Sarcoidosis en paciente adulto joven / Sarcoidosis in a young adult patient

RESUMEN La sarcoidosis es una enfermedad inflamatoria crónica caracterizada por la prevalencia de granulomas no caseosos que puede afectar múltiples órganos. Presentamos el caso de un varón de 35 años de edad, sin patologías conocidas previamente, albañil, procedente de zona urbana. Presenta un cuadro de 4 meses de evolución que inicia con disnea y tos seca, además de sensación febril y pérdida de peso. En la tomografía de tórax simple se visual condensaciones parahiliares y múltiples lesiones nodulares y micronodulares sólidas con claro predominio en lóbulos superiores. La histopatología informa granulomas no caseosos en la muestra de tejido pulmonar. Se instaura tratamiento con corticoides sistémicos con notable mejoría de los síntomas respiratorios.

ABSTRACT Sarcoidosis is a chronic inflammatory disease characterized by the prevalence of noncaseating granulomas that can affect multiple organs. We present the case of a 35-year-old male, with no previously known pathologies, a bricklayer, from an urban area. The patient presents a 4-month history that begins with dyspnea and dry cough, as well as a feverish feeling and weight loss. The simple chest tomography showed parahilar condensations and multiple solid nodular and micronodular lesions with a clear predominance in the upper lobes. Histopathology reports non-caseating granulomas in the lung tissue sample. Treatment with systemic corticosteroids was instituted with notable improvement in respiratory symptoms.

Algunas precisiones a un artículo sobre las enfermedades otorrinolaringológicas padecidas por José Martí

José Martí, el más universal de los cubanos, el hombre que unió a sus compatriotas en el exilio y organizó la Guerra Necesaria, padeció varias entidades patológicas, entre ellas algunas de tipo otorrinolaringológico; pero estas no lograron detenerlo en su labor por alcanzar una patria libre. En el presente trabajo se precisan algunos deslices encontrados en una publicación donde se exponen las enfermedades otorrinolaringológicas que aquejaban al Apóstol.

José Martí, the most universal of Cubans, the man who united his compatriots in exile and organized The Necessary War, suffered from several pathological entities, including some of the otorhinolaryngological type; but these illnesses did not stop him in his work to achieve a free homeland. In the present work, some errors found in a publication that exposes the otorhinolaryngological diseases that afflicted the Apostle are specified.

A case report of multisystemic sarcoidosis with extracardiac shunt

A 61-year-old female patient, presented with complaints of dry cough and gradually worsening dyspnea of 3 months duration, associated with significant weight loss of >14 kg. At presentation, patient was sick, hypoxemic, and tachypneic and system examination revealed fine basal crepitations on auscultation. CT chest showed random pulmonary nodules with bronchiectatic changes. In view of respiratory distress and elevated d-dimer, CT pulmonary angiogram was done and was normal. A contrast echocardiography study hinted towards a significant right to left extracardiac shunt. There was radiological evidence of chronic liver disease with portal hypertension and numerous poorly defined nodular opacities scattered in hepatic and splenic parenchyma in post contrast images. ANA profile was positive for ribosomal-p protein along with elevated serum ACE levels. An ultrasound guided liver biopsy was ultimately performed and histopathology revealed granulomatous lesion in liver compatible with sarcoidosis.

Effect of rehabilitation exercise combined with resistance training on the recovery of patients with pulmonary nodules following surgical intervention / 中国基层医药

