Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Los anticuerpos anti-Ro52/TRIM21 están asociados a circuitos inflamatorios aberrantes en pacientes con enfermedades reumáticas autoinmunes sistémicas / Anti-Ro52/TRIM21 antibodies are associated with aberrant inflammatory circuits in patients with systemic autoimmune rheumatic diseases

Resumen Introducción: Los anticuerpos anti-Ro52/TRIM21 son marcadores de varias enfermedades reumáticas autoinmunes sistémicas (ERAS). Objetivo: Evaluar si los anticuerpos anti-Ro52/TRIM21 están relacionados con anomalías en los circuitos inflamatorios. Métodos: Estudio transversal de pacientes consecutivos y ambulatorios con ERAS. Los anticuerpos anti-Ro52/TRIM21 y la proteína amiloide sérica se midieron mediante ELISA; los paneles para 18 citocinas y nueve quimiocinas se analizaron en una plataforma de lectura Luminex; la proteína C reactiva (hs-CRP) y el complemento se midieron mediante nefelometría. Resultados: Se incluyeron 167 pacientes, 143 con lupus eritematoso sistémico (LES), 16 con síndrome de Sjögren primario y ocho con esclerosis sistémica; 41 fueron positivos para anticuerpos anti-Ro52/TRIM21 (24 %). Los pacientes con anticuerpos anti-Ro52/TRIM21 tuvieron niveles séricos más altos de IL-2, IL-4, IL-6, GM-CSF, IL-21, IL-22, hs-CRP y quimiocinas CCL4, CXCL8, CXCL10 y CXCL12; y más bajos de complemento C4. Los títulos de anticuerpos anti-Ro52/TRIM21 correlacionaron positivamente con IL-2, IL-4, IL-6, IL-10, IL-21, IL-22, CXCL10 y hs-CRP; y negativamente con complemento C3 y C4. Al incluir solo LES, no se identificó asociación entre los anticuerpos anti-Ro52/TRIM21 y la actividad de la enfermedad o la afectación específica de órganos. Conclusiones: Los anticuerpos anti-Ro52/TRIM21 se asocian a circuitos aberrantes de citocinas y niveles elevados de moléculas angiogénicas y quimioatrayentes de neutrófilos y monocitos, lo que sugiere un papel activo de esos anticuerpos en las ERAS.

Abstract Introduction: Anti-Ro52/TRIM21 antibodies are markers for several systemic autoimmune rheumatic diseases (SARD). Objective: To assess whether anti-Ro52/TRIM21 antibodies are related to abnormalities in inflammatory circuits. Methods: Cross-sectional study of consecutive outpatients with SARD. Anti-Ro52/TRIM21 antibodies and serum amyloid A protein were measured by ELISA; panels for 18 cytokines and nine chemokines were analyzed on a Luminex reading platform, while high-sensitivity C-reactive protein (hs-CRP) and complement were measured by nephelometry. Results: Among 167 included patients, 143 had systemic lupus erythematosus (SLE), 16 had primary Sjögren's syndrome and eight had systemic sclerosis; 41 (24%) were positive for anti-Ro52/TRIM21 antibodies. Patients with anti-Ro52/TRIM21 antibodies had higher serum levels of IL-2, IL-4, IL-6, GM-CSF, IL-21, IL-22, hs-CRP and chemokines CCL4, CXCL8, CXCL10 and CXCL12, but lower levels of complement C4. Anti-Ro52/TRIM21 antibody titers were positively correlated with IL-2, IL-4, IL-6, IL-10, IL-21, IL-22, CXCL10, and hs-CRP, and negatively with complement C3 and C4. When only SLE patients were included, no association was identified between anti-Ro52/TRIM21 antibodies and disease activity or organ-specific involvement. Conclusions: Anti-Ro52/TRIM21 antibodies are associated with aberrant cytokine circuits and elevated levels of angiogenic molecules and neutrophil and monocyte chemoattractants, which suggests an active role for these antibodies in SARD.

