granulomatous diseases

  • 文章类型: Case Reports
    鼻孢子虫病是亚洲流行的肉芽肿性疾病之一,非洲,和欧洲,印度南部和斯里兰卡的患病率最高。通常理解为影响上呼吸道系统。与上呼吸道相比,气管以外的肺很少发生。我们发现了一例糖尿病患者的播散性鼻孢子虫病的罕见病例,最初表现为咳嗽和发烧相关的呼吸急促。在这些症状出现前两个月,他有口腔溃疡和吞咽困难,随后,减肥。胸片及胸部CT提示军用结节伴多发化脓性颈部及纵隔淋巴结肿大,双侧肾上腺病变。他最初接受了结核病调查,转移性恶性肿瘤,或淋巴管癌在活检前发现鼻孔孢子虫病。因此,组织病理学或实验室证据通常对于支持影像学问题至关重要,因此可以给予适当的治疗。
    Rhinosporidiosis is one of the granulomatous diseases endemic in Asia, Africa, and Europe, with Southern India and Sri Lanka having the greatest prevalence rates. It is typically understood to affect the upper respiratory system. Involvement of the lungs beyond the trachea is infrequent as compared to the upper respiratory tract. We revealed an uncommon case of disseminated rhinosporidiosis in a diabetic patient, who initially presented with shortness of breath associated with cough and fever. Two months prior to these symptoms, he was having oral ulcer and dysphagia and, subsequently, loss of weight. Chest radiograph and CT thorax revealed military nodules with multiple suppurative neck and mediastinal lymphadenopathy and bilateral adrenal lesions. He was initially investigated for tuberculosis, metastatic malignancy, or lymphangitic carcinomatosis before a biopsy revealed Rhinosporidiosis. Hence, histopathological or laboratory evidence is frequently crucial to back up imaging concerns so the appropriate treatment can be given.
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  • 文章类型: Journal Article
    背景:结核(TB)和结节病是两种常见的肉芽肿性疾病,累及淋巴结。鉴别诊断并不总是容易的,因为结核病中的病原体显示并不总是可能的,并且两种疾病在临床上都具有相同的意义。放射学和组织学模式。我们研究的目的是确定与每种诊断相关的因素,并建立结核病的预测评分。方法:回顾性分析所有淋巴结结核和结节病的病例。人口统计,临床特征,实验室和成像数据,收集并比较微生物学和组织学结果。结果:在接受筛查的441例患者中,最终分析包括192例患者。多变量分析表明,体重减轻,坏死性肉芽肿,正常血清溶菌酶水平和高丙种球蛋白血症与TB显著相关。基于这些变量建立TB的风险评分,并且能够区分TB与结节病,AUC为0.85(95%CI:0.79-0.91)。使用尤登的J统计量,其最大判别值(-0.36)与80%的敏感性和75%的特异性相关.结论:我们制定了基于体重减轻的评分,坏死性肉芽肿,正常的血清溶菌酶水平和高丙种球蛋白血症,具有出色的区分结核病和结节病的能力。这一分数仍需在多中心前瞻性研究中得到验证。
    Background: Tuberculosis (TB) and sarcoidosis are two common granulomatous diseases involving lymph nodes. Differential diagnosis is not always easy because pathogen demonstration in tuberculosis is not always possible and both diseases share clinical, radiological and histological patterns. The aim of our study was to identify factors associated with each diagnosis and set up a predictive score for TB. Methods: All cases of lymph node tuberculosis and sarcoidosis were retrospectively reviewed. Demographics, clinical characteristics, laboratory and imaging data, and microbiological and histological results were collected and compared. Results: Among 441 patients screened, 192 patients were included in the final analysis. The multivariate analysis showed that weight loss, necrotic granuloma, normal serum lysozyme level and hypergammaglobulinemia were significantly associated with TB. A risk score of TB was built based on these variables and was able to discriminate TB versus sarcoidosis with an AUC of 0.85 (95% CI: 0.79-0.91). Using the Youden\'s J statistic, its most discriminant value (-0.36) was associated with a sensitivity of 80% and a specificity of 75%. Conclusions: We developed a score based on weight loss, necrotic granuloma, normal serum lysozyme level and hypergammaglobulinemia with an excellent capacity to discriminate TB versus sarcoidosis. This score needs still to be validated in a multicentric prospective study.
