Lupus enteritis: A 10-year experience in a single Latin American center.

OBJECTIVE: The objective is to compare the clinical and laboratory characteristics of systemic lupus erythematosus (SLE) patients with and without lupus enteritis (LE) and to identify the factors associated with the occurrence of LE. METHODS: We performed a retrospective, case-control study in hospitalized patients with SLE who were admitted to our tertiary hospital between January 2012 and December 2021. Sixteen LE patients (cases) were matched (1:3 ratio) for sex and birth year with 48 non-LE patients (controls). Univariable and multivariable logistic regression analyses were used to identify the variables associated with LE. RESULTS: Of 2,479 SLE patients who were admitted to our hospital as inpatients, 16 (0.65%) were diagnosed as having LE. All patients, cases and controls, were of Mestizo ethnicity. SLE was diagnosed simultaneously with the first episode of LE in 10 (62.5%) patients. The median time from SLE diagnosis to the first episode of LE was 7 (IQR 0-78) months. LE patients had a shorter median disease duration [7 (0-78) vs 34 (9.5-79) months], and a significantly longer hospital stay (28.3 ± 15.8 vs 6.5 ± 7.9 days, p < 0.001) than non-LE patients. Most LE patients (93.8%) had concomitant lupus nephritis. LE patients had higher SLEDAI-2K scores than those without LE (20.5 ± 9.4 vs 9.8 ± 10.4, p < 0.001). By multivariable analysis, a higher SLEDAI-2K score (OR 1.10, 95% CI 1.02-1.18; p = 0.015) was independently associated with LE occurrence after adjusting for cutaneous involvement, lymphocyte count, serum creatinine, and serum complement C4. Recurrence was observed in two patients (12.5%), both with a bowel wall thickening > 8 mm. The two patients with large intestine-dominant LE developed intestinal pseudo-obstruction. No patient had life-threatening complications (intestinal hemorrhage, infarction, or perforation), and there were no deaths induced directly by LE itself. CONCLUSION: In patients of Mestizo ethnicity, LE occurs during the early course of SLE, frequently is one of the presenting manifestations of SLE, and in most cases, it presents with concomitant lupus nephritis. Higher levels of disease activity at diagnosis were independently associated with LE occurrence and when recurrences occur, they do so in the context of severe wall thickness.

Cardiac involvement in anti-MDA5 dermatomyositis: a case-based review.

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.

Mitochondria as a key player in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a heterogeneous, multisystemic autoimmune disease with a broad clinical spectrum. Loss of self-tolerance and chronic inflammation are critical markers of SLE pathogenesis. Although alterations in adaptive immunity are widely recognized, increasing reports indicate the role of mitochondrial dysfunction in activating pathogenic pathways involving the innate immune system. Among these, disarrangements in mitochondrial DNA copy number and heteroplasmy percentage are related to SLE activity. Furthermore, increased oxidative stress contributes to post-translational changes in different molecules (proteins, nucleic acids, and lipids), release of oxidized mitochondrial DNA through a pore of voltage-dependent anion channel oligomers, and spontaneous mitochondrial antiviral signaling protein oligomerization. Finally, a reduction in mitophagy, apoptosis induction, and NETosis has been reported in SLE. Most of these pathways lead to persistent and inappropriate exposure to oxidized mitochondrial DNA, which can stimulate plasmacytoid dendritic cells, enhance autoreactive lymphocyte activation, and release increased amounts of interferons through stimulation of toll-like receptors and cytosolic DNA sensors. Likewise, abnormal T-cell receptor activation, decreased regulatory T cells, enhanced Th17 phenotypes, and increased monocyte maturation to dendritic cells have also been observed in SLE. Targeting the players involved in mitochondrial damage can ultimately help.

T cell metabolism and possible therapeutic targets in systemic lupus erythematosus: a narrative review.

In the immunopathogenesis of systemic lupus erythematosus (SLE), there is a dysregulation of specific immune cells, including T cells. The metabolic reprogramming in T cells causes different effects. Metabolic programs are critical checkpoints in immune responses and are involved in the etiology of autoimmune disease. For instance, resting lymphocytes generate energy through oxidative phosphorylation (OXPHOS) and fatty acid oxidation (FAO), whereas activated lymphocytes rapidly shift to the glycolytic pathway. Specifically, mitochondrial dysfunction, oxidative stress, abnormal metabolism (including glucose, lipid, and amino acid metabolism), and mTOR signaling are hallmarks of T lymphocyte metabolic dysfunction in SLE. Herein it is summarized how metabolic defects contribute to T cell responses in SLE, and some epigenetic alterations involved in the disease. Finally, it is shown how metabolic defects could be modified therapeutically.