Clinical characteristics of patients with antisynthetase syndrome and positive anti-Ro52 antibody / 中华内科杂志

To analyze the clinical characteristics of patients with antisynthetase syndrome (ASS) and positive anti-Ro52 antibody. The clinical data of 203 ASS patients admitted to the First Affiliated Hospital of Zhengzhou University from 2017 to 2020 were analyzed retrospectively. Demographics, clinical manifestations, laboratory results, treatment and outcome were collected including data of 18 patients with rapidly progressive interstitial lung disease (RP-ILD). In total, the majority were women (148,72.9%). The average onset age was (51.9±13.3) years. There were 163 (80.3%) patients with positive anti-Ro52 antibody. The positivity in women (77.3% vs. 55.0%, P=0.004) was higher, and the median time from disease onset to diagnosis [4.5 (2.0, 24.0) months vs. 2.0 (1.0, 12.0) months, P=0.024] was longer in patients with positive anti-Ro52 antibody than those negative. Compared with negative patients, patients with positive anti-Ro52 antibody had a higher incidence of interstitial lung disease (ILD) (96.9% vs. 65.0%, P<0.001), arthritis (33.7% vs. 17.5%, P=0.046), and arthralgia (39.3% vs. 20.0%, P=0.022). Higher rate of positve antinuclear antibody (ANA) (85.3% vs. 55.0%, P<0.001), lower rate of positive anti-Jo-1 antibody (32.5% vs. 50.0%, P=0.039), lower albumin level [(34.6±5.2) g/L vs. (37.3±4.7) g/L, P=0.004] and lower lymphocyte counts [(1.4±0.8) ×10 9/L vs. (1.8±0.8) ×10 9/L, P=0.014] were more common in patients with positive anti-Ro52 antibody. The presence of anti-Ro52 antibody is associated with a particular phenotype of ASS, leading to common ILD, involvement of joints, high ANA positivity, low albumin and low lymphocyte counts.

Congenital heart block secondary to neonatal lupus

@#Neonatal lupus is a passively acquired autoimmune disease that occurs in children of mothers with anti-Ro/SSA and/or anti-La/SSB antibodies. [1-4] The most serious complication in the neonate is complete heart block. [3-8] This is a case report of a newborn female presenting with persistent bradycardia detected in utero. The diagnosis was confirmed by maternal anti-Ro/SSA and/or anti-La/SSB antibodies and in utero detection of fetal heart block on echocardiogram. Therapeutic management involved placement of a permanent pacemaker.

Serie de casos clínicos de pacientes con Miopatía inflamatoria idiopática y Enfermedad pulmonar intersticial del registro de Miopatías inflamatorias idiopáticas de la Sociedad Argentina de Reumatología / Clinical case series of patients with idiopathic inflammatory myopathy and interstitial lung disease from the SAR's Registry of Idiopathic Inflammatory Myopathies

Las Miopatías Inflamatorias Idiopáticas (MII) son un grupo heterogéneo de enfermedades que se caracterizan por debilidad muscular e inflamación subyacente en la biopsia muscular. Los principales órganos afectados son el músculo, la piel y también puede afectarse el pulmón. Se distinguen dentro de los subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), DM con la variante Dermatomiositis Clínicamente Amiopática (DMCA), el Síndrome Antisintetasa (SAS), la Miositis Necrotizante Inmunomediada, la Miositis por Cuerpos de Inclusión (MCI) y la Miositis Asociada a Neoplasia. La presencia de ciertos anticuerpos específicos y asociados predispone al desarrollo de manifestaciones clínicas, determinando el pronóstico de la enfermedad. Se presentan 4 pacientes del Registro de MII de la Sociedad Argentina de Reumatología (SAR) con estas características: un paciente con PM y anti Jo-1 positivo y tres pacientes con DM (uno con DMCA y anti-RO 52 y dos pacientes con anti-PL7 y anti-TIF1γ respectivamente).

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TIF1γ respectively).

