A. V. Novikova, N. Pravdyuk, E. Shmidt, A. Raksha, M. R. Mazra, A. R. Yunyaev, J. S. Zhulina
{"title":"一例肉芽肿病合并多血管炎:多学科医院快速诊断的最佳可能性","authors":"A. V. Novikova, N. Pravdyuk, E. Shmidt, A. Raksha, M. R. Mazra, A. R. Yunyaev, J. S. Zhulina","doi":"10.17650/1818-8338-2022-16-4-k673","DOIUrl":null,"url":null,"abstract":"Introduction. One of the distinguishing features of systemic vasculitis is their manifestation under the guise of a lesion of one or another organ system, which is often multi-organ in nature with signs of systemic inflammation. The latter is interpreted primarily as part of an infectious or paraneoplastic process, which causes a delay in the diagnosis.The aim of the study was to present the diversity of the clinical picture in vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), the speed and large volume of diagnostic measures with the effective cooperation of therapeutic and surgical specialists, radiologists on the way to verifying granulomatosis with polyangiitis in a young woman.Materials and methods. Patient K., 46 y. o., was hospitalized in the Otolaryngology Department of the N.I. Pirogov City Clinical Hospital No. 1 of the Moscow Health Department with complaints of hearing loss, pain and stuffiness in the left ear, unproductive cough, hoarseness and fever up to 38.5 °C. According to the radiography (RG) of the chest organs, right-sided pneumonia was detected. Conducted antibiotic therapy without effect. As part of the differential diagnostic search, the following nosologies were excluded: infective endocarditis, sepsis, tuberculosis, primary multiple or central lung cancer complicated by paracancer pneumonia, metastatic lesion, infectious, brucellosis spondylodiscitis.Results. In the blood test, attention was drawn to a decrease in the level of hemoglobin to 111 g / l, an increase in the rate of erythrocyte sedimentation to 45 mm / h and the level of C-reactive protein to 142 mg / l, microhematuria according to the general urine analysis. Instrumental research methods – RG of 16.02.22, MSCT of the chest organs on 17.02.22, 27.02.22, 10.03.22 showed progressive bilateral focal pneumonia with a focus of consolidation in the middle lobe, EchoCG, ultrasound of the abdominal cavity and small pelvis, RG of the temporal bone, bronchoscopy with bronchoalveolar lavage and microscopic analysis, for atypia and bacteriological culture. A gynecological examination and a smear from the cervical canal for microscopic analysis were performed, atypical cells, consulted by a phthisiatrician (no data for tuberculosis), consulted three times by a thoracic surgeon (exclusion of volumetric formation of the middle lobe of the right lung). Given the history and clinical presentation (female gender, young age, bilateral otitis media, hoarseness, and destructive nature of pneumonia), granulomatosis with polyangiitis was suspected, and tests for ANCA were prescribed. A transthoracic biopsy of the right lung was performed. A rheumatologist prescribed induction pulse therapy with corticosteroids, and after serological and histological confirmation (antibodies to Proteinase-3 Anti-PR3 > 200 IU / ml, productive pneumonitis, granulomas without signs of tuberculosis), immunosuppressive therapy with cyclophosphamide. Against the background of pathogenetic treatment, a pronounced clinical and laboratory effect was noted.Conclusion. In this clinical situation, the simultaneous involvement of specialists of various profiles, the performance of a large number of laboratory and instrumental studies in dynamics, the absence of delay in histological verification made it possible to quickly exclude common diseases in the population and suspect systemic vasculitis, establishing a correct diagnosis within 5 weeks of the hospitalization period.","PeriodicalId":82998,"journal":{"name":"The Clinician","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Case of granulomatosis with polyangiitis: optimal possibilities for rapid diagnosis in a multidisciplinary hospital\",\"authors\":\"A. V. Novikova, N. Pravdyuk, E. Shmidt, A. Raksha, M. R. Mazra, A. R. Yunyaev, J. S. Zhulina\",\"doi\":\"10.17650/1818-8338-2022-16-4-k673\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Introduction. One