Factors associated with lethality from pneumonia in an acute care for the elderly unit: a retrospective cohort / [Factores asociados con la letalidad por neumonía en una unidad de atención del paciente geriátrico agudo: una cohorte retrospectiva].

Introduction: Pneumonia is the leading cause of death due to infection in the world, mainly affecting the older adult population. Objective: To determine the risk factors associated with lethality at 30 days in patients with pneumonia in an acute care for the elderly unit. Materials and methods: We conducted an observational, analytical retrospective cohort study including 114 patients aged 60 years and older hospitalized in an acute care geriatric unit with a diagnosis of pneumonia. The primary outcome was lethality at 30 days. Bivariate and multivariable log-binomial regression analyzes were performed to explore the relationship between independent variables and the primary outcome. Results: The 30-day lethality was 26.3% and the mean age was 84.45 ± 7.37 years; 54.4% of participants were men. In the multivariable analysis, age ≥90 years (Relative Risk, RR=1.62, 95% CI: 1.05-2.68, p=0.04), multilobar commitment (RR=1.92, 95% CI:1.12-3.32, p=0.02), elevated urea nitrogen (≥22.5 the median; RR=3.93, 95% CI:1.67-9.25, p<0.01), and a score of zero in the Lawton index at admission (RR=3.20, 95% CI:1.05-9.78, p=0.04) were independent predictors of 30-day lethality from pneumonia. Conclusion: In older adults hospitalized for pneumonia in an acute care geriatric unit, advanced age, the presence of multilobar commitment, dependency in functional status, and elevated ureic nitrogen levels were the main predictors of short-term lethality risk.

Introducción. La neumonía es la principal causa de muerte por infección en el mundo y afecta principalmente a la población de adultos mayores. Objetivo. Determinar los factores de riesgo asociados con la letalidad a 30 días en pacientes con neumonía en una unidad de atención del paciente geriátrico agudo. Materiales y métodos. Se hizo un estudio observacional y analítico de cohorte retrospectiva. Se incluyeron 114 pacientes de 60 años o más hospitalizados en una unidad de atención del paciente geriátrico agudo con diagnóstico de neumonía. El resultado primario fue la letalidad a 30 días. Se hicieron análisis de regresión log-binomial bivariado y multivariable para explorar la relación entre las variables independientes y el resultado primario. Resultados. La letalidad a 30 días fue de 26,3 % y la edad media de 84,45 ± 7,37 años. El 54,4 % de los participantes era de sexo masculino. En el análisis multivariable se encontró que una edad de 90 años o más (riesgo relativo, RR=1,62; IC95%: 1,05-2,68; p=0,04), el compromiso multilobar (RR=1,92; IC95%: 1,12-3,32; p=0,02), el nitrógeno ureico elevado (mediana≥22,5; RR=3,93; IC95%: 1,67-9,25; p<0,01), y un puntaje de cero en la escala de Lawton al ingreso (RR=3,20; IC95% 1,05-9,78; p=0,04) fueron factores predictores independientes de la letalidad a 30 días en adultos mayores con neumonía. Conclusión. En los pacientes adultos mayores hospitalizados por neumonía en una unidad de atención del paciente geriátrico agudo la edad muy avanzada, la presencia de compromiso multilobar, la dependencia en el estado funcional y los niveles de nitrógeno ureico elevados fueron los principales factores de riesgo de letalidad a corto plazo.

Factores asociados con la letalidad por neumonía en una unidad de atención del paciente geriátrico agudo: una cohorte retrospectiva / Factors associated with lethality from pneumonia in an acute care for the elderly unit: a retrospective cohort