Serie de casos clínicos de pacientes con Miopatía inflamatoria idiopática y Enfermedad pulmonar intersticial del registro de Miopatías inflamatorias idiopáticas de la Sociedad Argentina de Reumatología / Series of clinical cases of patients with idiopathic inflammatory myopathy and interstitial lung disease from the registry of idiopathic inflammatory myopathies of the Argentine Society of Rheumatology

Las Miopatías Inflamatorias Idiopáticas (MII) son un grupo heterogéneo de enfermedades que se caracterizan por debilidad muscular e inflamación subyacente en la biopsia muscular. Los principales órganos afectados son el músculo, la piel y también puede afectarse el pulmón. Se distinguen dentro de los subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), DM con la variante Dermatomiositis Clínicamente Amiopática (DMCA), el Síndrome Antisintetasa (SAS), la Miositis Necrotizante Inmunomediada, la Miositis por Cuerpos de Inclusión (MCI) y la Miositis Asociada a Neoplasia. La presencia de ciertos anticuerpos específicos y asociados predispone al desarrollo de manifestaciones clínicas, determinando el pronóstico de la enfermedad. Se presentan 4 pacientes del Registro de MII de la Sociedad Argentina de Reumatología (SAR) con estas características: un paciente con PM y anti Jo-1 positivo y tres pacientes con DM (uno con DMCA y anti- RO 52 y dos pacientes con anti-PL7 y anti-TIF1γ respectivamente).

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TIF1γ respectively).

Lupus eritematoso neonatal. Reporte de caso de lactante concebida por inseminación artificial y madre con artritis reumatoidea / Lupus eritematoso neonatal Report of the infant case conceived by artificial insemination and mother with rheumatoid arthritis

El Lupus Eritematoso Neonatal es una enfermedad de origen autoinmune caracterizada por rash cutáneo transitorio, bloqueo cardíaco congénito permanente, función hepática anormal con o sin enfermedad biliar y compromiso hematológico asociado a la presencia de autoanticuerpos maternos contra la ribonucleoproteinas solubles (SSB/La, SSA/Ro y Anti-RNP). Se presenta el caso de una niña de cinco meses de edad con hallazgos clínicos e histopatológicos de Lupus eritematoso neonatal. Es una condición que no suele dejar secuelas, aunque se han reportado casos de atrofia cutánea e hiperpigmentación

Neonatal Lupus Erythematosus is an autoimmune disease characterized by transitory cutaneous rash, congenital heart block, abnormal liver function test with or without cholestasis and hematologic features associated to anti-Ro and anti-La autoantibodies. A 5-month-old female is brought to the hospital with clinical and histopathology findings of Neonatal lupus. This medical condition does not leave long term physical damage, however there have been cases reported with cutaneous atrophy and hyperpigmentation

Potential value of autoantibodies as biomarkers of chronic graft-versus-host disease after allogeneic stem cell transplantation / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

We investigated the value of autoantibodies as biomarkers of chronic graft-versus-host disease (cGVHD) by analyzing the autoantibody profiles of 65 patients (34 cGVHD and 31 non-cGVHD) surviving longer than three months after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Autoantibodies to at least one antigen were detected in 45 patients (70.8%), with multiple autoantibodies detected in 30 patients (46.2%). Antinuclear antibodies (ANAs) were the most frequently detected autoantibodies, with a significantly higher prevalence in non-cGVHD patients and cGVHD patients than that in healthy controls (HCs). ANA-nucleolar (ANA-N) was the main immunofluorescence pattern of ANA-positivity in both the non-cGVHD and cGVHD groups. There was a higher prevalence of anti-Ro52-positivity in non-cGVHD and cGVHD patients than in HC. Liver cGVHD was significantly associated with anti-Ro52-positivity. However, cGVHD activity and severity were not associated with the presence of autoantibodies. Similarly, there were no significant differences in overall survival or relapse among the four groups of patients expressing autoantibodies. Our results suggest that autoantibodies have limited value in predicting cGVHD.

