natural selection. With respect to the immune system of mammals, these changes have a lot to do with the interactions that occur continuously with other living species, especially microorganisms. The immune system is primarily designed to defend from germs and this response triggers inflammatory reactions which must be regulated in order not to generate damage to healthy tissue. The regulatory processes were added over time to prevent such damage. Through evolution the species have stored “an immunological experience,” which provides information that is important for developing effective responses in the future. The human species, which is at a high level of evolutionary immunological accumulation, have multiple immune defense strategies which, in turn, are highly regulated. Imbalances in these can result in autoimmunity.