Objective:To investigate the effect of rehabilitation exercise combined with resistance training on the postoperative recovery and quality of life among patients with pulmonary nodules.Methods:A randomized controlled study was conducted on 90 patients with pulmonary nodules who underwent thoracoscopic resection of pulmonary nodules at Zhejiang Veteran Hospital between January 2022 and April 2023. Patients were randomly allocated into an observation group and a control group, with 45 patients in each group using the random number table method. The control group underwent routine rehabilitation exercise, whereas the observation group received resistance training combined with routine rehabilitation exercise. All patients were treated for 1 week. The incidence of postoperative complications and the changes in lung function, exercise endurance, and quality of life from baseline levels were compared between the two groups.Results:The incidence of postoperative complications in the observation group was significantly lower than that in the control group [6.7% (3/45) vs. 24.4% (11/45), χ2 = 5.41, P = 0.020). After intervention, the forced expiratory volume in 1 second, maximal voluntary ventilation per minute, and forced vital capacity in the observation group were (83.84 ± 4.35)%, (96.53 ± 3.45) L/min, and (2.87 ± 0.16) L, respectively, which were higher than those in the control group [(78.98 ± 4.01)%, (92.13 ± 3.08) L/min, (2.62 ± 0.19) L, t = -5.51, -6.38, -6.75, all P < 0.001]. Additionally, the modified Medical Research Council dyspnea score in the observation group was (0.42 ± 0.13) points, which was significantly lower than that in the control group [(0.87 ± 0.19) points, t = 13.11, P < 0.001). The modified Barthel index score in the observation group was significantly higher than that in the control group [(89.53 ± 3.67) points vs. (82.94 ± 4.23) points, t = -7.89, P < 0.001). Conclusion:The combination of rehabilitation exercise and resistance training can effectively enhance lung function, exercise endurance, and overall quality of life in patients with pulmonary nodules. Furthermore, this combined therapy markedly reduces postoperative complications.

Analysis on Pathogenesis and Treatment of Pulmonary Sarcoidosis Based on the Theory of"Yang Leading to Qi and Yin Leading to Formation" / 中国中医药信息杂志

Theory of"yang leading to qi and yin leading to formation",as the law of nature giving birth to life,has wide applicability and can be used to interpret the nature and pathogenesis characteristics of pulmonary sarcoidosis.Based on yin-yang theory,this article elaborated on the pathological essence of pulmonary sarcoidosis.Under the guidance of the theory of"yang leading to qi and yin leading to formation",the treatment strategy of discerning the degree of yang qi deficiency and stagnation when forming pulmonary sarcoidosis,replenishing and unblocking the body's yang qi,and simultaneously soothing the liver and lungs,clearing collaterals,detoxifying,and dispersing masses to dissipate yin and coagulation,has certain guiding significance for clinical diagnosis and treatment of pulmonary sarcoidosis.

Relative incidence of interstitial lung diseases in Brazil / Incidência relativa de doenças pulmonares intersticiais no Brasil

ABSTRACT Objective: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. Methods: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. Results: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). Conclusions: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

RESUMO Objetivo: Avaliar a frequência relativa de casos incidentes de doenças pulmonares intersticiais (DPI) no Brasil. Métodos: Levantamento retrospectivo de casos novos de DPI em seis centros de referência entre janeiro de 2013 e janeiro de 2020. O diagnóstico de DPI seguiu os critérios sugeridos por órgãos internacionais ou foi feito por meio de discussão multidisciplinar (DMD). A condição foi caracterizada como DPI não classificável quando não houve um diagnóstico final específico após a DMD ou houve discordância entre dados clínicos, radiológicos ou histológicos. Resultados: A amostra foi composta por 1.406 pacientes (média de idade = 61 ± 14 anos), sendo 764 (54%) do sexo feminino. Dos 747 casos expostos a antígenos para pneumonite de hipersensibilidade (PH), 327 (44%) tiveram diagnóstico final de PH. Houve relato de história familiar de DPI em 8% dos casos. Os achados de TCAR foram indicativos de fibrose em 74% dos casos, incluindo faveolamento, em 21%. Autoanticorpos relevantes foram detectados em 33% dos casos. Biópsia transbrônquica foi realizada em 23% dos pacientes, e biópsia pulmonar cirúrgica, em 17%. Os diagnósticos finais foram: DPI associada à doença do tecido conjuntivo (em 27%), PH (em 23%), fibrose pulmonar idiopática (em 14%), DPI não classificável (em 10%) e sarcoidose (em 6%). Os diagnósticos variaram significativamente entre os centros (c2 = 312,4; p < 0,001). Conclusões: Nossos achados mostram que DPI associada à doença do tecido conjuntivo é a DPI mais comum no Brasil, seguida pela PH. Esses resultados destacam a necessidade de uma estreita colaboração entre pneumologistas e reumatologistas, a importância de fazer perguntas detalhadas aos pacientes a respeito da potencial exposição a antígenos e a necessidade de campanhas de saúde pública destinadas a enfatizar a importância de evitar essa exposição.

