Evaluation of serum microRNA-30e-5p expression in systemic lupus erythematosus and its association with clinical manifestations: A cross-sectional study / Evaluación de la expresión del microARN-30e-5p sérico en el lupus eritematoso sistémico y su asociación con las manifestaciones clínicas: un estudio transversal

ABSTRACT Background: MicroRNAs (miRNAs) are non-coding RNAs that regulate gene expression post-transcriptionally. Accumulating evidence indicates that the miR-30 family takes part in the development of multiple tissues and organs, and is a potential contributor to various dis eases, including autoimmune disorders such as systemic lupus erythematosus (SLE). The aim of this study was to evaluate the expression of miR-30e-5p, a member of the miR-30 fam ily, and investigate its potential relationship to clinical characteristics and possible disease activity in an Egyptian SLE cohort. Methods: Serum samples from 40 SLE patients and 37 age and gender matched healthy sub jects were tested for miR-30e-5p expression level using the Taqman quantitative reverse transcription-polymerase chain reaction. Analysis was performed using the 2 - AACT method. Results: The mean age of the patients was 28.7 ± 7.9 years, with a mean disease duration of 6.4 ±5.3 years. The median fold change in serum miR-30e-5p among our SLE cohort was significantly higher 1.748 (0.223-20.485) compared to the control group 0.877 (0.058-3.522) (P = 0.02). Receiver operating characteristic curve analysis revealed that miR-30e-5p expres sion level can discriminate SLE patients from controls at a cut-off value >1.06 with the area under the curve (AUC) = 0.676 (95% CI: 0.559-0.794, P = 0.02), with 64.3% sensitivity and 61.5% specificity. There was no correlation between any of the demographic features, clinical manifestations (apart from serositis, P = 0.013) or disease activity and miR-30e-5p levels. Conclusion: Our study demonstrated elevated miR-30e-5p expression levels in serum sam ples of SLE patients. Apart from serositis, it was not associated with any other disease characteristics.

RESUMEN Antecedentes: Los microARN (miRNA) son ARN no codificantes que regulan la expresión de los genes después de la transcripción. Las pruebas acumuladas indican que la familia de miR-30 participa en el desarrollo de múltiples tejidos y órganos, y es un posible contribuyente a diversas enfermedades, incluidos los trastornos autoinmunes como el lupus eritematoso sistémico (LES). El objetivo de este estudio fue evaluar la expresión del miR-30e-5p, un miembro de la familia miR-30, e investigar su posible relación con las características clínicas y la posible actividad de la enfermedad en una cohorte egipcia de LES. Métodos: Se analizaron muestras de suero de 40 pacientes con LES y 37 sujetos sanos de edad y sexo similares para determinar el nivel de expresión de miR-30e-5p, utilizando la reacción en cadena de la polimerasa de transcripción inversa cuantitativa Taqman. El análisis se llevó a cabo empleando el método 2-AACT. Los resultados: La edad media de los pacientes fue de 28,7 ± 7,9 años, mientras que la duración media de la enfermedad fue de 6,4 ± 5,3 años. La mediana del cambio de pliegue del suero miR-30e-5p entre nuestra cohorte de LES fue significativamente mayor, 1,748 (0,223-20,485), en comparación con el grupo de control, 0,877 (0,058-3,522) (p = 0,02). El análisis de la curva característica de funcionamiento del receptor reveló que el nivel de expresión del miR-30e-5p puede discriminar a los pacientes con LES de los controles en un valor de corte > 1,06, con el área bajo la curva (AUC) = 0,676 (IC del 95%: 0,559-0,794; p = 0,02), una sensibilidad del 64,3% y una especificidad del 61,5%. No hubo asociación entre ninguna de las características demográficas, manifestaciones clínicas (aparte de la serositis, p = 0,013) o actividad de la enfermedad y los niveles de miR-30e-5p. Conclusión: Nuestro estudio demostró niveles elevados de expresión de miR-30e-5p en mues tras de suero de pacientes con LES. Aparte de la serositis, no se asoció con ninguna otra característica de la enfermedad.