Resumen: Introducción. La neumonía es la principal causa de muerte por infección en el mundo y afecta principalmente a la población de adultos mayores. Objetivo. Determinar los factores de riesgo asociados con la letalidad a 30 días en pacientes con neumonía en una unidad de atención del paciente geriátrico agudo. Materiales y métodos. Se hizo un estudio observacional y analítico de cohorte retrospectiva. Se incluyeron 114 pacientes de 60 años o más hospitalizados en una unidad de atención del paciente geriátrico agudo con diagnóstico de neumonía. El resultado primario fue la letalidad a 30 días. Se hicieron análisis de regresión log-binomial bivariado y multivariable para explorar la relación entre las variables independientes y el resultado primario. Resultados. La letalidad a 30 días fue de 26,3 % y la edad media de 84,45 ± 7,37 años. El 54,4 % de los participantes era de sexo masculino. En el análisis multivariable se encontró que una edad de 90 años o más (riesgo relativo, RR=1,62; IC95%: 1,05-2,68; p=0,04), el compromiso multilobar (RR=1,92; IC95%: 1,12-3,32; p=0,02), el nitrógeno ureico elevado (mediana≥22,5; RR=3,93; IC95%: 1,67-9,25; p<0,01), y un puntaje de cero en la escala de Lawton al ingreso (RR=3,20; IC95% 1,05-9,78; p=0,04) fueron factores predictores independientes de la letalidad a 30 días en adultos mayores con neumonía. Conclusión. En los pacientes adultos mayores hospitalizados por neumonía en una unidad de atención del paciente geriátrico agudo la edad muy avanzada, la presencia de compromiso multilobar, la dependencia en el estado funcional y los niveles de nitrógeno ureico elevados fueron los principales factores de riesgo de letalidad a corto plazo.

Abstract: Introduction: Pneumonia is the leading cause of death due to infection in the world, mainly affecting the older adult population. Objective: To determine the risk factors associated with lethality at 30 days in patients with pneumonia in an acute care for the elderly unit. Materials and methods: We conducted an observational, analytical retrospective cohort study including 114 patients aged 60 years and older hospitalized in an acute care geriatric unit with a diagnosis of pneumonia. The primary outcome was lethality at 30 days. Bivariate and multivariable log-binomial regression analyzes were performed to explore the relationship between independent variables and the primary outcome. Results: The 30-day lethality was 26.3% and the mean age was 84.45 ± 7.37 years; 54.4% of participants were men. In the multivariable analysis, age ≥90 years (Relative Risk, RR=1.62, 95% CI: 1.05-2.68, p=0.04), multilobar commitment (RR=1.92, 95% CI:1.12-3.32, p=0.02), elevated urea nitrogen (≥22.5 the median; RR=3.93, 95% CI:1.67-9.25, p<0.01), and a score of zero in the Lawton index at admission (RR=3.20, 95% CI:1.05-9.78, p=0.04) were independent predictors of 30-day lethality from pneumonia. Conclusion: In older adults hospitalized for pneumonia in an acute care geriatric unit, advanced age, the presence of multilobar commitment, dependency in functional status, and elevated ureic nitrogen levels were the main predictors of short-term lethality risk.

Características clínico-radiológicas de la hiperostosis esquelética idiopática difusa en 2 centros médicos de Cali, Colombia: reporte de 24 casos / Clinical and radiological characteristics of diffuse idiopathic skeletal hyperostosis in two medical centers in Cali, Colombia: Report of 24 cases

RESUMEN Introducción: La hiperostosis esquelética idiopática difusa (DISH, por sus siglas en inglés) es una afección caracterizada por la calcificación y la osificación progresiva de los ligamentos y las entesis. La mayoría de los pacientes permanecen asintomáticos hasta etapas avanzadas de la enfermedad, donde la limitación y el dolor son característicos. Objetivo: Describir las características demográficas, clínicas y radiológicas de los pacientes con DISH evaluados en el Centro Médico Imbanaco de Cali y en la Clínica de Artritis Temprana, en Cali, Colombia. Materiales y métodos: Es un estudio descriptivo, de corte transversal. Se revisaron los registros de pacientes diagnosticados con DISH, seguidos entre enero de 2000 y octubre de 2018. El diagnóstico se confirmó según los criterios de Resnick-Niwayama. Se encontraron 24 pacientes, todos se incluyeron para el análisis final. Resultados y discusión: En esta serie se encontraron 20 varones y 4 mujeres. La mediana de edad al diagnóstico fue de 70,5 arios (RIQ: 61,3-73,8 arios), siendo menor en las mujeres (71,5 versus 60 años; p = 0,04). La mediana de tiempo de evolución de los síntomas fue de 5 años (RIQ: 3-10 años), la duración fue menor en el grupo de las mujeres (5 versus 4 años; p = 0,20). El 54,2% tenían sobrepeso y el 20,8% eran diabéticos. El síntoma principal fue la limitación cervical. Los segmentos vertebrales (C: cervical; T: torácico; L: lumbar) más afectados por la osificación del ligamento longitudinal anterior (LLA) fueron C5-C6, T8-T10 y L1-L3. La afección periférica predominó en las crestas ilíacas. Todos los pacientes realizaron terapia física y 3 fueron sometidos a cirugía cervical. El grado de limitación funcional fue valorado en 19 pacientes a través de los cuestionarios modified Health Assessment Questionnaire (mHAQ) y Bath Ankylosing Spondylitis Functional Index (BASFI). La mediana del puntaje de ambos cuestionarios fue 2 veces más alta en las mujeres. Conclusión: La DISH fue más frecuente en varones mayores de 65 años y se asoció con enfermedades metabólicas como la obesidad y la diabetes. Aunque las diferencias no fueron significativas, los resultados sugieren que las mujeres presentan un fenotipo grave de la enfermedad explicado por el inicio temprano y curso progresivo de los síntomas, así como mayor limitación funcional medida por mHAQ y BASFI.