Antisynthetase syndrome with positive anti-Ro52 antibody: A report of 2 cases and literature review / 吉林大学学报(医学版)

Objective: To summarize the relationship between anti-Ro-52 antibody and anti-synthase syndrome(ASS)by analyzing the diagnosis and treatment of ASS patients with positive anti-Ro-52 antibody, and to clarify its significance. Methods: The clinical materials of 2 ASS patients with positive anti-Ro52 antibody were collected,and the clinical characteristics were analyzed; combined with literature review, the relationship between anti-Ro-52 antibody and ASS was explored.Results: Two young female patients were admitted to hospital because of the obvious symptoms of both hand joints. On physical examination, arthroncus of both hand joints was observed, both hands showed "mechanic hand", muscle strength and muscle tension of limbs were normal. The activity of creatine kinase was increased. Myositis antibody spectrum, lung CT, electromyography and muscle biopsy were performed and the patients were given the related treatments after confirmed diagnosis. The anti-Jo-1 and anti-Ro-52 antibodies were strongly positive in the myositis antibody spectrum of the two patients; the lung CT results showed interstitial pheumonia;the electromyogram results indicated myogenic damage; the results of muscle biopsy was consistent with the changes of idiopathic inflammatory myopathy. Both patients were diagnosed as ASS. After treatment of glucocorticoids and immunosuppressive agents, the symptoms were improved and the creatine kinase activity was decreased. No disease activity and secondary tumor signs were found in the follow-up.Conclusion: As a myositis-associated antibody,anti-Ro-52 antibody can lead to a series of atypical clinical manifestations in the ASS patients and may be associated with the poor prognosis of the ASS patients. Follow-up should be paid attention to.

Potential value of autoantibodies as biomarkers of chronic graft-versus-host disease after allogeneic stem cell transplantation / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

We investigated the value of autoantibodies as biomarkers of chronic graft-versus-host disease (cGVHD) by analyzing the autoantibody profiles of 65 patients (34 cGVHD and 31 non-cGVHD) surviving longer than three months after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Autoantibodies to at least one antigen were detected in 45 patients (70.8%), with multiple autoantibodies detected in 30 patients (46.2%). Antinuclear antibodies (ANAs) were the most frequently detected autoantibodies, with a significantly higher prevalence in non-cGVHD patients and cGVHD patients than that in healthy controls (HCs). ANA-nucleolar (ANA-N) was the main immunofluorescence pattern of ANA-positivity in both the non-cGVHD and cGVHD groups. There was a higher prevalence of anti-Ro52-positivity in non-cGVHD and cGVHD patients than in HC. Liver cGVHD was significantly associated with anti-Ro52-positivity. However, cGVHD activity and severity were not associated with the presence of autoantibodies. Similarly, there were no significant differences in overall survival or relapse among the four groups of patients expressing autoantibodies. Our results suggest that autoantibodies have limited value in predicting cGVHD.

Permanent pacemaker implantation in complete congenital fetal atrioventricular (AV) block: a case report / Implantación de marcapasos permanente en bloqueo atrioventricular (AV) fetal congénito completo: reporte de caso

Abstract: One of the most common pathologies attributed to lupus neonatal refers to atrioventricular (AV) congenital block, which diagnosis can be made between 16 and 30 weeks of gestation due to persistent fetal bradycardia. The development of this disease is mostly related to maternal anti-Ro/SSA and anti-Smith autoantibodies. Currently, there are a number of alternatives for prenatal and postnatal treatment, with some controversy about their viability. The placement of a permanent pacemaker is presented as one of the most appropriate procedures currently, even with the risks awarded. This case report describes the placement of a permanent pacemaker to a two-month-old newborn with high maternal contents of anti-Ro/SSA and anti-Smith nuclear autoantibodies, with a favorable outcome.(AU)