The Effect of Mycophenolate Mofetil on Non-Renal Manifestations in Systemic Lupus Erythematosus: Results from Korean Lupus Network Registry

BACKGROUND: The objective of this study was to identify the effects of mycophenolate mofetil (MMF) on non-renal manifestations in systemic lupus erythematosus (SLE). METHODS: The study population comprised 439 SLE patients from the Korean Lupus Network registry who were followed up annually and completed the baseline survey and two follow-up visits from 2014 to 2018. Disease activity, laboratory markers, and clinical manifestations including mucocutaneous lesions, arthritis, serositis, neurological disorders, and hematologic/immunologic abnormalities were assessed. All variables by group (MMF and non-MMF) effects with time (baseline, 1st follow-up, and 2nd follow-up) were analyzed by generalized estimation equation. RESULTS: Seventy-two patients were treated with MMF. There was significant difference in frequencies of malar rash, arthritis, renal disorder, and hematologic disorder between MMF and non-MMF groups in total SLE patients. In subgroup analysis of hematologic abnormalities in total patients, frequency of leukopenia was significantly different between the two groups during follow-up (P = 0.001), but frequencies of hemolytic anemia, lymphopenia, and thrombocytopenia were not. In addition, frequencies of leukopenia in patients without lupus nephritis were significantly decreased in MMF group compared to non-MMF group (P = 0.012). CONCLUSION: This study showed that MMF might be a beneficial treatment for hematologic abnormalities, especially leukopenia, in SLE.

Aspectos patológicos e imuno-histoquímicos da polisserosite em búfalos (Bubalus bubalis) / Histopathological and immunohistochemical study of the polyserositis in buffalo (Bubalus bubalis)

Polisserosites são alterações inflamatórias das serosas viscerais e parietais das cavidades corpóreas. Um tipo especial destas alterações foi identificado em bubalinos abatidos para consumo nos anos 80, sendo associada a infecção por Chlamydia psittaci. Apesar da importância da bubalinocultura no Pará, do caráter zoonótico da C. psittaci e da possibilidade de envolvimento de outros agentes na afecção, são raros trabalhos sobre a enfermidade desde os estudos pioneiros. No presente trabalho casos identificados como polisserosite pelo serviço de inspeção sanitária em búfalos abatidos para consumo foram coletados com objetivo de determinar a frequência e origem dos mesmos e de caracterizar as lesões e pesquisar antígenos de Chlamydia spp. nas mesmas. De um total de 2.887 bubalinos abatidos no período de estudo foram identificados 48 (1,66%) casos. O município de Santa Cruz do Arari na Ilha de Marajó apresentou a maior porcentagem de casos em relação ao número de animais abatidos (6,49%, 5/77). Na macroscopia as lesões se caracterizaram por áreas opacas, branco-amareladas de espessamento das serosas, por vezes com franjas fibrosas na superfície. Os resultados permitem concluir que os casos classificados como polisserosite pelos veterinários da inspeção sanitária nos búfalos abatidos para consumo correspondem na histopatologia a um tipo incomum de polisserosite, caracterizada por infiltrado linfocitário, com ocasionais formações de folículos linfoides terciários ou ectópicos. Foi observado ainda nestas lesões um predomínio de linfócitos T (CD3 positivos) no infiltrado, sendo os linfócitos B (CD79 positivos) presentes em maior número no interior das estruturas foliculares. Não foram demonstrados antígenos de clamídias nas lesões do presente estudo, o que demonstra a necessidade de estudos adicionais relativos a(s) etiologia(s) das lesões.(AU)