Sarcoidosis ocupacional o ambiental: diagnóstico enigmático con muchos progenitores para un mismo síndrome / Occupational or environmental sarcoidosis: enigmatic diagnosis with many parents to the same syndrome

RESUMEN Es conocida desde hace más de veinte años, la posibilidad de que fármacos o anti cuerpos monoclonales utilizados en el tratamiento de diferentes enfermedades pueden ocasionar la aparición de un síndrome símil sarcoidosis. Ciertas líneas de evidencia han sugerido que determinados lugares de trabajo están asociados con el riesgo de sarcoidosis En sarcoidosis, diferentes exposiciones pueden estar relacionadas con otros compromi sos viscerales. La afección presenta más de una causa y provocar fenómenos disímiles de la enfermedad o fenotipo. De las enfermedades ocupacionales de pulmón, la que más se asemeja es la beriliosis. Cuando la causa es de origen laboral, su reconocimiento es primordial para admitir un tratamiento eficaz mediante el retiro del trabajador afectado de la exposición y para establecer una intervención dirigida a la prevención primaria mediante las secciones especializadas en seguridad e higiene industrial. Por lo tanto, y por lo expuesto, debe considerarse que la sarcoidosis es un síndrome con cuantiosos factores etiológicos probables, a lo que hay que asociar tanto el fenotipo como la sensibilidad individual ante una noxa determinada. Esto hace que, en forma periódica, se publiquen casos en los que la clínica y el cuadro histológico de granuloma epitelioide no caseoso hallado en biopsias, se vincule a un determinado riesgo ambiental y/o laboral.

SUMMARY It has been known for more than 20 years, the possibility that drugs or monoclonal anti bodies used in the treatment of different diseases can cause a Sarcoidosis-like syndrome. Certain lines of evidence have suggested that certain workplaces are associated with the risk of Sarcoidosis. In Sarcoidosis, different exposures may be related to visceral involvement. The condition has more than one cause and brings about dissimilar pheno mena of the disease or phenotype. Of the occupational lung diseases, the one that most closely resembles it, is Berylliosis. When the cause is of occupational origin, its recognition is essential to allow effective treatment by removing the affected worker from exposure and to establish an intervention aimed at primary prevention through sections specialized in industrial hygiene and security. Therefore, it must be considered that Sarcoidosis is a syndrome with nume rous probable etiological factors, to which both the phenotype and individual susceptibility to a given toxic effect must be associated. Due to what has been stated, this means that cases are periodically publis hed in which the clinical and histological features of non-caseous epithelioid granuloma found in biopsies are linked to a certain environmental and/or occupational risk.

Neurosarcoidosis: inicio con compromiso de múltiples sitios neurológicos / Neurosarcoidosis: Onset with involvement of multiple neurological sites

Resumen Presentamos el caso de una mujer joven sana, que consultó por parálisis facial periférica izquierda asociada a fiebre, tos seca, disnea y astenia de dos semanas de evolución. Al examen físico se evidenció hipoestesia en dermatomas D6 a D12 izquierdos y galactorrea bi lateral. En el laboratorio presentaba serologías virales negativas, eritrosedimentación, títulos de anticuerpos antinucleares, prolactina y hormona tiroestimulante elevados, con anticuerpos antiperoxidasa positivos. La tomografía computarizada mostró múltiples adenopatías cervicales, mediastinales e hiliares bilaterales, sin com promiso del parénquima pulmonar. El cultivo de líquido cefalorraquídeo fue negativo para gérmenes comunes, micobacterias (Xpert MTB/RIF), y la citología no mostró atipia. Se realizó una resonancia magnética con con traste endovenoso de cerebro sin hallazgos patológicos y de columna con alteración de la señal centromedular de D6 a D9 de casi la totalidad del espesor del cordón, con refuerzo con contraste endovenoso. Durante la in ternación recuperó la sensibilidad en tronco izquierdo y no repitió episodios febriles o tusígenos. Se realizó mediastinoscopía con biopsia ganglionar con anatomía patológica con presencia de numerosos granulomas no caseificantes compatibles con sarcoidosis. Se clasificó como neurosarcoidosis probable e inició tratamiento con corticoides con mejoría de los síntomas neurológi cos restantes, realizándose una resonancia magnética a los tres meses, donde la alteración de la señal se limitaba desde D7 a D8. Nuestro objetivo es destacar la presentación neurológica en múltiples sitios que obligó a descartar otras entidades más frecuentes, así como la evolución favorable incluso previo al inicio de un esquema de tratamiento de primera línea.