Resumen: Una de las patologías más comunes atribuidas al lupus neonatal se refiere al bloqueo congénito atrioventricular (AV), diagnóstico que se puede realizar entre 16 y 30 semanas de gestación debido a bradicardia fetal persistente. El desarrollo de esta enfermedad se relaciona principalmente con los anticuerpos anti-Ro/SSA materno y anti-Smith. Actualmente, existen varias alternativas para el tratamiento prenatal y postnatal, con cierta controversia sobre su viabilidad. La colocación de un marcapasos permanente se presenta como uno de los procedimientos más adecuados actualmente, incluso con los riesgos adjudicados. Este relato de caso describe la colocación de un marcapasos permanente en un recién nacido de dos meses con alto contenido materno de autoanticuerpos anti-Ro/SSA y anti-Smith, con un resultado favorable.(AU)

Analysis on results difference of two methods for detecting 146 cases of anti-nuclear antibody / 国际检验医学杂志

Objective To investigate the results difference of the indirect immunofluorescence (IIF) and immunoblotting (LIA) for detecting ant-inuclear antibodies (ANA) and clinical value .Methods One hundred and forty-six ANA detection specimens of IIF negative and LIA positive were collected and performed the detection of HBV antibodies and anti-HCV antibodies .Results (1) In 146 specimens of IIF (-)LIA (+ ) ,the positive specimen numbers of single anti-Ro52 antibody ,anti-SSA antibody and anti-AMA-M2 antibody were 69 cases ,42 cases and 15 cases respectively ,which of anti-RNP antibody ,anti-PCNA antibody and PM-Scl antibody were 3 cases ,5 cases and 2 cases respectively ,the combined 2-item positive was in 8 cases .(2)Among positive specimens of single anti-Ro52 antibody ,HBsAg(+ ) ,anti-HBe(+ ) ,anti-HBc(+ ) model and HBsAg(+ ) ,HBeAg(+ ) ,HBcAb(+ ) model of hepatitis B ,and hepatitis C were 51 cases ,4 cases and 7 cases respectively ,7 cases were non-hepatitis patients .(3) Among positive specimens of single ant-SSA antibody ,6 cases were hepatitis B patients and 36 cases were the patients with non-hepatitis B .Conclu-sion Anti-Ro52 antibody and anti-SSA antibody are easier to be missed by the IIF detection .Anti-Ro52 antibody positive has a cer-tain relation with small three positive of hepatitis B .

Sjogren's syndrome: An underdiagnosed condition in mixed connective tissue disease

OBJECTIVE: To determine the prevalence of sicca symptoms, dry eye, and secondary Sjögren's syndrome and to evaluate the severity of dry eye in patients with mixed connective tissue disease. METHODS: In total, 44 consecutive patients with mixed connective tissue disease (Kasukawa's criteria) and 41 healthy controls underwent Schirmer's test, a tear film breakup time test, and ocular surface staining to investigate dry eye. In addition, the dry eye severity was graded. Ocular and oral symptoms were assessed using a structured questionnaire. Salivary gland scintigraphy was performed in all patients. Classification of secondary Sjögren's syndrome was assessed according to the American-European Consensus Group criteria. RESULTS: The patients and controls had comparable ages (44.7±12.4 vs. 47.2±12.2 years) and frequencies of female gender (93 vs. 95%) and Caucasian ethnicity (71.4 vs. 85%). Ocular symptoms (47.7 vs. 24.4%) and oral symptoms (52.3 vs. 9.7%) were significantly more frequent in patients than in controls. Fourteen (31.8%) patients fulfilled Sjögren's syndrome criteria, seven of whom (50%) did not have this diagnosis prior to study inclusion. A further comparison of patients with mixed connective tissue disease with or without Sjögren's syndrome revealed that the former presented significantly lower frequencies of polyarthritis and cutaneous involvement than did the patients without Sjögren's syndrome. Moderate to severe dry eye was found in 13 of 14 patients with mixed connective tissue disease and Sjögren's syndrome (92.8%). CONCLUSIONS: Sjögren's syndrome, particularly with moderate to severe dry eye, is frequent in patients with mixed connective tissue disease. These findings alert the physician regarding the importance of the appropriate diagnosis of this syndrome in such patients. .