Polyserositis are inflammatory changes of the visceral and parietal serous of body cavities. A special type of polyserositis was identified in buffaloes in the 80s, being associated with infection by Chlamydia psittaci. Since these pioneering studies, there are no additional works about the condition. Considering the importance of buffalo in Pará, the zoonotic character of C. psittaci and the possibility of involvement of other agents in polyserositis in buffaloes the present study is proposed. We collected cases identified as polyserositis by sanitary inspection service in buffalo slaughtered for consumption in Belem for a complementary characterization of inflammatory cell and the research of Chlamydia spp antigens in lesions. Of 2.887 buffaloes slaughtered in a period of six months, there were 48 (1.66%) cases of polyserositis and 39 analyzed. Santa Cruz do Arari in Marajó Island was the city with the highest frequency of cases, whereas 6.49% of buffaloes had lesions. However, 50% of the present study cases came from Soure municipality in Marajó Island, which provided about 49% of buffaloes slaughtered in the period. In the macroscopy, there were opaque areas with white-yellow thickening of the serous, sometimes with fibrous fringes on the surface. Histopathology showed connective tissue projections partially lined by cuboid or flattened mesothelial cells. Often in projections there were mononuclear infiltrate of variable intensity, consisting mainly of lymphoid cells, with occasional ectopic or tertiary lymphoid follicles.(AU)

Endoscopic Findings of Children with Familial Mediterranean Fever / 대한소아소화기영양학회지

PURPOSE: Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by periodic fever, synovitis and serositis. Patients may be admitted to gastroenterology units due to gastrointestinal symptoms. In this study; we aimed to analyze endoscopic findings and diagnostic utility of endoscopic procedure in children with FMF. METHODS: Patient with FMF that was performed endoscopy for the gastrointestinal symptoms were included to the study (39 of 164 patients, 53 procedure). A control group was randomly designed as age and gender matched four endoscopic procedures per one endoscopic procedure of patients with FMF (n=212). RESULTS: No different was found between the patients and control group in esophagogastroscopy findings. However, the diagnosis of gastrointestinal pathology was made by esophagogastroscopy in 46.2% patients. Colonoscopic examination revealed that the frequency of inflammatory bowel disease (IBD) was higher in undiagnosed patients compared to both the control group (50.0% vs. 6.9%, p < 0.05, odds ratio [OR]:13.4 and 95% confidence inteval [95% CI]: 2.1–84.3) and the patients under colchicine treatment (50.0% vs. 8.3%, p < 0.05, OR: 11 and 95% CI: 0.8–147.8). Colonoscopic procedure that was made after the diagnosis was found to provide contribution by 16.7% in determining the etiology of the additional symptoms. CONCLUSION: Patients with FMF may be admitted to pediatric gastroenterology outpatient clinic prior to diagnosis or during the follow-up period. The frequency of IBD is high in undiagnosed patients with FMF. Endoscopic procedures may be helpful in these patients for the diagnosis accompanying mucosal lesions.

Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.

Autoimmune thyroiditis as initial presentation of Systemic Lupus Erythematosus complicated by massive ascites: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Autoimmune thyroiditis in the course of other autoimmune diseases such as systemic lupus erythematosus (SLE) is common because these disorders are attributed to the production of autoantibodies against various autoantigens. Beyond this association, autoimmune thyroiditis can occur before, during or after the development of SLE. In this report, we describe a female who presented with facial puffiness, lethargy and progressive abdominal distension. She was diagnosed with autoimmune thyroiditis followed by the diagnosis of SLE complicated by a massive ascites, a rare form of lupus peritonitis, which is sterile ascites that results from severe serositis. Her presentation was complex and posed a diagnostic challenge and dilemma to the physicians involved in her care.

Rituximab for refractory anemia and thrombocytopenia in a patient with systemic lupus erythematosus

INTRODUCTION: Hematologic manifestations of Systemic Lupus Erythematosus (SLE) such as hemolytic anemia, leucopenia and thrombocytopenia are among the common causes of morbidity and hospitalization among patients. This is a case report of a patient presenting with refractory cytopenias.CASE: The  patient  is  a  42-year-old  female,  diagnosed with  SLE,  having  met  the  Systemic  Lupus  Erythematosus  International  Collaboration  Clinics  Criteria  for  diagnosis Persistent  serositis  (pleural  effusion  and  ascites) and worsening of anemia prompted treatment with oral corticosteroid (prednisone 1.0 mg per kg per day) which showed improvement of shortness of breath and  pleural effusion. Cytopenias persisted despite increasing IV pulse steroid to 6.0  mkd  prednisone then further to 13 mkd prednisone.Rituximab given as 1.0 g infusion once every two weeks for two doses which resulted to improvement of anemia, thrombocytopenia and serositis.  CONCLUSION: Hematologic manifestations may present as life threatening complications of lupus.Most cases are responsive  to  corticosteroid  therapy,however,in a few refractory cases,less used conventional treatment such as rituximab,may provide significant response.