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  • 文章类型: Journal Article
    肉芽肿病伴多血管炎是一种罕见的多器官受累疾病。它通常影响上呼吸道,下呼吸道和肾脏。90%的患者患有鼻窦炎等上呼吸道疾病,鼻息肉病,鼻中隔穿孔,浆液性中耳炎,声门下狭窄引起的听力受损和喘鸣。疾病以两种形式表现为局限性疾病,即没有肾脏受累和广泛性疾病,即有肾脏受累。需要将其与结核病等慢性肉芽肿疾病区分开来,并应防止治疗延迟。在这里,我们提出了一个仅限于鼻和鼻旁窦的疾病病例。
    Granulomatosis with polyangiitis is a rare disease with involvement of multiple organ system. It classically affects the upper respiratory tract, lower respiratory tract and kidneys. 90% of patients present with upper respiratory diseases like rhinosinusitis, nasal polyposis, nasal septal perforation, serous otitis media, impaired hearing and stridor due to subglottic stenosis. Disease manifests in two forms as limited disease i.e. without renal involvement and generalized disease i.e. with renal involvement. It needs to be differentiated from chronic granulomatous diseases like tuberculosis and delay in the treatment should be prevented. Here we present a case with disease limited to Nose and Paranasal sinuses.
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  • 文章类型: Case Reports
    结核病和结节病之间的区别有时会带来临床挑战。他们在同一患者中的顺序发生并不常见。我们介绍了一名42岁的女性,其确诊为结核性淋巴结炎,经过10个月和14个月的抗结核治疗后,分别出现了鼻结核和肺结节病。患者表现为双侧颈部淋巴结肿大。结核菌素皮试阴性。胸部X线检查正常。进行了切除活检,组织病理学检查确定了结核病诊断。开始使用抗结核药物治疗,颈淋巴结肿大表现为进行性消退。随后,近10个月后,患者出现新的颈部淋巴结肿大和鼻塞。活检证实鼻粘膜结核。氧氟沙星和乙胺丁醇增强了抗结核生物疗法。胸部CT扫描显示双肺有几个结节成分,双侧纵隔腺病增大。支气管肺泡灌洗显示淋巴细胞性肺泡炎,CD4/CD8比值为5,与肺结节病的诊断一致。皮质类固醇治疗,以口服泼尼松龙的形式被引入,结节病诊断确定后3个月;由于胸部CT发现肺纤维化。全身皮质治疗持续3年,直到所有的病灶清除.本病例强调了结核病和结节病之间的可能关联。
    The distinction between tuberculosis and sarcoidosis presents sometimes a clinical challenge. Their sequential occurrence in the same patient is uncommon. We present the case of a 42-year-old female with a proven diagnosis of tuberculous lymphadenitis who has developed successively nasal tuberculosis and pulmonary sarcoidosis respectively after 10 and 14 months of antituberculosis treatment. The patient presented with bilateral cervical lymphadenopathy. Tuberculin skin test was negative. Chest radiography was normal. An excision biopsy was taken and histopathological examination established tuberculosis diagnosis. Therapy with antituberculosis drugs was started, and cervical lymphadenopathy showed progressive resolution. Subsequently, nearly 10 months after, the patient developed new cervical lymphadenopathies and nasal obstruction. Tuberculosis of the nasal mucosa was confirmed by biopsy. Antituberclosis bitherapy was enhanced by ofloxacin and ethambutol. Thoracic CT scan showed several nodular elements in both lungs, with bilateral enlarged mediastinal adenopathy. Bronchoalveolar lavage showed a lymphocytic alveolitis with a CD4/CD8 ratio of 5, consistent with the diagnosis of pulmonary sarcoidosis. Corticosteroid treatment, in form of oral prednisolone was introduced, 3 months after sarcoidosis diagnosis have been setteled; because of pulmonary fibrosis noticed on thoracic CT. Systemic corticotherapy was continued for a further period of 3 years, until all the lesions cleared out. The present case emphasizes the possible association between tuberculosis and sarcoidosis.
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  • 文章类型: Case Reports
    一名患者被转诊至口腔科,嘴唇和脸颊红肿,和口腔内病变。活检和实验室检查提示诊断为结节病。在这项研究中,我们讨论了与结节病相关的口腔发现。
    A patient was referred to the oral medicine department with redness and swelling of the lips and cheek, and an intra-oral lesion. Biopsy and laboratory investigations suggested a diagnosis of sarcoidosis. In this study we discuss oral findings associated with sarcoidosis.
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  • 文章类型: Journal Article
    肉芽肿的病因包括感染,血管炎,化学品,恶性肿瘤,淋巴增生性疾病,和免疫学疾病。我们假设肉芽肿患者患有潜在的原发性免疫缺陷病(PIDD)。
    我们在294例经活检证实的肉芽肿患者(0-20岁)中,回顾性纳入了82例进行免疫学评估的患者。同时,我们对同一年龄组的1910名患者进行了随访.
