Clinical application of dense fine spot anti-nuclear antibody in children with autoimmune diseases / 中华实用儿科临床杂志

Objective:To analyze the differences of the clinical characteristics and laboratory indexes in children with positive dense fine spot (DFS) type anti-nuclear antibody, and thereby to explore the value of positive DFS in the diagnosis of immunological diseases.Methods:Among 9 613 cases who were routinely tested for antinuclear antibody (ANA) from August 2017 to February 2020, there were 197 cases with DFS positive, who were subjected to a retrospective analysis.These patients were divided into the autoimmune diseases (AID) group (39 cases) and the non-AID group (158 cases) according to clinical diagnosis.Healthy children in the same physical examination were used as healthy control group (40 cases). T test was applied to analyze the differences of humoral immunity markers between AID and non-AID groups.What′s more, DFS positive patients in different clinical departments, initial symptom and the part of body were further compared. Results:Among 9 613 children tested for autoantibodies, 2 654 (27.61%) were ANA positive, with the highest detection rate of the spotted type and 197 DFS positive cases, accoun-ting for 7.42% of ANA positive children; 97 DFS positive male patients accounted for 8.20% (97/1 183 case) of ANA positive male patients, 100 DFS positive female patients accounted for 6.80% (100/1 471 cases) of ANA positive female patients, and there was no significant difference in the positive rate.The departments with high positive ANA detection included the nephrology department (27.88%) and the rheumatology department (24.83%). The departments with a higher ANA positive rate in DFS positive children included the gastroenterology department (13.25%) and the infectious department (11.76%). Among the children with DFS antibody positive, 39 cases had AID, among which 38 cases had organ-specific AID, and juvenile idiopathic arthritis (JIA) had the highest detection rate in 13 cases.The diseases with a high DFS positive rate in 158 non-AID cases included allergic purpura (46 cases). Serum immunoglobulin (IgG) level in the AID group was significantly lower than this in the non-AID group, serum IgM and C 4 levels in AID children were significantly lower than those in the non-AID group and healthy control group, and the serum IgA level of DFS positive group was significantly higher than that of children in the healthy control group.All children with DFS antibody positive had no specific autoantibodies. Conclusions:DFS antibody positive is important for the diagnosis of systemic AID in children.The combined detection with the DFS, other autoimmunity antibody index, humoral immune function index contributes to the early differential diagnosis of autoimmune diseases in children.

Enfermedades del tejido conectivo y sus cambios morfoestructurales / Morphological changes in connective tissue diseases

Introducción: Las enfermedades del tejido conectivo constituyen un problema de salud para las instituciones sanitarias de cualquier país, no solo por los síntomas que las acompañan, lo cual resulta motivo de atención médica frecuente, sino por la elevada carga asistencial, los elevados costos sanitarios y las afectaciones a la calidad de vida que genera en las personas que las padecen. Objetivo: Reflexionar sobre algunas enfermedades del tejido conectivo y sus complejidades morfológicas estructurales. Desarrollo: En la literatura consultada pudo constatarse la existencia de un grupo de enfermedades autoinmunes de tipo sistémico; tales como la artritis reumatoide, el lupus eritematoso sistémico, la esclerosis sistémica, la dermatomiositis, las miopatías inflamatorias, el síndrome antifosfolípido, el síndrome de Sjögren y las vasculitis necrotizantes sistémicas, que llaman la atención de los especialistas. Conclusiones: La mayoría de estas afecciones evolucionan con dolor, molestias y limitaciones para desarrollar las actividades de la vida cotidiana por lo que los pacientes suelen expresar sufrimiento, en particular, porque son enfermedades progresivas que pueden producir daños irreversibles con pronóstico no siempre favorable y cuya respuesta al tratamiento específico no siempre es satisfactoria, lo que acentúa el malestar y los síntomas en general y dan lugar a angustia y sufrimiento espiritual al paciente y sus familiares(AU)

Introduction: Connective tissue diseases are a health concern for healthcare institutions in any country, not only because of the symptoms they present, which is a reason for frequent medical attention, but also because of the need for care, high health costs and the great negative effects on the quality of life generated in the lives of people who suffer from them. Objective: To reflect about some connective tissue diseases and their structural and morphological complications. Development: During the analysis of the consulted literature, we could verify the existence of a group of systemic autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, dermatomyositis, inflammatory myopathies, antiphospholipid syndrome, Sjögren's syndrome and systemic necrotizing vasculitis, which has drawn the specialists' attention. Conclusions: Most of these conditions develop with pain, discomfort and limitations to perform activities of daily life, a reason why patients often feel distressed, particularly because these are diseases that can cause irreversible damage with a not always favorable prognosis and whose response to specific treatment is not always satisfactory, which accentuates the discomfort and symptoms in general and gives rise to spiritual anguish and suffering in the patient and their relatives(AU)

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.

Comparison of Clinical Manifestations between Patients with Ocular Myasthenia Gravis and Generalized Myasthenia Gravis

PURPOSE: To compare the clinical manifestations between patients with ocular myasthenia gravis and those with generalized myasthenia gravis (MG). METHODS: The medical records of 71 patients diagnosed with MG between January 1995 and December 2007 were reviewed. Demographics, sensitivities of diagnostic methods, the presence of systemic autoimmune diseases, ophthalmic complications caused by MG, and treatments were evaluated and compared. RESULTS: Fourteen patients (20%) were diagnosed with ocular MG and 57 patients (80%) with generalized MG. Sensitivities of anti-acetylcholine receptor antibody and repetitive nerve stimulation tests were significantly higher in the generalized MG group (84%, 89%) compared to those in the ocular MG group (50%, 54%) (p = 0.011, p = 0.008). The sensitivity of the neostigmine test was the highest in both groups (98% of generalized MG, 79% of ocular MG), and the difference between the two groups was borderline significant (p = 0.058). The most common symptoms were ptosis and diplopia, and both groups presented with pain, blurred vision, and tearing. Systemic autoimmune disease was more prominent in the generalized MG group (21%) than in the ocular MG group (14%), and steroid therapy was used more frequently in the generalized MG group (82%) than in the ocular MG group (57%). Ophthalmic complications associated with long-term steroid treatment were more profound in the generalized MG (30%) compared to those of the ocular MG (21%). CONCLUSIONS: The generalized MG group was associated with higher sensitivities to diagnostic tests, more systemic steroid use, higher ophthalmic complications caused by systemic autoimmune disease, and long-term steroid treatment compared to those of the ocular MG group.