Familial Mediterranean fever presenting as fever of unknown origin in Korea / 소아과

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, MEFV which encodes the pyrin protein. Although FMF predominantly affects people from Mediterranean and Middle Eastern ethnic origins, 3 cases of FMF have been reported in Korea since 2012. We report another case of FMF in Korea in which the patient presented with a month-long fever without serositis. After treatment with colchicine was initiated, the patient’s symptoms quickly subsided. The response to colchicine was helpful for diagnosis. We compare the FMF genotypes in Korea with in other countries. Studying FMF cases in Korea will help establish the best MEFV exons to use for screening and diagnosis of Korean FMF.

Autoimmune thyroiditis as initial presentation of Systemic Lupus Erythematosus complicated by massive ascites: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Autoimmune thyroiditis in the course of other autoimmune diseases such as systemic lupus erythematosus (SLE) is common because these disorders are attributed to the production of autoantibodies against various autoantigens. Beyond this association, autoimmune thyroiditis can occur before, during or after the development of SLE. In this report, we describe a female who presented with facial puffiness, lethargy and progressive abdominal distension. She was diagnosed with autoimmune thyroiditis followed by the diagnosis of SLE complicated by a massive ascites, a rare form of lupus peritonitis, which is sterile ascites that results from severe serositis. Her presentation was complex and posed a diagnostic challenge and dilemma to the physicians involved in her care.

Taponamiento cardiaco por pericarditis lúpica: reporte de dos casos / Cardiac tamponade due to lupic pericarditis: report of two cases

La serositis es uno de los hallazgos clínicos en pacientes con lupus eritematoso sistémico.Cuando se presenta a nivel de pericardio, generalmente causa derrames pericárdicos cró-nicos que no generan inestabilidad hemodinámica en el paciente. Presentamos 2 casosclínicos de mujeres con lupus con derrame pericárdico que lleva a taponamiento cardiaco,presentación inusual de esta manifestación clínica del lupus eritematoso sistémico...

Serositis is one of the clinical findings in patients with systemic lupus erythematosus. Whenis present generally causes chronic pericardial effusions and not generate hemodynamicinstability in the patient. We present two cases of women with lupus with pericardial effusionleading to cardiac tamponade, unusual presentation of this clinical manifestation insystemic lupus erythematosus...

Cardiac tamponade as a rare manifestation of systemic lupus erythematosus: A report on four cases in the Philippine General Hospital

SYNOPSIS: Cardiac tamponade among systemic lupus erythematosus (SLE) patients is an unusual event. The pericardial effusion may be a consequence of uremia, infections in the pericardium, or the lupus pericarditis itself. We present four atypical cases of cardiac tamponade from pericarditis of connective tissue disease (CTD), all of which were treated with drainage and immunosuppressants. Due to the rarity of this combination, management was a challenge.CLINICAL PRESENTATION: Four females each sought consult for dyspnea associated with typical manifestations of connective tissue disease such as arthritis, characteristic rashes, serositis, typical laboratory features, and a positive ANA and/or anti-dsDNA. The first three cases fulfilled the criteria for SLE, while the fourth fulfilled the criteria for SLE-dermatomyositis overlap syndrome. Echocardiography was done due to suspicion of pericardial involvement and revealed massive pericardial effusion in tamponade physiology in all cases.DIAGNOSIS: Cardiac tamponade from serositis due to connective tissue disease [SLE (case 1 to 3) or SLE-dermatomyositis overlap (case 4). Other common etiologies of tamponade such as bacterial, tuberculous, malignant, and uremic pericardial effusion were ruled out by clinical and laboratory tools, including Gram stain and culture, cytology, PCR, and biochemical testing. The pericardial fluid of the first case tested positive for lupus erythematosus (LE) cells, indicative of lupus serositis.TREATMENT AND OUTCOME: All patients underwent pericardial drainage via tube pericardiostomy. They received high dose glucocorticoids after infectious etiologies for the pericardial effusion were ruled out. The fourth case with the overlap syndrome, however, required more immunosuppressants using azathioprine and methotrexate. Resolution of pericardial effusion was noted with this approach. Three of four were discharged improved, however, the third case suffered from worsening nephritis and pulmonary hemorrhage leading to her demise.SIGNIFICANCE AND RECOMMENDATIONS: Four cases of cardiac tamponade as a manifestation of connective tissue disease were presented. Literature underlines the rarity of this condition anytime during the course of SLE. Despite this, SLE should be considered as one of the differential diagnosis of cardiac tamponade, especially in patients who manifest with multi-systemic findings. Likewise, massive pericardial effusion should be considered in patients with a connective tissue disease presenting with subtle evidence of pericardial involvement. It requires timely identification and treatment with high dose steroids, after other causes such as infections have been excluded. Immediate drainage through pericardiocentesis or pericardiostomy in combination with immunosuppressants may be life-saving.