Abstract We present the case of a healthy young woman who consulted for left peripheral facial palsy associated with fever, dry cough, dyspnea, and asthenia of two weeks' evolution. Physical examination revealed hypoesthesia in left T6 to T12 dermatomes and bilateral galactorrhea. In the laboratory, she presented negative viral serology, elevated erythrocyte sedimentation rate, antinuclear an tibody titers, prolactin and thyroid-stimulating hormone, with positive antiperoxidase antibodies. Computed to mography showed multiple bilateral cervical, mediastinal, and hilar adenopathies, without involvement of lung parenchyma. Cerebrospinal fluid culture was negative for common germs, mycobacteria, and Xpert MTB/RIF, and cytology did not show atypia. Contrast-enhanced magnetic resonance was performed on the brain without pathological findings and on the spine with alteration of the centromedullary signal from T6 to T9 of almost the entire thickness of the cord, with posterior enhancement with gadolinium. During hospitalization, she recovered sensitivity in the left trunk and did not repeat febrile or cough episodes. She was referred to another center for mediastinoscopy with lymph node biopsy revealing the presence of numerous non-caseating granulomas compatible with sarcoidosis. It was classified as probable neurosarcoidosis and started treatment with corticoste roids with improvement of the remaining neurological symptoms. A magnetic resonance was performed three months later where the signal alteration was limited from T7 to T8. Our objective is to highlight the florid neu rological presentation that made it necessary to rule out other more frequent entities and the favorable evolution even before starting a first-line scheme of treatment.

Sarcoidosis presenting as ovarian mass with omental and peritoneal nodules with elevated CA 125 level; mimicking malignancy

Sarcoidosis is a systemic inflammatory disease of unknown aetiology with a variety of nonspecific clinicoradiological features making diagnosis challenging. Sarcoidosis commonly involves pulmonary and lymphoreticular systems; ovarian involvement being extremely rare. We present a case of a 50-year Indian postmenopausal female, with a history of abdominal pain and distension, referred from the peripheral hospital for management of a solid complex left adnexal mass and elevated CA 125 levels. Based on ultrasonography and contrast-enhanced computed tomography, ovarian carcinoma was a differential diagnosis. Ascitic and pleural fluid cytology and ovarian fine needle aspiration cytology (FNAC) didn抰 give any conclusive diagnosis. Hence surgical intervention was carried out. Histopathology revealed noncaseating granulomas in ovaries, peritoneum, and omentum. Tissue polymerase chain reaction (PCR) for tuberculous and nontuberculous mycobacteria was negative. Post-operatively serum angiotensin-converting enzyme (ACE) and calcium levels were elevated. These findings supported the diagnosis of sarcoidosis and the patient responded well to systemic steroids.

Granulomatous hepatitis, deciphering an elusive spectrum: a clinical case-based approach

Granulomatous hepatitis is a well-defined histopathologic entity characterized by aggregates of modified macrophages (epithelioid in appearance) and other inflammatory cells that accumulate after persistent exposure to antigens. It induces a cellular immune response mediated by the release of various cytokines (including interferon-gamma [INF-γ], tumor necrosis factor-alpha [TNF-α], and interleukin 12 [IL-12]) that stimulate mononuclear cell fusion, culminating in the formation of multinucleated giant cells with a surrounding border of lymphocytes and fibroblasts. It represents between 2% and 15% of all pathological liver studies, usually during an infiltrative or cholestatic biochemical pattern. A practical approach is proposed based on a challenging clinical case of a patient with a fever of unknown origin in an intermediate incidence area for tuberculosis, such as Colombia.