of the distinguishing features of systemic vasculitis is their manifestation under the guise of a lesion of one or another organ system, which is often multi-organ in nature with signs of systemic inflammation. The latter is interpreted primarily as part of an infectious or paraneoplastic process, which causes a delay in the diagnosis.The aim of the study was to present the diversity of the clinical picture in vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), the speed and large volume of diagnostic measures with the effective cooperation of therapeutic and surgical specialists, radiologists on the way to verifying granulomatosis with polyangiitis in a young woman.Materials and methods. Patient K., 46 y. o., was hospitalized in the Otolaryngology Department of the N.I. Pirogov City Clinical Hospital No. 1 of the Moscow Health Department with complaints of hearing loss, pain and stuffiness in the left ear, unproductive cough, hoarseness and fever up to 38.5 °C. According to the radiography (RG) of the chest organs, right-sided pneumonia was detected. Conducted antibiotic therapy without effect. As part of the differential diagnostic search, the following nosologies were excluded: infective endocarditis, sepsis, tuberculosis, primary multiple or central lung cancer complicated by paracancer pneumonia, metastatic lesion, infectious, brucellosis spondylodiscitis.Results. In the blood test, attention was drawn to a decrease in the level of hemoglobin to 111 g / l, an increase in the rate of erythrocyte sedimentation to 45 mm / h and the level of C-reactive protein to 142 mg / l, microhematuria according to the general urine analysis. Instrumental research methods – RG of 16.02.22, MSCT of the chest organs on 17.02.22, 27.02.22, 10.03.22 showed progressive bilateral focal pneumonia with a focus of consolidation in the middle lobe, EchoCG, ultrasound of the abdominal cavity and small pelvis, RG of the temporal bone, bronchoscopy with bronchoalveolar lavage and microscopic analysis, for atypia and bacteriological culture. A gynecological examination and a smear from the cervical canal for microscopic analysis were performed, atypical cells, consulted by a phthisiatrician (no data for tuberculosis), consulted three times by a thoracic surgeon (exclusion of volumetric formation of the middle lobe of the right lung). Given the history and clinical presentation (female gender, young age, bilateral otitis media, hoarseness, and destructive nature of pneumonia), granulomatosis with polyangiitis was suspected, and tests for ANCA were prescribed. A transthoracic biopsy of the right lung was performed. A rheumatologist prescribed induction pulse therapy with corticosteroids, and after serological and histological confirmation (antibodies to Proteinase-3 Anti-PR3 > 200 IU / ml, productive pneumonitis, granulomas without signs of tuberculosis), immunosuppressive therapy with cyclophosphamide. Against the background of pathogenetic treatment, a pronounced clinical and laboratory effect was noted.Conclusion. In this clinical situation, the simultaneous involvement of specialists of various profiles, the performance of a large number of laboratory and instrumental studies in dynamics, the absence of delay in histological verification made it possible to quickly exclude common diseases in the population and suspect systemic vasculitis, establishing a correct diagnosis within 5 weeks of the hospitalization period.\",\"PeriodicalId\":82998,\"journal\":{\"name\":\"The Clinician\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-02-09\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The Clinician\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.17650/1818-8338-2022-16-4-k673\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Clinician","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.17650/1818-8338-2022-16-4-k673","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