Familial Mediterranean Fever With Complete Symptomatic Remission During Pregnancy

Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder, ethnically restricted and commonly found among populations surrounding the Mediterranean Sea. FMF is the most prevalent autoinflammatory disease; is characterized by recurrent, self-limited episodes of fever with serositis; and is caused by Mediterranean fever gene (MEFV) mutations on chromosome 16. We describe a case of adult-onset FMF with complete symptomatic remission during pregnancy, without the use of colchicine. A 25-year-old woman had presented with periodic fever, abdominal pain, and vomiting since she was 21. Her abdominal computed tomography scan showed intestinal nonrotation. She underwent exploratory laparotomy and appendectomy for her symptoms 1 year prior. She had a symptom-free pregnancy period, but abdominal pain and fever recurred after delivery. Mutation analysis of the MEFV gene revealed two point mutations (p.Leu110Pro and p.Glu148Gln). We report an adult female patient with FMF in Korea with complete symptomatic remission during pregnancy.

A Case of Intravascular Large B-cell Lymphoma Mimicking Systemic Lupus Erythematosus / 대한내과학회지

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin's lymphoma (NHL) and that progresses rapidly and is usually fatal. Because it usually presents with nonspecific symptoms, such as fever, the early diagnosis of IVLBCL is very difficult and it is often misdiagnosed as another disease. Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various organs. The clinical manifestation of SLE ranges from rash and arthritis through anemia and thrombocytopenia to serositis, nephritis, seizures, and psychosis. Thus, it can be easily confused with many other disorders. We report a case of IVLBCL mimicking SLE in the initial diagnosis.

Ciento quince pacientes con lupus eritematososistémico: características clínicas e inmunológicas / A series of 115 patients with systemic lupus erythematosus: Clinical and immunological characteristics

Introducción: El lupus eritematoso sistémico es una enfermedad autoinmune, multisistémicade etiología desconocida, que afecta principalmente a mujeres en edad reproductiva. Dentrode las manifestaciones más frecuentes se encuentran los compromisos renal e inmunoló-gico; el primero es el responsable de gran parte de la morbimortalidad de los pacientes y elsegundo está fuertemente asociado a múltiples manifestaciones clínicas.Objetivo: Analizar la prevalencia y características de las principales manifestaciones clínicase inmunológicas de 115 pacientes con diagnóstico de lupus eritematoso sistémico del HospitalUniversitario San Vicente Fundación y establecer la asociación entre los anticuerposespecíficos y el compromiso de órgano.Materiales y métodos: Los pacientes fueron vistos por el Grupo de Reumatología de la Universidadde Antioquia entre 2008 y 2012. Los datos clínicos e inmunológicos se recolectaron delarchivo o sistema electrónico de historias clínicas del hospital, en un formato previamenteprotocolizado.Resultados y conclusiones: Similar a lo informado por otros estudios, se encontró asociaciónentre hipocomplementemia y compromiso renal, y mayor alteración de las pruebas defunción renal como creatinina, nitrógeno ureico en sangre, depuración de creatinina y proteinuriaen 24h. La frecuencia de anticuerpos anti-ADN de doble cadena fue del 79,1% y estosse encontraron asociados con compromiso renal y puntuaciones más altas de ®SystemicLupus Erythematosus Disease Activity Index¼. Se observó también asociación entre anticuerposanti-Sm con serositis y lupus discoide...