Rhizomelic Chondrodysplasia Punctata With Maternal Systemic Lupus Erythromatosus.

We report Rhizomelic Chondrodysplasia Punctata (RDCP), a rare, autosomal recessive disorder with rhizomelic shortening of limbs, congenital cataracts and seizures but without any biochemical abnormality. The mother of the baby developed Systemic Lupus Erythromatosus (SLE) with Ro/SSA antibodies 11 months after delivery. Ro/SSA antibodies may generate calreticulin antibodies causing characteristic skeletal changes.

A Case of ANA-negative, Anti-dsDNA Negative, and Anti-Ro/SSA Positive Membranous Lupus Nephropathy

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of diverse autoantibodies with various systemic organ involvements. In patients with SLE, autoantibodies, such as antinuclear antibody (ANA) and anti-dsDNA antibody, play an important role not only in diagnosing the disease, but also representing the pathogenesis of the disease. ANA is the main screening tool in diagnosis and serum complement levels and anti-dsDNA antibody level are closely related to the disease activities. Nevertheless, exceptionally, some patients represent with negative ANA and/or anti-dsDNA antibody leading to difficulties in diagnosing the disease. Here, we report a case of 37-year old female SLE patient with negative ANA, negative anti-dsDNA antibody, and positive anti-Ro/SSA antibody, which manifested with nephrotic syndrome.

Presença de anti-RO prediz aparecimento de lesão valvar em artrite reumatoide / Presence of anti-RO predicts onset of valve lesions in rheumatoid arthritis

JUSTIFICATIVA E OBJETIVOS: Alguns autoanticorpos são associados com achados clínicos peculiares. Pacientes com artrite reumatoide (AR) podem apresentar autoanticorpo anti-RO. O objetivo deste estudo foi estudar a prevalência e associações clínicas do anticorpo anti-RO em pacientes com AR. MÉTODO: Foram estudados 385 pacientes diagnosticados com AR e que possuíam teste Elisa para anti-RO. Também foram analisadas informações referentes ao perfil do paciente, avaliação funcional, DAS-28, manifestações extra-articulares, função tireoidiana, perfil de autoanticorpos e de tratamento.RESULTADOS: A prevalência do anticorpo anti-RO foi de 8,31%. Não houve diferença significativa em relação ao sexo, HAQ, DAS-28, classificação funcional em pacientes com anti-RO positivo (p = ns). Pacientes com anti-RO apresentaram idade menor ao diagnóstico (p = 0,02). Em relação às manifestações extra-articulares, encontrou-se maior prevalência de lesões valvares cardíacas (p < 0,001) em pacientes com anticorpo anti-RO.Não foram encontradas diferenças significativas na análise de outras desordens extra-articulares, associação com hipotireoidismo,amiloidose, tratamentos indicados, presença de fator reumatoide (FR) e anticorpo antipeptídeo cíclico citrulinado (anti-CCP). CONCLUSÃO: Pacientes com AR que apresentam o anticorpo anti-RO tem um início de doença mais precoce. O anticorpoanti-RO é um fator de risco para o desenvolvimento de lesões valvares. Não foram encontradas relações do anti-RO com tireoidopatias, amiloidose secundária e escolha de tratamento.

BACKGROUND AND OBJECTIVES: Some auto antibodies are associated with peculiar clinical findings. Patients with rheumatoid arthritis (RA) may have anti-RO antibodies. The objective of this study was to investigate the prevalence and clinical associations of anti-RO antibodies in RA patients. METHOD: We studied 385 patients with RA for anti-RO by Elisa testing and for clinical profile, functional assessment, DAS-28, extra-articular manifestations, thyroid function, auto antibodies and treatment. RESULTS : The prevalence of anti-RO was 8.31%. There was no significant difference in sex distribution, HAQ, DAS-28,functional classification in patients with positive anti-RO (p = ns). Patients with anti-RO were younger at diagnosis (p = 0.02).Analyzing extra-articular disorders we found a greater prevalence of cardiac valve lesions (p < 0.001) in patients with anti-RO antibodies. No differences were found in other extra-articular manifestations, associated hypothyroidism, amyloidosis, treatment requirements, presence of rheumatoid factor (RF) and anti citrullinated protein antibodies (ACPA). CONCLUSION : RA patients with anti-RO have disease onset at earlier age. Anti-RO is a risk factor for the development of valve lesions. There is no association between this antibody and thyroid disease, amyloidosis and treatment needs.

