Association of Smoking and Obesity on the Risk of Developing Primary Sjögren Syndrome: A Population-based Cohort Study.

OBJECTIVE: To explore the role of smoking and obesity in primary Sjögren syndrome (pSS). METHODS: Olmsted County (Minnesota, USA) residents (n = 106) diagnosed with pSS from 2000 to 2015 were compared to 3 controls without pSS and matched for age and sex who were randomly selected from Olmsted County residents. RESULTS: Current smokers were less likely to be pSS cases (OR 0.34, 95% CI 0.14-0.85), while there was no association between former smoking and case/control status (OR 1.27, 95% CI 0.80-2.03) compared to never smokers. Smoking status was not associated with antinuclear antibody, anti-SSA, anti-SSB, or rheumatoid factor positivity (p > 0.05). OR for obesity was 0.79 (95% CI 0.48-1.30). CONCLUSION: In this population-based study, current smoking was inversely associated with case/control status, while body mass index lacked any association.

Vasculitis por IgA del adulto. Caso clínico

La vasculitis por IgA, es la vasculitis más frecuente en pediatría. Puede presentarse en adultos, con una clínica y evolución diferente y un pronóstico más grave que en los niños, incluida la progresión a enfermedad renal terminal. La historia natural de la enfermedad y de la nefritis, ha sido poco estudiada en adultos; no se dispone de criterios diagnósticos universalmente aceptados y el tratamiento es controvertido, dada la ausencia de estudios controlados, randomizados que lo avalen. Se reporta el caso de un paciente que presentó un síndrome purpúrico petequial, microhematuria, proteinuria y una evolución rápida a la insuficiencia renal, de cuyo estudio etiológico surge el diagnóstico de vasculitis por IgA del adulto.

The IgA vasculitis is the most common vasculitis in Pediatrics. It can also present in adults but with a different clinical course and a worse prognosis, including the possibility of progression to end stage renal disease. The natural history of the disease and its nephritis have been scarcely studied in adults. There is no universal agreement in diagnostic criteria and the treatment is controversial given the absence of controlled randomized trials. We report the case of a patient who presented clinically with a petechial purpuric rash, microhematuria, proteinuria and rapid progression to renal failure that was diagnosed with IgA vasculitis in adult.

A vasculite por IgA é a vasculite mais comum em pediatria. Pode ocorrer em adultos, com apresentação e evolução clínica diferentes e prognóstico mais grave do que em crianças, incluindo progressão para doença renal terminal. A história natural da doença e da nefrite tem sido pouco estudada em adultos; Não existem critérios diagnósticos universalmente aceitos e o tratamento é controverso, dada a ausência de estudos controlados e randomizados que o apoiem. É relatado o caso de um paciente que apresentou síndrome purpúrica petequial, microhematúria, proteinúria e rápida evolução para insuficiência renal, de cujo estudo etiológico surge o diagnóstico de vasculite por IgA do adulto.

Hospitalisation rates among patients with primary Sjögren's syndrome: a population-based study, 1995-2016.

OBJECTIVE: To determine rates and primary discharge diagnoses of hospitalisation in a cohort of patients with incident primary Sjögren's syndrome (pSS) compared with the general population. METHODS: This was a retrospective population-based cohort study focused on Olmsted County, Minnesota. The pSS cohort consisted of patients with incident pSS in the 1976-2015 period and was compared with a cohort of individuals without pSS matched 3:1 for age, sex and calendar year, randomly selected from the same population. Hospitalisations in 1995-2016 were examined. Discharge diagnoses were categorised using the Clinical Classifications Software for International Classification of Diseases, 9th revision, Clinical Modification. RESULTS: A total of 385 hospitalisations occurred in the 160 patients with pSS during 1592 person-years of follow-up. Among 466 comparators, there were 899 hospitalisations during 4660 person-years of follow-up, resulting in a significantly higher rate of hospitalisations in patients with pSS (rate ratio (RR): 1.25, 95% CI: 1.11 to 1.41). Rates of hospitalisation were increased among patients with pSS for endocrine, nutritional and metabolic diseases and immunity disorders (RR 1.82, 95% CI 1.08 to 2.98), diseases of the musculoskeletal system and connective tissue (RR 1.49, 95% CI 1.05 to 2.05) and for injuries and poisoning (RR 1.46, 95% CI 1.01 to 2.06). While not significantly increased overall, hospitalisations for diseases of the circulatory system were significantly increased in patients with pSS aged ≥75 years (RR 1.54, 95% CI 1.11 to 2.11). CONCLUSIONS: Patients with pSS experienced higher rates of hospitalisation than the general population. Hospitalisations for endocrine/metabolic disorders, diseases of the circulatory system, diseases of the musculoskeletal system and connective tissue disorders, and injuries were more common among patients with pSS than comparators.