La hepatitis granulomatosa es una entidad histopatológica bien definida que se caracteriza por la presencia de agregados de macrófagos modificados (de aspecto epitelioide) y otras células inflamatorias que se acumulan luego de la exposición persistente a antígenos, lo que induce una respuesta inmunitaria celular mediada por la liberación de varias citocinas (entre las que se destacan el interferón gamma [INF-γ], el factor de necrosis tumoral alfa [TNF-α] y la interleucina 12 [IL-12]) que estimulan la fusión de células mononucleares para culminar en la formación de células gigantes multinucleadas con un borde circundante de linfocitos y fibroblastos. Representa entre el 2 % y el 15 % de todos los estudios anatomopatológicos de hígado, en el curso por lo general de un patrón bioquímico de tipo infiltrativo o colestásico. Se propone un enfoque práctico a partir de un caso clínico retador de un paciente con fiebre de origen desconocido en una zona de incidencia intermedia para tuberculosis, como lo es Colombia.

Implementation of positron emission tomography with 18F-fluorodeoxyglucose associated with computed tomography in patients with sarcoidosis. Its effect on disease staging and the therapeutic approach

Objetives: to evaluate the benefit of implementing 18F-FDG PET/TC in the staging and treatment adjustment of patients with sarcoidosis, compared with the signs and symptoms and complementary test results usually employed. Materials and methods: an observational, analytical electronic chart review of a retrospective cohort of patients seen for sarcoidosis in the internal medicine department of a Spanish university hospital. Results: a total of 31 patients (18 males) were evaluated, with an average age of 54.6±14.71 years and 11±5.75 years since their sarcoidosis diagnosis. In the 84.6% of the reviews, positive uptake was objectified on the 18F-FDG PET/TC. In the 42.3% of the occasions, the objectified find ing allowed restaging of the patient. The 18F-FDG PET/TC result justified the choice of treatment in the 71% of the reviews. Conclusions: 18F-FDG PET/TC provided additional advantages in the staging and therapeutic management of patients with sarcoidosis, compared with the evaluation of signs and symptoms and other clinical tests usually employed in follow up, due to its greater accuracy in determining the activity and extension of the disease. (Acta Med Colomb 2022; 48. DOI: https://doi.org/10.36104/amc.2023.2778).

A rare case of patient with severe thrombocytopenia associated with sarcoidosis: a case study

Thrombocytopenia is a problem causing drop in platelet counts through different mechanisms. Patients typically present with petechiae to systemic bleeding, which are indications of a low platelet count. A smaller percentage of drop may be asymptomatic. The major mechanisms include increased sequestration in the spleen, underproduction from the bone marrow, and peripheral destruction. Many etiological factors can cause this. Sarcoidosis is one of the most uncommon etiologies. A careful diagnosis is required because, if the condition is not treated, it can be fatal. Steroid therapy and platelet transfusions remain the mainstay of treatment. Here, we describe a patient who presented with signs and symptoms of severe thrombocytopenia, which further led to the diagnosis of sarcoidosis. An adult male presented to the emergency department with a rash that deteriorated throughout the day. Based on complaints and laboratory testing, severe thrombocytopenia was noted. After a detailed examination and history-taking, he was found to have sarcoidosis. In association with drug administration and sarcoidosis, this could have caused severe thrombocytopenia. Written consent was taken from the patient mentioned in the study. The study was approved by the hospital and institutional ethics committee. Thrombocytopenia is a disorder where platelet counts drop below 150×109/l due to many different mechanisms. Among different etiological factors, sarcoidosis is the rarest and may present with very severe thrombocytopenia and lead to fatal complications. Such patients require close monitoring and treatment. Corticosteroids and platelet transfusions can be used as treatments. In the presented case, the patient was successfully treated, and on subsequent follow-up, the patient’s condition improved. Manifestations of severe thrombocytopenia can be present even before the diagnosis of sarcoidosis. Further, the history of amoxicillin administration due to infection could have triggered the appearance of thrombocytopenia. Confirmation of sarcoidosis was made via biopsy. Multiple etiological factors that resulted in diagnostic ambiguity in our patient's presentation include the diagnosis of sarcoidosis, a history of infection, amoxicillin, and mild splenomegaly. The patient's treatment and recovery may indicate that corticosteroids, in conjunction with platelet transfusions, are beneficial. This is a novel case report of the presentation of severe thrombocytopenia, which was present even before the diagnosis of sarcoidosis.