ABSTRACT Introduction: Diffuse idiopathic skeletal hyperostosis (DISH) is a condition characterised by calcification and progressive ossification of ligaments and entheses. Most patients remain asymptomatic until advanced stages of the disease, where limitation and pain are characteristic. Objective: To describe the demographic, clinical, and radiological characteristics of patients with DISH evaluated in the Centro Médico Imbanaco and Clínica de Artritis Temprana, in Cali, Colombia. Materials and methods: A descriptive, cross-sectional study was conducted by reviewing the records of patients diagnosed with DISH between January 2000 and October 2018. The diagnosis was confirmed according to the Resnick-Niwayama criteria. A total of 24 patients were found, and all were included for the final analysis. Results and discussion: The series included 20 men and 4 women, with a median age at diagnosis of 70.5 years (IQR 61.3-73.8 years), beinglower in women (71.5 versus 60 years; P=.04). The median time of onset of the symptoms was 5 years (IQR 3-10 years), and the duration was shorter in women (5 versus 4 years; P=.20). It was observed that 54% were overweight and 20% were diabetic. The main symptom was cervical limitation. The most affected vertebral segments due to the ossification of the anterior longitudinal ligament (ALL) were C5-C6, T8-T10 and L1-L3 (cervical C; thoracic T; lumbar L). The peripheral involvement was mainly in the iliac crests. All patients received physiotherapy, and three of them underwent cervical surgery. The degree of functional limitation was assessed in 19 patients using the mHAQ (Modified Health Assessment Questionnaire) and BASFI (Functional Ankylosing Spondylitis Functional (BASFI) questionnaires. The median score of both questionnaires was 2 times higher in women Conclusion: Diffuse idiopathic skeletal hyperostosis was more frequent in men over 65 years of age, and was associated with metabolic conditions such as obesity and diabetes. Although the differences were not significant, the results suggest that women have a more severe phenotype of the disease, explained by the early onset and progressive course of symptoms, as well as greater functional limitation measured by mHAQ and BASFI.

Restrictive cardiomyopathy in a patient with systemic sclerosis and Fabry disease: a case-based review.

Systemic sclerosis (SSc) is a rare immune-mediated vasculopathy characterized by fibrosis of the skin and internal organs. Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene producing α-galactosidase-A enzyme (α-Gal A) deficiency. Being a systemic disease, cardiac involvement in FD has a high mortality rate due to heart failure and arrhythmia. The coexistence of these two entities has not been reported previously. We describe the case of a female patient with limited SSc (lcSSc), a diagnosis based on the presence of sclerodactyly, Raynaud phenomenon, microvascular involvement, and positive anti-centromere antibodies. On follow-up, she developed chest pain, a second-degree A-V block, and restrictive cardiomyopathy (without cardiovascular risk factors). Although heart involvement is common in these two entities, the abnormal thickening of lateral and inferior wall, the infiltration pattern and the conduction system disorders presented herein are more characteristic in a heterozygous female with a cardiac variant of FD. The diagnosis of FD was confirmed with high globotriaosylsphingosine (Lyso-Gb3) levels and identification of GLA gene mutation. The patient was treated with enzymatic replacement (agalsidase alpha) following mild improvement in ventricular mass at 6th month, without clinical deterioration. The related literature on SSc associated with FD is also reviewed.