Citopenias hematológicas en enfermedades autoinmunes sistémicas / Blood cytopenias in systemic autoimmune conditions

Las citopenias hematológicas son un hallazgo frecuente y potencialmente grave en las Enfermedades Autoinmunes Sistémicas. Sus causas pueden ser muy variadas, de ahí la importancia de un abordaje diagnóstico sistematizado que asegure además el tratamiento correcto. En este artículo se revisan las generalidades de las citopenias hematológicas en cuanto a frecuencia, causas, así como su enfoque diagnóstico y terapéutico.

The hematologic cytopenias are a frequent and potentially dangerous finding in the Systemic Autoimmune Diseases. It may have different etiologies, and for that reason it is important a systematic approach to ensure the correct diagnosis and treatment. In this article, the frequency, etiology, diagnostic approach and treatment of the hematologic cytopenias are reviewed.

Prevalence and characteristics of immunomediated neuropathies in a group of patients with autoimmune diseases.

OBJECTIVE: To determine the prevalence and the clinical-neurophysiological characteristics of immunomediated peripheral neuropathies (PN) in a group of patients with systemic autoimmune diseases. METHOD: Fifty-nine patients with proved systemic autoimmune diseases were included. Patients underwent clinical examination and nerve conduction studies to diagnose the PN. RESULTS: Immune PNs were detected in 18 patients (30.5%). Out of the total number of PNs (18), 39% were sensory-motor polyneuropathies, 33% mononeuritis multiplex, 11% pure sensory polyneuropathies, 11% cranial neuropathies, and 6% proximal motor neuropathies, such as the Guillain-Barré syndrome. Nine PNs (50%) appeared at the onset of the connective tissue disorders, and the rest of the cases appeared during the course of the disease. Of the total of PNs detected in this study, only 45% had a previous diagnosis. Vasculitis was the disease that presented more associated PNs. Systemic lupus erythematosus showed the widest range of PN clinical varieties. CONCLUSIONS: The first national prevalence rate of PNs in patients with systemic autoimmune diseases was provided: 30.5%. No comparative data were found in the international bibliography. Sensory-motor polyneuropathy was the most frequently observed form of PN, followed by mononeuritis multiplex. The NPs appeared with the same frequency both at the onset and during the course of the diseases under study; these predominated at the onset of vasculitis and primary Sjögren syndrome. The compromise of the peripheral nervous system is underdiagnosed.

Prevalencia de cefaleas primarias y subtipos en un grupo de pacientes con enfermedades autoinmunes sistémicas / Prevalence of primary headaches and subtypes in a group of patients with systemic autoimmune diseases

Objetivos: Conocer prevalencia global de cefalea y subtipos en Enfermedades Autoinmunes Sistémicas (EAS) y compararla con la población general. Establecer en pacientes con Lupus Eritematoso Sistémico (LES) la relación entre cefalea y anticuerpos antifosfolípidos (aPL), actividad, otras manifestaciones neurológicas. Comparar la eficacia entre la segunda clasificación de la International Headache Society (ICHD-II), 2004, y la del American College of Rheumatology (ACR), 1999, para clasificar cefaleas en LES. Métodos: Noventa pacientes fueron evaluados con un cuestionario basado en criterios de la ICHD-II. En pacientes con LES también se aplicó la clasificación de cefaleas del ACR. Prevalencia anual de cefalea y migraña se comparó con la de Uruguay. Resultados: La prevalencia anual de cefalea en EAS fue similar a la población general (50,6 por ciento vs 58,4 por ciento). Fueron primarias 47 (52 por ciento): Tipo tensional 31 (34,4 por ciento), migraña 16 (17,8 por ciento), sin aura 13. Se halló asociación significativa entre EAS y migraña, teniendo 2,87 más posibilidades de presentarla estos individuos que la población general (17,8 por ciento vs 6,2 por ciento, P = 0,0001). De 19 lúpicos con cefalea, relación con actividad: 1, aPL positivos: 13, otras manifestaciones neurológicas: 5. Aplicando la ICHD-II se diagnosticó el 100 por ciento de cefaleas en LES; con la clasificación del ACR se diagnosticó el 86 por ciento. Conclusión: La prevalencia de cefalea fue similar ala población general. Predominaron las primarias, tipo tensional y migraña. La migraña fue más prevalente en las EAS. En LES no se demostró asociación entre cefalea y actividad, aPL, otros síndromes neuropsiquiátricos. La ICHD-II debería utilizarse en LES, y la clasificación del ACR debería revisarse.