A rare case of patient with severe thrombocytopenia associated with sarcoidosis: a case study

Thrombocytopenia is a problem causing drop in platelet counts through different mechanisms. Patients typically present with petechiae to systemic bleeding, which are indications of a low platelet count. A smaller percentage of drop may be asymptomatic. The major mechanisms include increased sequestration in the spleen, underproduction from the bone marrow, and peripheral destruction. Many etiological factors can cause this. Sarcoidosis is one of the most uncommon etiologies. A careful diagnosis is required because, if the condition is not treated, it can be fatal. Steroid therapy and platelet transfusions remain the mainstay of treatment. Here, we describe a patient who presented with signs and symptoms of severe thrombocytopenia, which further led to the diagnosis of sarcoidosis. An adult male presented to the emergency department with a rash that deteriorated throughout the day. Based on complaints and laboratory testing, severe thrombocytopenia was noted. After a detailed examination and history-taking, he was found to have sarcoidosis. In association with drug administration and sarcoidosis, this could have caused severe thrombocytopenia. Written consent was taken from the patient mentioned in the study. The study was approved by the hospital and institutional ethics committee. Thrombocytopenia is a disorder where platelet counts drop below 150×109/l due to many different mechanisms. Among different etiological factors, sarcoidosis is the rarest and may present with very severe thrombocytopenia and lead to fatal complications. Such patients require close monitoring and treatment. Corticosteroids and platelet transfusions can be used as treatments. In the presented case, the patient was successfully treated, and on subsequent follow-up, the patient’s condition improved. Manifestations of severe thrombocytopenia can be present even before the diagnosis of sarcoidosis. Further, the history of amoxicillin administration due to infection could have triggered the appearance of thrombocytopenia. Confirmation of sarcoidosis was made via biopsy. Multiple etiological factors that resulted in diagnostic ambiguity in our patient's presentation include the diagnosis of sarcoidosis, a history of infection, amoxicillin, and mild splenomegaly. The patient's treatment and recovery may indicate that corticosteroids, in conjunction with platelet transfusions, are beneficial. This is a novel case report of the presentation of severe thrombocytopenia, which was present even before the diagnosis of sarcoidosis.

Evaluation of Immunoglobulin Levels in Lavage Fluid in Active and Inactive Disease

Background: Fluid obtained by whole gut lavage usually contains traces of immunoglobulin (lg) G, albumin, and a-1-antitrypsin; higher concentrations have been found in patients with inflammatory bowel disease (IBD). Immunoglobulin (lg) levels increase in the lower respiratory tract of patients with pulmonary sarcoidosis. The aim of this study is to assess the Evaluation of immunoglobulin levels in lavage fluid in active and inactive disease. Material & Methods: This is an observational study. The study used to be carried out in the admitted patient’s Department of microbiology and immunology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Bangladesh. In Bangladesh for the duration of the period from October 2015 to March 2017. Results: This study shows that the according to age of 33 Patients aged 1 to 9 years. Here according to Age distribution, 2(6.1%) were 1-3 years, 10(30.30%) were >3 6 years, 9(27.27%) were >6-9 years and 12(36.4%) were >9 years. And according to gender 13(39.4%) were Male and 20(60.6%) were Female. Conclusion: The study concluded that high and abnormal levels of immunoglobulin (IgG, IgM, and IgA) is present among JIA patient in active disease state which became normal in inactive state.