    在82名患者中,男女比例为45/37。症状发作的中位年龄为5岁(28天-17.4岁),诊断时肉芽肿的年龄为8.6岁(36天-19.4岁).发病时常见症状为发热(23.2%),淋巴结肿大(19.6%),腹痛(12.2%),咳嗽(12.2%)。肉芽肿多发于淋巴结(26.8%),皮肤(19.5%),肺(13.4%),骨骼(11%)。分离出的常见感染因子是分枝杆菌。(23.2%)和EBV(4.9%)。我们记录了76.8%(63/82)的患者的PIDD。经免疫学评估的肉芽肿患者中有49.4%(40/81)患有低丙种球蛋白血症。小儿PIDD的肉芽肿率为3.3%(63/1910)。多发性肉芽肿患者(n=16)诊断为PIDD。肺部受累在PIDD中增加了三倍。大脑受累仅见于PIDD。在没有记录的PIDD的患者和未完成评估的患者中,纤维脂肪组织和肝脏受累更为频繁。PIDD伴肉芽肿的死亡率为15.9%,而没有PIDD的患者没有死亡。
    如本文所述,甚至在大学医院,免疫学评估比率约为三分之一。我们在肉芽肿患儿中显示出较高的PIDD频率,伴有肉芽肿的PIDD死亡率较高。因此,免疫学家精心进行的免疫学评估是准确诊断和决定个性化治疗方案的必要条件.
    Granuloma etiology includes infections, vasculitis, chemicals, malignancies, lymphoproliferative disorders, and immunological diseases. We hypothesized that patients with granuloma have an underlying primary immunodeficiency disease (PIDD).
    We retrospectively enrolled 82 patients with immunological evaluation among 294 biopsy-proven granuloma patients (0- to 20-year-old). At the same time frame, we followed up with 1910 patients in the same age group.
    Out of 82 patients, male/female ratio was 45/37. Median age at symptom onset was 5 years (28 days-17.4 years), age of granuloma at diagnosis was 8.6 years (36 days-19.4 years). Common symptoms at disease onset were fever (23.2%), lymphadenopathy (19.6%), abdominal pain (12.2%), and cough (12.2%). Granuloma was frequent in lymph nodes (26.8%), skin (19.5%), lung (13.4%), and bone (11%). Common infectious agents isolated were Mycobacterium spp. (23.2%) and EBV (4.9%). We document PIDD in 76.8% (63/82) of patients. 49.4% (40/81) of immunologically evaluated granuloma patients had hypogammaglobulinemia. Granuloma rate in pediatric PIDD was 3.3%(63/1910). Patients with multiple granulomas (n = 16) had a PIDD diagnosis. Lung involvement was three times more in PIDD. Brain involvement was only seen in PIDD. Fibroadipose tissue and liver involvement were more frequent in patients without documented PIDD and patients whose evaluation was not completed. The mortality rate in PIDD with granuloma was 15.9%, whereas there was no mortality in patients without PIDD.
    As documented here, even in a university hospital, the immunologic evaluation ratio is about one-third. We showed high PIDD frequency in children with granuloma, and higher mortality in PIDD with granuloma. Thus, an immunologic evaluation performed meticulously by immunologists is a must for accurate diagnosis and decision of individualized therapeutic options.