IntroductionSystemic lupus erythematosus is an autoimmune disease. The etiology is unknown, and it primarily affects women of reproductive age. Among the most common manifestations are the renal and immune system involvement; the first one is responsible for the majority of the morbidity and mortality of patients, and the second strongly associated with multiple clinical manifestations.ObjectiveTo analyze the prevalence and characteristics of the main clinical and immunological manifestations of 115 patients diagnosed with systemic lupus erythematosus in the Hospital Universitario San Vicente Fundación, and to determine the association between autoantibodies and organ involvement.Materials and methodsPatients were seen by the Rheumatology Group, University of Antioquia, between 2008 and 2012. Clinical and immunological data were collected from the files or electronic medical records using a previously designed format.Results and conclusionsThe study found an association between hypocomplementemia and renal involvement, similar to other studies. Nephritis was found in patients with active lupus, as well as a greater impairment of renal function tests such as, creatinine, blood urea nitrogen, creatinine clearance, and proteinuria. The percentage of patients with anti-dsDNA was 79.1%, and these antibodies were associated with renal involvement, as well as higher Systemic Lupus Erythematosus Disease Activity Index scores; anti-Sm antibodies was associated with serositis and discoid lupus...

Hemólisis, exantema, serositis. Manifestaciones extrapulmonares del Mycoplasma pneumoniae: Reporte de un caso / Hemolysis, serositis and exanthema induced by Mycoplasma pneumoniae infection: Report of one case

Mycoplasma infections have extrapulmonary manifestations that may be associated with respiratory symptoms and may have skin, heart, gastrointestinal, rheumatologic, neurologic, hematologic involvement. Cold agglutinin mediated autoimmune hemolytic anemia is the most common hematological manifestation. We report a 27-year-old woman infected with Mycoplasma pneumoniae, who presented respiratory involvement with pneumonia, exanthema, serositis and acute hemolytic anemia that required transfusion. The key for the diagnosis were the extrapulmonary manifestations associated with respiratory involvement after five days of hospitalization.

Lupus eritematoso sistémico en el anciano: una presentación atípica de una enfermedad común / Systemic lupus erythematosus in the elderly: An atypical presentation of a common disease

El lupus eritematoso sistémico (LES) del anciano, también llamado lupus de aparición tardía,es una enfermedad autoinmune que aparece después de los 50-60 años, con un curso clínicoy manifestaciones clínicas que difieren del LES clásico, cuya prevalencia es en personas másjóvenes, predominantemente mujeres. Se presenta, en este artículo, el caso de un pacientemasculino de 72 años con cuadro clínico de un mes de evolución de dolor en hemitóraxderecho, tipo pleurítico asociado a disnea, además de la presencia de lesiones eritematosasy descamativas en región malar y zona de exposición solar en tórax. Se descartó origeninfeccioso y neoplásico mediante imágenes diagnósticas y laboratorios y, posteriormente,se realiza perfil inmunológico que reporta ANAS positivo, Anti-DNA positivo y complementoconsumido, con evidencia de derrame pleural derecho masivo recidivante hasta la fecha.

Systemic lupus erythematosus (SLE) in the elderly, also called late-onset lupus, is an autoimmune disease that appears after 50-60 years old, with a clinical course and clinical manifestations that differ from classic SLE, with a prevalence predominantly in younger women. In this article a case of a 72 year-old male patient who, for one month, had clinical symptoms of right chest pain, associated with dyspnea and the presence of erythematous and scaly lesions on the malar area and sun exposure in the thorax. The possibilities of infectious or neoplastic origin were dismissed using diagnostic images and laboratory tests. An immunological profile was subsequently performed, reporting positive ANAS and Anti-DNA, positive, and consumed complement, with evidence of recurrent massive right pleural effusion to date.