Systemic sarcoidosis presenting as acute rapidly progressive proptosis / Sarcoidose sistêmica apresentando-se como proptose aguda rapidamente progressiva

ABSTRACT Sarcoidosis is a generalized systemic chronic inflammation that rarely involves the orbit. As a chronic inflammation, sarcoidosis typically manifests with an insidious onset and slowly progressive course. We report a case of acute-onset proptosis resulting from a rapidly growing diffuse orbital mass that simulated malignant growth, which was biopsy proven to be the first manifestation of systemic sarcoidosis. The patient demonstrated complete resolution of proptosis and systemic involvement with long-term corticosteroid treatment.

RESUMO A sarcoidose é uma inflamação sistêmica generalizada que raramente envolve a órbita. Sendo uma inflamação crônica, a sarcoidose geralmente se manifesta com um início insidioso e uma evolução lentamente progressiva. Neste artigo, é relatado um caso de proptose de início agudo, resultante de uma massa orbitária difusa de rápido crescimento, simulando um crescimento maligno, mas cuja biópsia comprovou ser a primeira manifestação de uma sarcoidose sistêmica. O paciente apresentou resolução completa da proptose e do envolvimento sistêmico com tratamento de longo prazo com corticosteroides.

Sarcoidosis cutánea. Presentación de un caso

La sarcoidosis es una enfermedad granulomatosa sistémica de causa desconocida, con mayor prevalencia en el sexo femenino, entre los 20 a 40 años de edad; caracterizada por la formación de granulomas no caseificantes en distintos órganos. La afectación cutánea de forma exclusiva es rara, pues se describe solo en el 4-5 % de los pacientes, ya que las lesiones en piel pueden aparecer antes o después del compromiso sistémico, o bien coincidir con este. Se presenta el caso de una paciente de 35 años con lesiones en piel de cuatro años de evolución. Considerando el cuadro clínico y la histopatología, se concluyó con el diagnóstico de sarcoidosis cutánea, crónica y asintomática. Se trata de una enfermedad muy polimorfa en sus manifestaciones cutáneas, lo cual la convierte en una gran simuladora, y su diagnóstico constituye un reto para el dermatólogo.

Sarcoidosis is a systemic granulomatous disease of unknown cause, with a higher prevalence in females, between 20 and 40 years of age; characterized by the formation of non-caseating granulomas in different organs. Exclusive skin involvement is rare, as it is described in only 4-5% of patients, since skin lesions may appear before or after systemic involvement, or coincide with it. The case of a 35-years-old patient with skin lesions of four years of evolution is presented. Considering the clinical case and histopathology, the diagnosis of chronic and asymptomatic cutaneous sarcoidosis was concluded. It is a very polymorphic disease in its skin manifestations, which makes it a great simulator, and its diagnosis constitutes a challenge for the dermatologist.

Analysis of 8 Cases of Blau Syndrome/Early-onset Sarcoidosis——Clinical Manifestations, Histopathology Features and Gene Mutation Diversity / 罕见病研究

  Objective  To summarize the clinical manifestations, pathological features and gene mutation diversity of Blau syndrome/early-onset sarcoidosis.  Methods  We collected general data, clinical manifestations, and auxiliary examination results from 8 patients who were diagnosed of Blau syndrome/early-onset sarcoidosis and treated in our hospital from January 2011 to December 2022, and then summarized and analyzed their characteristics and diversity.  Results  Among the 8 patients, 4 were males and 4 were females. The onset age was 3 to 8 months old. Rash was the first symptom in 7 patients(87.5%). 6 patients(75.0%) had papules and erythema.3 cases(37.5%) had arthritis. 2 cases(25.0%) had uveitis and other eye inflammation. 4 cases (50.0%) also showed intermittent fever. 3 cases (37.5%) showed symptoms in nerve and respiratory system, and hypertension respectively. The skin histopathology of 8 patients showed non-caseous granuloma formation. In laboratory detection, CRP and TNF-α were significantly increased before treatment, while IL-6, IL-8, TNF-α and IL-2 receptor(IL-2R) were significantly decreased in 5 patients after glucocorticoid therapy. The results of genetic testing showed that 4 of the 7 patients had p.R334W(c.1000C > T) mutation, 1 had p.H313R(c.938A > G) and p.R471C(c.1411C > T)double mutation, and 1 had p.476_477del (c.1427_1429delcct).  Conclusions  Blau syndrome/early-onset sarcoidosis has significant features in clinical manifestations, histopathology and gene mutation, but it also has diversity.