Correlación de hallazgos serológicos y compuestos orgánicos en saliva en el Síndrome de Sjõgren / Correlation of serological findings and saliva’s organic compounds in Sjõgren’s Syndrome

Introducción: El objetivo del presente trabajo es evaluar los cambios en flujo y composición orgánica en saliva, así como la presencia de anticuerpos anti Ro/SSA y La/SSB séricos y salivales y su implicancia en el diagnóstico no invasivo del SS. Diseño del estudio: Estudio de corte transversal, de 73 pacientes distribuidos en los siguientes grupos experimentales: Síndrome de Sjõgren primario (SSp) (n = 15), SS secundario (SSs) (n = 17), boca seca y ojo seco sin Síndrome de Sjõgren (BO) (n = 20), y controles sanos (C) (n = 21). Se realizó una determinación del flujosalivalbasal y una toma de muestras de saliva para la medición de proteínas totales, IgA, urea y anticuerpos. Se determinaron anticuerpos anti Ro/SSA y La/SSB en muestras de suero y saliva. Resultados: El flujo salival en SSp, SSs, BO fue significativamente menor (p < 0,001) comparado con C. La composición salival de SS mostró modificaciones de componentes estudiados. Los anticuerpos anti Ro/SSA y anti La/SSB aparecieron con mayor frecuencia en suero y saliva en pacientes con SS en comparación con BO y C, siendo la frecuencia de positividad superior en suero en comparación con saliva. Conclusión: La determinación de anticuerpos Ro/SSA en saliva podrían ayudar a diagnosticar a pacientes con xerostomía como el Síndrome de Sjõgren.

Introduction: The aim of this study was to evaluate changes in flow andorganic composition in saliva, the presence of anti Ro/SSA and La/SSBantibodies in serum and saliva and its implication in the noninvasivediagnosis of SS.Study Design: Cross sectional study, 73 patients divided into four experimentalgroups: Primary Sjögren's syndrome (pSS) (n = 15), secondarySS (sSS) (n = 17), dry eye and dry mouth syndrome without Sjögren's(DEMS) (n = 20) and healthy controls (C) (n = 21). We performed a determinationof basal salivary flow and saliva sampling for measurement oftotal protein, IgA, urea and antibodies. We determined anti Ro/SSA andLa/SSB in serum and saliva.Results: The salivary flow in pSS, sSS, DEMS patients was significantlylower (p <0.001) compared with C. The composition of SS salivary componentsstudied showed changes. The anti Ro/SSA and anti La/SSB occurredmore frequently in serum and saliva in SS patients comparedwith DEMS and C, the frecuency of positivity was higher in serum thanin saliva.Conclusion: The determination in saliva of antibodies Ro/SSA may helpdiagnose patients with xerostomy as Sjögren's Syndrome.