Autoinflammatory Diseases

Autoinflammatory diseases (AIDs) refer to a broad range of genetically mediated conditions characterized by recurrent attacks of systemic inflammation with typical manifestations of fever, rash, serositis, lymphadenopathy, and musculoskeletal symptoms. The discovery of genetic basis for these conditions have led to the understanding of novel intracellular receptors for infectious and noninfectious danger signals in innate immunity. Innate immunity has a key role in the development of AIDs, in contrast with autoimmune disease, which arise from problems in adaptive immunity. Advances in understanding the molecular mechanisms of intracellular inflammatory cascades have led to renewed interest in its role in the pathogenesis of more common non-genetic autoinflammatory rheumatic conditions, such as Behcet's disease, gouty arthritis, Adult onset Still's diseases, and systemic onset juvenile arthritis. The characterization of cryopyrin (inflammasome) and its significant role in the activation of proinflammatory cytokines, such as IL-1beta and TNF-alpha in the development of AIDs, has provided rational targets of anti-cytokine biologic treatment for some of these conditions.

Systemic Lupus Erythematosus with Initial Presentation of Ascites / 이화의대지

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown etiology and is characterized by presence of variable pathogenic auto-antibodies and multiple organ involvement. Serositis is common in SLE, but peritoneal involvement is relatively rare. This is a case report of 28-year-old female who initially presented with abdominal pain and ascites. After ruling out many other possibilities such as liver cirrhosis, neoplasm, and infectious etiologies, we confirmed SLE with clinical features, serologic tests and radiological findings. To conclude, her abdominal pain and ascites were caused by lupus peritonitis. After administration of corticosteroid therapy, her symptoms fairly improved.

An Unusual Case of Severe Neonatal Lupus Mimicking Neonatal Sepsis and Literature Review

Neonatal lupus is an uncommon autoimmune disease that results from transplacental passage of the maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Pancytopenia or severe jaundice is a rare manifestation of neonatal lupus, respectively, and could be misdiagnosed with other neonatal illnesses, such as infection, hematologic disease, or hepatobiliary disease. Here, we report an unusual case of a premature newborn with severe neonatal lupus manifested with skin rash, fever, pancytopenia, and severe jaundice with abnormal liver function tests. His mother had been clinically asymptomatic before delivery; however, she revealed peripheral edema, bilateral pleural effusion, and ascites after delivery and diagnosed with systemic lupus erythematosus based on positive anti-nuclear and anti-cardiolipin antibodies, proteinuria, and serositis. The newborn and his mother had anti-SSA/Ro and anti-SSB/La antibodies. His pancytopenia and jaundice were progressively aggravated, and his illness was confused with neonatal sepsis or biliary obstruction. We decided to treat with high dose of corticosteroid and intravenous immunoglobulin, and he gradually recovered completely with the treatment. His corticosteroid was stopped at 5 months of age without relapse or complication.

A Case of Rheumatoid Pneumoconiosis Presenting with Pleuritis and Pericarditis / 대한내과학회지

Caplan's syndrome is characterized by multiple small distinct nodules with progressive massive fibrosis and rheumatic arthritis in pneumoconiosis. Although pleural effusions occur infrequently as an extra-articular manifestation, pleuritis can develop without joint involvement in patients with rheumatoid arthritis. We treated an 81-year-old man who had been diagnosed with silicosis with progressive massive fibrosis. He suffered from progressive dyspnea, and chest computed tomography (CT) and echocardiography revealed pleural and pericardial effusions. We speculated that the multiple serositis was related to a rheumatic disorder because the rheumatic factor was elevated in both the pleural and pericardial effusions. After corticosteroid treatment, the serositis improved. We suggest that this case is an atypical pattern of Caplan's syndrome presenting as serositis without arthritis. Rheumatoid serositis should be considered as the cause of pleural or pericardial effusions in patients with pneumoconiosis.