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  • 文章类型: Case Reports
    结节病是一种病因不明的多器官肉芽肿性疾病。结节病的神经系统受累并不常见,但颅内单神经病,特别是涉及VII和VIII脑神经,高度怀疑是神经结节病.我们报道了一名54岁的女性发烧的病例,盗汗,减肥,多关节痛,和双侧听力损失。在发射断层扫描(PET)阳性扫描中,存在纵隔和肺门淋巴结肿大。低剂量类固醇无效。然后,她出现了双侧前葡萄膜炎和右侧周围性面神经麻痹。头部磁共振成像(MRI)显示右侧耳蜗有炎症,膝状神经节,和双侧前庭耳蜗束。脑脊液分析与无菌性脑膜炎兼容。纵隔淋巴结切除活检证实存在非干酪性肉芽肿。诊断为患有严重神经系统受累的系统性结节病,并使用大剂量类固醇治疗可明显改善临床效果。结节病仍然是基于支持性临床的排除诊断,放射学,和组织学发现。这个案例凸显了诊断这种疾病的挑战。结节病的神经系统受累相对罕见,并且具有不可预测的临床病程和预后。
    Sarcoidosis is a multi-organ granulomatous disease of unknown etiology. Neurological involvement in sarcoidosis is uncommon but cranial mononeuropathies, especially involving the VII and VIII cranial nerves, are highly suggestive of neurosarcoidosis. We report the case of a 54-year-old woman who presented with fever, night sweats, weight loss, polyarthralgia, and bilateral hearing loss. Mediastinal and hilar lymphadenopathies with hypercaptation on positive emission tomography (PET) scans were present. Low-dose steroids were ineffective. She then developed bilateral anterior uveitis and right-sided peripheral facial palsy. Head magnetic resonance imaging (MRI) showed inflammatory involvement of the right cochlea, geniculate ganglion, and bilateral vestibulocochlear bundle. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Excisional biopsy of mediastinal lymph nodes confirmed the presence of noncaseating granulomas. The diagnosis of systemic sarcoidosis with serious neurological involvement was made and treatment with high-dose steroids led to significant clinical improvement. Sarcoidosis remains a diagnosis of exclusion based on supportive clinical, radiological, and histological findings. This case highlights the challenge it was to diagnose this disorder. Neurologic involvement in sarcoidosis is relatively uncommon and has an unpredictable clinical course and prognosis.
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  • 文章类型: Journal Article
    An association between lymphoma and sarcoidosis was first suggested in 1960. We report a case of sarcoidosis-lymphoma syndrome, which is a diagnostically challenging condition. We conclude that an associated lymphoma should be considered in all patients with suspected sarcoidosis, especially those who do not respond to treatment or who present with persistent haematological abnormalities. Splenomegaly should prompt splenectomy to rule out lymphoma if a less invasive approach has failed to confirm the diagnosis.
    UNASSIGNED: Clinical consideration should be given to an associated lymphoma in all patients with sarcoidosis, especially those who do not respond to treatment or who present with persistent haematological abnormalities.Splenomegaly should raise the possibility of splenectomy to rule out associated lymphoma.
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  • 文章类型: Journal Article
    UNASSIGNED: Clinically, cutaneous leishmaniasis (CL) can be confused with granulomatous diseases and skin cancers, and it may lead to erroneous diagnosis and treatment. Diagnosis based and histopathology can have some difficulties due to low number of parasites, especially in chronic CL cases. We aimed to emphasize the necessity of considering CL in the differential diagnosis for cases of granulomatous diseases and basal cell carcinoma, particularly in areas where CL is endemic.
    UNASSIGNED: One hundred and seven paraffin-embedded tissue biopsy specimens were selected from the archive, as of 2002, of Pathology Department, School of Medicine, University of Hatay Mustafa Kemal in Hatay, Turkey. After DNA isolation, performed with the samples were used for PCR analysis with specific 13A, 13B primers targeting kinetoplastid DNA (kDNA) found in all Leishmania species. Another PCR was performed with LITSR and L5.8S primers targeting ITS-1 internal-transcribed-spacer-1 (ITS-1) region to subtype positive samples. Then these samples were further analyzed for subtyping with PCR-RFLP using HaeIII enzyme (BsuRI).
    UNASSIGNED: Ten out of 107 tissue specimens were positive via kDNA-PCR. Lupus vulgaris, sarcoidosis, skin lymphoma and Leishmania cutis appeared in 9 out of 10 positive specimens. One of the cases presented with a mass on the cheek and was pre-diagnosed with hemangioma, but leishmaniasis did not appear. All of 10 specimens were diagnosed as granulomatous dermatitis. Two out of 10 samples, found positive with kDNA-PCR, were analyzed with ITS-1-PCR and identified as L. infantum/donovani after RFLP.
    UNASSIGNED: Molecular methods should be utilized in the differential diagnosis of CL to eliminate false diagnoses of granulomatous diseases and skin cancers.
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  • 文章类型: Journal Article
    Breast sarcoidosis is an extremely rare entity (about 1%). Conventional imaging significantly contributes to the detection of breast lesions, but it has been unable to establish a definite diagnosis. Histological examination should be mandatory, over imaging assessments, in order to confirm an early diagnosis and to avoid unjustified treatments. Malignancy should be excluded as a primary differential diagnosis. However, in the presence of granulomas, it is important to recognize other granulomatous disorders such as tuberculosis, Wegener\'s granulomatosis, or idiopathic granulomatous mastitis, since therapeutic strategies differ. This report clarifies the current clinical assessments and differential diagnosis of breast sarcoidosis.
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