Objectives: Learn the global prevalence of headache and its subtypes in Systemic Autoimmune Diseases (SADs); compare it with the general population. In Systemic lupus erythematosus (SLE), to establish the relationship between headache and: antiphospholipid antibodies (APA), activity, other neurological manifestations. To compare the efficacy between the second classification of the International Headache Society (ICHD-II), 2004 and the classification of the American College of Rheumatology (ACR) 1999 to classify headaches in SLE. Methods: Ninety patients were evaluated with a questionnaire based on criteria of the ICHD-II. In patients with SLE the criteria of the ACR were also used. The annual prevalence of headache and migraine was compared with Uruguay’s. Results. The annual prevalence of headache in SADs was similar to the general population (50.6 percent vs. 58.4 percent). There were 47 primary headaches (52 percent): 31 tension type (34,4 percent), 16 migraines (17,8 percent), 13 without aura. A significant association was found between SADs and migraine; these patients had a 2.86 times greater chance of presenting migraines than the general population (17.8 percent vs. 6.2 percent, P = 0,0001). In the case of the 19 patients with SLE and headache, there was a relationship with activity in 1 case, 13 were APA positive, 5 had other neurologic manifestations. Applying the ICHD-II criteria 100 percent of headaches were diagnosed in SLE, applying those of the ACR, 86 percent. Conclusion: The primary headaches predominated, with the same prevalence and subtypes (tension type and migraine) than in the general population. Individuals with SADs have a higher possibility of suffering from migraine. In SLE, no relationship was demonstrated between headache and activity, APA or other neuropsychiatric syndromes. The ICHD-II should be the one to be used in SLE patients, the ACR classification should be revised.

Leptospirosis con compromiso respiratorio predominante. Presentación de cinco casos clínicos / Leptospirose com comprometimento respiratório. Apresentação de cinco casos clínico

La leptospirosis es una infección generalizada aguda reemergente en Uruguay y en el Cono Sur. El compromiso respiratorio es frecuente pero generalmente leve y subdiagnosticado. Es inhabitual como inicio de la enfermedad. En los últimos años se ha informado un cambio en la presentación clínica, con mayor incidencia de formas graves anictéricas, sin disfunción renal y con compromiso pulmonar predominante. Se describen cinco casos clínicos de leptospirosis "primariamente" pulmonares, asistidos en el Hospital Maciel en el año 2005. Se trata de hombres jóvenes, con ocupaciones de riesgo y contacto con roedores. Clínicamente se presentan con un cuadro respiratorio de evolución aguda con fiebre, tos con expectoración hemoptoica e insuficiencia respiratoria (PO2 promedial de 70 mmHg, SaO2 94p/ciento), destacándose como síntomas extrapulmonares: mialgias intensas, cefaleas, escalofríos, dolor ocular e hiperemia conjuntival. El examen pleuropulmonar puede ser normal o presentar signología bilateral y difusa. La mayoría de estos casos cursan sin ictericia y ningún paciente presenta insuficiencia renal grave. Radiológicamente predomina el patrón alveolar bilateral y difuso compatible con hemorragia pulmonar. El diagnóstico de leptospirosis se basa en el alto índice de sospecha y contexto clínico-epidemiológico compatible y se confirma con pruebas serológicas. En todos los paciente se realizó tratamiento de soporte de la función respiratoria y antibioticoterapia empírica precoz con antibióticos betalactámicos, con buena evolución clínico-radiológica.