中文摘要:肉芽肿肉芽肿韦格纳·梅鲁帕坎·萨图·梅格纳·梅格纳·梅格纳·拉赫·特鲁塔玛·梅布鲁·达拉赫·凯西尔·丹·萨朗阿基巴·阿基巴·阿迪亚·雷克西·科普莱克·伊蒙。桑派沙因科肉芽肿病。诊断颗粒Wegenersalahsatunyaadalahterdeteksinyaantibodi胞浆抗中性粒细胞胞浆抗体(c-ANCA),akantetapipemeriksaanANCAnegatifatautidakditemukanpadakasWegener肉芽肿病是一种自身免疫性疾病,由于免疫复合物反应而影响中小型血管壁。同时,目前,该疾病的病因尚不明确。诊断之一是检测细胞质抗中性粒细胞胞浆抗体(c-ANCA),但是ANCA检查阴性是非常罕见的。因此,这是一名33岁男子的病例报告,他抱怨双腿有疮,很难治愈。患者还经历了关节痛,晚上发烧,减肥,脱发以及原因不明的经常性流鼻血。此外,体格检查发现马鞍鼻子和从左右腹股沟到腿后部的黑点。在该区域和足背也发现了多个不同大小的不规则溃疡。化验结果显示Hb为8.7克/分升,130mm/小时ESR。根据外周血涂片,该患者被怀疑患有低色素性小红细胞性贫血,导致慢性过程和出血。IF模式也有斑点,滴度为1:320,ANCA试验为阴性(-)。同时,尿液常规检查结果肉眼可见血+4,微可见白细胞增多2-10LPB和8-21LPB红细胞增多。左下肢多普勒超声显示左足软组织肿胀,皮肤病变区周围动脉炎。解剖病理学检查显示在毛囊和脚掌区域有非特异性慢性炎症。随后,患者由外科医生进行伤口清创术,充血红细胞(PRC)输血,甲泼尼龙毫克,硫唑嘌呤,还有头孢克肟.治疗后,不再感觉到流鼻血,关节疼痛减轻,发烧有所改善。美国inisangatjarangterjadi。阿蒂克尼·梅巴哈斯·门格奈·卡苏斯·拉基33塔洪·达唐·登安·克鲁汉·卢卡·帕达·通格·卡基·杨·苏利特·塞姆布赫·迪塞尔泰·尼耶里·帕达·森迪,DemamPadaMalamhari,巴丹半岛半岛,朗托克·丹·米米米桑·贝鲁朗·杨·蒂达克·迪克塔惠·彭耶巴尼亚。Padapemeriksaanfisikditemukan鞍形鼻子,terdapatbercakkehitamandarikeduapangkalpahakanandankirisampaikepunggungkeduakaki.帕达·雷迪奥·克里里斯·丹·多萨姆·皮迪迪帕特坎·阿达尼亚·乌尔库斯多,ukuranbervariasi,不规则。Hasilpemeriksaan实验室menunjukkanHb8,7gr/dl,LED130毫米/卡纸,哈普桑·达拉·泰皮:贫血hipokromik-mikrositiksusepeketcausaproseskronisbersamandenganprosesperdarahan。ANA-IF波拉斑点1:320,ANCAteshasilnegatif(-)。Hasilpemeriksaanurinrutinmakroskopisdarah+4,danmikroskopisditemukan白细胞尿症2-10LPB,eritrosituria8-21LPB。HasilUSG多普勒padaregioekstremitas下左侧menunjukkankesan:肿胀的jaringanlunak脚左侧dengan动脉炎周围pada区lesikutis。HasilpemeriksaanPatologiAnatomimenunjukkanperadangankronisnonspesifiketregiocrurisdanpedis.Tatalaksanayangdilakukan清创LukaOlehspesialisbedah,输血包红细胞(PRC),甲泼尼龙毫克,硫唑嘌呤,头孢克肟。Hasilnyamimisansudahtidakdirasakan,nyerisendiberkurangtetapimasihada,DanDemamMembaik.KataKunci:肉芽肿病,Vaskuitisautoimun,Wegener肉芽肿病是一种自身免疫性疾病,由于免疫复合物反应而影响中小型血管壁。同时,目前,该疾病的病因尚不明确。诊断之一是检测细胞质抗中性粒细胞胞浆抗体(c-ANCA),但是ANCA检查阴性是非常罕见的。因此,这是一名33岁男子的病例报告,他抱怨双腿有疮,很难治愈。患者还经历了关节痛,晚上发烧,减肥,脱发以及原因不明的经常性流鼻血。此外,体格检查发现马鞍鼻子和从左右腹股沟到腿后部的黑点。在该区域和足背也发现了多个不同大小的不规则溃疡。化验结果显示Hb为8.7克/分升,130mm/小时ESR。根据外周血涂片,该患者被怀疑患有低色素性小红细胞性贫血,导致慢性过程和出血。IF模式也有斑点,滴度为1:320,ANCA试验为阴性(-)。同时,尿液常规检查结果肉眼可见血+4,微可见白细胞增多2-10LPB和8-21LPB红细胞增多。左下肢多普勒超声显示左足软组织肿胀,皮肤病变区周围动脉炎。解剖病理学检查显示在毛囊和脚掌区域有非特异性慢性炎症。随后,患者由外科医生进行伤口清创术,充血红细胞(PRC)输血,甲泼尼龙毫克,硫唑嘌呤,还有头孢克肟.治疗后,不再感觉到流鼻血,关节疼痛减轻,发烧有所改善。关键词:韦格纳肉芽肿病,自身免疫性血管炎,阴性ANCA。