Miocarditis y bloqueo aurículo-ventricular fetal asociado a lupus eritematoso sistémico materno / Myocarditis and atrioventricular block associated with systemic lupus erythematosus

El lupus eritematoso sistémico (LES) es una de las patologías autoinmunes más frecuentes durante el embarazo, asociándose con distintas complicaciones fetales y neonatales, sobre todo cardíacas, secundario al traspaso de anticuerpos maternos a través de la placenta. Estos anticuerpos se unen a los cardiomioci-tos fetales, desencadenando una respuesta inflamatoria local que determina la aparición de lesiones que pueden ser permanentes y letales. Presentamos el caso de una paciente embarazada con LES, en la cual se observó en el feto la presencia de bloqueo aurículo-ventricular de primer grado y signos sugerentes de miocarditis. Estas complicaciones se caracterizan por un aumento en la morbimortalidad perinatal, por lo que las estrategias actuales están dirigidas a la detección precoz de éstas y también en la prevención de las mismas. Un tratamiento estándar aun es tema de investigación, pese a los reportes que muestran la efectividad de corticoides como la dexametasona. En embarazadas con anticuerpos anti-Ro positivo se recomienda efectuar ecocardiograma fetal seriados cada 1-2 semanas desde la semana 16, para detectar precozmente anomalías cardiacas sobre las cuales pudiese intervenirse.

Systemic lupus erythematosus (SLE) is one of the most common autoimmune disease during pregnancy, associated with various fetal and neonatal complications, especially heart disease, secondary to the transfer of maternal antibodies through the placenta. These antibodies bind to fetal cardiomyocytes, triggering a local inflammatory response that determines the appearance of lesions that may become permanent and deadly. We report a pregnant patient with SLE, in which was observed the presence of atrioventricular block of 1st degree and signs suggestive of myocarditis in the fetus. These complications are characterized by an increase in fetal and neonatal morbidity and mortality, so that current strategies are aimed at early detection of these and also in preventing them. A standard therapy for atrioventricular block is still matter of investigation, although corticosteroids like dexamethasone have been reported to be effective for associated cardiomyo-pathy. Serial echocardiograms and obstetric sonograms, performed at least every 1-2 weeks starting from the 16th week of gestational age, are recommended in anti-Ro/SSA-positive pregnant women to detect early fetal abnormalities that might be a target of preventive therapy.

Annular Erythema Associated with Anti-Ro/La Antibody Occurring in Plantar Area / 대한피부과학회지

Recurrent annular erythema associated with anti-Ro/La antibody is a diagnostic term for annular erythemas that usually occurs in the face and the upper extremities of patients with positive anti-Ro/La antibodies. They have been reported in patients with Sjogren's syndrome, lupus erythematosus, or Sjogren's syndrome/systemic lupus erythematosus syndrome. Recently, there have been cases without any underlying autoimmune diseases. We, hereby, report an annular erythema, associated with anti-Ro/La antibody occurring in both soles, which is an unusual location for this disease.

Bloqueo cardiaco fetal completo: reporte de dos casos y revisión de la literatura / Fetal complete atrioventricular block: two case report and literature review

Presentamos 2 casos de embarazos controlados en nuestro servicio con el diagnóstico de bloqueo aurículo-ventricular fetal. Este es un tipo de arritmia poco frecuente, relacionado con la presencia de anticuerpos antiribonucleoproteínas (Ro y La). El manejo es expectante en la mayoría de los casos ya que no existe forma de revertir el bloqueo; en caso de evidenciar una descompensación hemodinámica fetal, se pueden administrar corticoides como medida terapéutica con un éxito limitado. No existe contraindicación del parto vaginal y el uso de pH de cuero cabelludo y oximetría de pulso parecen ser métodos adecuados para la evaluación de la condición fetal intraparto. Recomendamos el enfoque multidisciplinario en esta patología para evitar intervenciones innecesarias, anticipar los riesgos fetales y obtener un mejor pronóstico postnatal.

Here we report the perinatal outcome of two patients with fetal complete atrioventricular (AV) block. This is an uncommon disease, related to the presence of autoantibodies against ribonucleoproteins (Ro and La). Management should be expectant in most cases because a treatment to revert the AV block is not available; when fetal hemodynamic problems are detected corticosteroids can be used, but with limited effectiveness. Vaginal delivery is allowed; fetal scalp pH and pulse oximetry are appropriate for intrapartum fetal surveillance. We recommend a multi disciplinary approach to avoid unnecessary interventions, anticipate fetal risk and obtain a better perinatal outcome.