Diagnosis and surgical treatment of pulmonary nodules / 国际外科学杂志

With the widespread application of low-dose CT in lung cancer screening, more and more pulmonary nodules are detected. Early diagnosis and treatment of pulmonary nodules can significantly reduce the risk of death from lung cancer. However, a series of problems still need to be solved in the early diagnosis and treatment of lung cancer, such as how to avoid over-diagnosis or under-diagnosis through necessary and accurate examination, how to determine the timing of surgery and how to adopt the best surgical method. This article will focus on the diagnostic value of imaging examination, tumor markers and invasive examination in the evaluation of benign and malignant pulmonary nodules, as well as the key clinical issues such as timing, method and extent of resection in the surgical treatment of pulmonary nodules, sorting out the clinical diagnosis and treatment ideas, and discussing management strategies, so as to provide references for the standardized clinical diagnosis and treatment of pulmonary nodules.

Research progress in animal models of sarcoidosis / 中国病理生理杂志

Sarcoidosis is a systemic disease of unknown etiology characterized by the formation of noncaseating granulomas.Its clinical manifestations are heterogeneous,and the determinants of clinical course(such as active vs inac-tive,remission vs chronic progression,and fibrosis vs non-fibrosis)are poorly understood.Despite considerable effort over many years,the exact pathogenesis of sarcoidosis has not been fully elucidated.Animal models have made significant con-tributions to understanding the etiology and the development of this disease.In this review,we presented the clinical rele-vance of animal models in sarcoidosis,summarized the methods for constructing the models,and discussed how they have strengthened our understanding of sarcoidosis.

Value of the Brock model and the Lung Reporting and Data System in the differential diagnosis of benign and malignant pulmonary nodules / 中国基层医药

Objective:To investigate the value of Brock model and the Lung Reporting and Data System (LU-RADS) in the differential diagnosis of benign and malignant pulmonary nodules.Methods:A total of 84 patients with pulmonary nodules who received treatment at Zhejiang Jinhua Guangfu Tumor Hospital from March 2021 to March 2022 were retrospectively included in this study. The patients were divided into a benign group ( n = 26) and a malignant group ( n = 58) based on their pathological examination results. The Brock model parameters and LU-RADS classification were compared between the two groups. The value of the Brock model and the LU-RADS in the differential diagnosis of benign and malignant pulmonary nodules was analyzed. Results:In the malignant group, the the Brock model parameters including age, nodule diameter, the presence of spicule signs surrounding the nodule, the composition of mixed ground glass nodules, the proportion of multiple nodules, and the probability of malignancy were (56.25 ± 4.15) years, (14.65 ± 2.35) mm, 44.8%, 77.6% , 51.7%, and (0.26 ± 0.11), respectively. These parameters were (47.35 ± 3.56) years, (8.92 ± 1.65) mm, 15.4%, 53.8%, 26.9%, and (0.05 ± 0.02) in the benign group. There were significant differences in these parameters between the two groups ( t = 9.48, 11.18, χ2 = 6.78, 4.84, 4.48, t = 9.63, all P < 0.05). The proportion of malignant nodules under LU-RADS classification in the malignant group was 79.3%, which was significantly higher than 42.3% in the benign group ( χ2 = 11.27, P < 0.05). According to the Receiver Operating Characteristic (ROC) curve, the Brock model outperformed LU-RADS in the differential diagnosis of benign and malignant pulmonary nodules. Conclusion:Compared with LU-RADS classification, the Brock model demonstrates higher value in the differential diagnosis of benign and malignant pulmonary nodules, and this evaluation method can be prioritized in clinical practice.