ABSTRAK Penyakit Granulomatosis Wegener merupakan suatu penyakit autoimun yang mengenai dinding pembuluh darah terutama pembuluh darah kecil dan sedang akibat adanya reaksi komplek imun. Sampai saat ini etiologi penyakit Granulomatosis Wegener belum diketahui secara pasti. Diagnosis Granulosis Wegener salah satunya adalah terdeteksinya antibodi cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), akan tetapi pemeriksaan ANCA negatif atau tidak ditemukan pada kasWegener\'s granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved.us ini sangat jarang terjadi. Artikel ini membahas mengenai kasus laki-laki 33 tahun datang dengan keluhan luka pada kedua tungkai kaki yang sulit sembuh disertai nyeri pada sendi, demam pada malam hari, penurunan berat badan, rambut rontok dan mimisan berulang yang tidak diketahui penyebabnya. Pada pemeriksaan fisik ditemukan saddle nose, terdapat bercak kehitaman dari kedua pangkal paha kanan dan kiri sampai ke punggung kedua kaki. Pada regio cruris dan dorsum pedis didapatkan adanya ulkus multiple, ukuran bervariasi, irregular. Hasil pemeriksaan laboratorium menunjukkan Hb 8,7 gr/dl, LED 130 mm/jam, Hapusan darah tepi: anemia hipokromik-mikrositik suspek et causa proses kronis bersamaan dengan proses perdarahan. ANA-IF pola speckled 1:320, ANCA tes hasil negatif (-). Hasil pemeriksaan urin rutin makroskopis darah +4, dan mikroskopis ditemukan leukosituria 2-10 LPB, eritrosituria 8-21 LPB. Hasil USG doppler pada regio ekstremitas inferior sinistra menunjukkan kesan: swelling jaringan lunak pedis sinistra dengan arteritis perifer pada area lesi kutis. Hasil pemeriksaan Patologi Anatomi menunjukkan peradangan kronis non spesifik et regio cruris dan pedis. Tatalaksana yang dilakukan debridement luka oleh spesialis bedah, transfusi pack red cell (PRC), metylprednisolon mg, azathioprine, cefixime. Hasilnya mimisan sudah tidak dirasakan, nyeri sendi berkurang tetapi masih ada, dan demam membaik. Kata Kunci: Granulomatosis wegener, Vaskulitis autoimun, ANCA negatif ABSTRACT Wegener\'s granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved. Keywords: Wegener\'s granulomatosis, Autoimmune vasculitis, negative ANCA.