介绍。全身性血管炎的一个显著特征是其表现在一个或另一个器官系统病变的幌子下,这通常是多器官的性质,具有全身性炎症的迹象。后者主要被解释为感染或副肿瘤过程的一部分,导致诊断延误。该研究的目的是展示与抗中性粒细胞细胞质抗体(ANCA)相关的血管炎的临床表现的多样性,在治疗和外科专家的有效合作下,快速和大量的诊断措施,放射科医生在验证年轻女性肉芽肿病合并多血管炎的过程中。材料和方法。患者K., 46岁,住在莫斯科卫生部N.I. Pirogov市第一临床医院耳鼻喉科,主诉听力丧失,左耳疼痛和充血,咳嗽无效,声音嘶哑,发烧高达38.5°C。根据胸部器官x线片(RG),发现右侧肺炎。抗生素治疗无效。作为鉴别诊断搜索的一部分,排除了以下疾病:感染性心内膜炎、败血症、结核病、原发性多发性或中枢性肺癌合并癌旁肺炎、转移性病变、感染性布鲁氏菌病脊柱炎。在血液检查中,注意到血红蛋白水平下降到111 g / l,红细胞沉降率增加到45 mm / h, c反应蛋白水平下降到142 mg / l,根据一般尿液分析出现微量血尿。仪器研究方法:16.02.22 RG, 17.02.22、27.02.22、10.03.22胸部脏器MSCT示进行性双侧局灶性肺炎,中叶病灶实变,超声心动图,腹腔及小骨盆超声,颞骨RG,支气管镜伴支气管肺泡灌洗及镜检,异型及细菌学培养。进行了妇科检查和宫颈管涂片进行显微镜分析,非典型细胞,咨询了一位肺结核医生(没有结核病的资料),咨询了三次胸外科医生(排除右肺中叶的体积形成)。考虑到病史和临床表现(女性、年轻、双侧中耳炎、声音嘶哑和肺炎的破坏性),怀疑肉芽肿病合并多血管炎,并开具了ANCA检查。行右肺经胸活检。一位风湿病医生开了糖皮质激素诱导脉冲治疗,在血清学和组织学证实后(蛋白酶-3抗pr3抗体> 200 IU / ml,生产性肺炎,无结核迹象的肉芽肿),环磷酰胺免疫抑制治疗。在病理治疗的背景下,注意到明显的临床和实验室效果。在这种临床情况下,各种专家同时参与,进行了大量的实验室和仪器动力学研究,没有延迟组织学验证,可以快速排除人群中的常见疾病和疑似全身性血管炎,在住院期的5周内建立正确的诊断。
Case of granulomatosis with polyangiitis: optimal possibilities for rapid diagnosis in a multidisciplinary hospital
Introduction. One of the distinguishing features of systemic vasculitis is their manifestation under the guise of a lesion of one or another organ system, which is often multi-organ in nature with signs of systemic inflammation. The latter is interpreted primarily as part of an infectious or paraneoplastic process, which causes a delay in the diagnosis.The aim of the study was to present the diversity of the clinical picture in vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), the speed and large volume of diagnostic measures with the effective cooperation of therapeutic and surgical specialists, radiologists on the way to verifying granulomatosis with polyangiitis in a young woman.Materials and methods. Patient K., 46 y. o., was hospitalized in the Otolaryngology Department of the N.I. Pirogov City Clinical Hospital No. 1 of the Moscow Health Department with complaints of hearing loss, pain and stuffiness in the left ear, unproductive cough, hoarseness and fever up to 38.5 °C. According to the radiography (RG) of the chest organs, right-sided pneumonia was detected. Conducted antibiotic therapy without effect. As part of the differential diagnostic search, the following nosologies were excluded: infective endocarditis, sepsis, tuberculosis, primary multiple or central lung cancer complicated by paracancer pneumonia, metastatic lesion, infectious, brucellosis spondylodiscitis.Results. In the blood test, attention was drawn to a decrease in the level of hemoglobin to 111 g / l, an increase in the rate of erythrocyte sedimentation to 45 mm / h and the level of C-reactive protein to 142 mg / l, microhematuria according to the general urine analysis. Instrumental research methods – RG of 16.02.22, MSCT of the chest organs on 17.02.22, 27.02.22, 10.03.22 showed progressive bilateral focal pneumonia with a focus of consolidation in the middle lobe, EchoCG, ultrasound of the abdominal cavity and small pelvis, RG of the temporal bone, bronchoscopy with bronchoalveolar lavage and microscopic analysis, for atypia and bacteriological culture. A gynecological examination and a smear from the cervical canal for microscopic analysis were performed, atypical cells, consulted by a phthisiatrician (no data for tuberculosis), consulted three times by a thoracic surgeon (exclusion of volumetric formation of the middle lobe of the right lung). Given the history and clinical presentation (female gender, young age, bilateral otitis media, hoarseness, and destructive nature of pneumonia), granulomatosis with polyangiitis was suspected, and tests for ANCA were prescribed. A transthoracic biopsy of the right lung was performed. A rheumatologist prescribed induction pulse therapy with corticosteroids, and after serological and histological confirmation (antibodies to Proteinase-3 Anti-PR3 > 200 IU / ml, productive pneumonitis, granulomas without signs of tuberculosis), immunosuppressive therapy with cyclophosphamide. Against the background of pathogenetic treatment, a pronounced clinical and laboratory effect was noted.Conclusion. In this clinical situation, the simultaneous involvement of specialists of various profiles, the performance of a large number of laboratory and instrumental studies in dynamics, the absence of delay in histological verification made it possible to quickly exclude common diseases in the population and suspect systemic vasculitis, establishing a correct diagnosis within 5 weeks of the hospitalization period.