Granulomatous

肉芽肿
  • 文章类型: Case Reports
    特发性肉芽肿性乳腺炎并不常见,女性乳房的长期炎症状况。它的特征是疼痛的乳房肿块的发展,其大小逐渐增加。这种情况是良性的,原因不明。它主要影响有怀孕和母乳喂养史的育龄妇女。特发性肉芽肿性乳腺炎的主要特征是乳房中存在慢性炎症。
    方法:我们报告了一名36岁女性,有妊娠和哺乳史,右侧乳房疼痛和肿胀。体格检查显示肿块,乳头发红和缩回。成像显示局部密度和低回声区域与收集,提示肉芽肿性乳腺炎.活检证实了诊断。在局部麻醉下通过小切口引流脓肿。用皮质类固醇治疗导致显着改善,一个月后完成决议。
    必须对潜在原因进行全面评估,以确认特发性肉芽肿性乳腺炎的诊断。组织学上,它的特点是主要存在中性粒细胞和不存在干酪样坏死。治疗仍然存在争议,最近的文献支持保守治疗与类固醇和免疫抑制治疗的疗效,对复杂和难治性病例进行手术切除。
    结论:特发性肉芽肿性乳腺炎是一种罕见的乳腺疾病,其确切原因和推荐的治疗方法尚不明确。重要的是要考虑到处于生育期的女性的这种情况。
    UNASSIGNED: Idiopathic granulomatous mastitis is an uncommon, long-lasting inflammatory condition of the female breast. It is characterized by the development of a painful breast mass that gradually increases in size. This condition is benign and its cause is unknown. It primarily affects women of childbearing age who have a history of pregnancy and breastfeeding. The main feature of idiopathic granulomatous mastitis is the presence of chronic inflammation in the breast.
    METHODS: We report a 36-year-old woman with a history of pregnancy and breastfeeding presented with pain and swelling of the right breast. Physical examination revealed a mass with redness and retraction of the nipple. Imaging revealed localized density and hypoechoic areas with collection, suggesting granulomatous mastitis. A biopsy confirmed the diagnosis. The abscess was drained through a small incision performed under local anesthesia. Treatment with corticosteroids resulted in significant improvement, with complete resolution after one month.
    UNASSIGNED: A comprehensive evaluation of potential causes is necessary to confirm the diagnosis of idiopathic granulomatous mastitis. Histologically, it is distinguished by the predominant presence of neutrophils and the absence of caseous necrosis. Treatment remains controversial, with recent literature supporting the efficacy of conservative management with steroid and immunosuppressive therapy, leaving surgical excision for complicated and refractory cases.
    CONCLUSIONS: Idiopathic granulomatous mastitis is an uncommon breast condition where the exact causes and recommended treatment approaches are not well-defined. It is important to consider this condition in women who are in their reproductive years.
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  • 文章类型: Journal Article
    目的:描述33只接受眼球摘除和眼部组织病理学检查的无菌肉芽肿性全葡萄膜炎综合征的临床和组织病理学特征。
    方法:回顾33例患者的病历和眼部组织病理学报告。纳入标准是摘除术结合特征性临床和组织病理学特征。
    结果:代表了13个品种(包括杂交品种)。葡萄膜炎是急性和暴发性的,继发性青光眼是常见的(n=27)。从初次就诊到摘除的间隔为99天(中位数为33天,5-605天)。眼球摘除的平均年龄为6.7岁。眼部体征最初为单侧(n=18)或双侧(n=15)。在最初接受单侧摘除的18/25例病例中,该疾病变为双侧疾病,导致9/18的摘除或安乐死(平均间隔168天)。59只眼中的7只眼在局部抗炎和全身免疫抑制治疗后具有良好的结果。从就诊到随访,没有一条狗有旅行史或相关的全身体征(平均619天,范围16-3012天)。组织病理学显示组织细胞和淋巴浆细胞性葡萄膜炎伴色素分散,在光学显微镜上没有发现感染因子。
    结论:据作者所知,这是英国首次报道犬的无菌肉芽肿性葡萄膜炎综合征.临床症状很严重,随着快速发展,并可能导致受影响的狗的双侧摘除或安乐死。似乎没有年龄或品种倾向,然而,在这方面需要进一步的研究。早期和积极的干预,局部和全身免疫抑制治疗,建议减少失明的风险,摘除,和安乐死。
    OBJECTIVE: To describe the clinical and histopathological features of a sterile granulomatous panuveitis syndrome in 33 dogs that underwent enucleation and ocular histopathology.
    METHODS: Retrospective review of the medical records and ocular histopathology reports of 33 cases. Inclusion criteria were enucleation in conjunction with characteristic clinical and histopathological features.
    RESULTS: Thirteen breeds were represented (including crossbreeds). Panuveitis was acute and fulminating, and secondary glaucoma was common (n = 27). Interval from initial presentation to enucleation was 99 days (median 33 days, range 5-605 days). The mean age at enucleation was 6.7 years. Ocular signs were initially unilateral (n = 18) or bilateral (n = 15). The disease became bilateral in 18/25 cases that initially underwent unilateral enucleation, resulting in enucleation or euthanasia in 9/18 (mean interval of 168 days). Seven out of 59 eyes had a good outcome following topical anti-inflammatory and systemic immunosuppressive therapy. None of the dogs had travel history nor relevant systemic signs from presentation to follow-up (mean 619 days, range 16-3012 days). Histopathology revealed histiocytic and lymphoplasmacytic panuveitis with pigment dispersion, and no infectious agents were identified on light microscopy.
    CONCLUSIONS: To the authors\' knowledge, this is the first report of a sterile granulomatous panuveitis syndrome in dogs in the UK. The clinical signs are severe, with rapid progression, and can result in bilateral enucleation or euthanasia in affected dogs. There does not appear to be an age or breed predisposition, however further research is necessary in this regard. Early and aggressive intervention, with both topical and systemic immunosuppressive therapy, is recommended to reduce the risk of blindness, enucleation, and euthanasia.
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  • 文章类型: Journal Article
    结节病是一种无菌的非坏死性肉芽肿性疾病,没有已知的原因,可能涉及多个器官,对肺和胸淋巴结有好感。在世界范围内,估计影响2-160/100,000人,并且5年的死亡率约为7%。对于结节病患者,在60%的病例中,死亡原因是结节病,其中高达80%是由于晚期心肺衰竭(肺动脉高压和呼吸道微生物感染),在所有种族中,除了日本,超过70%的结节病死亡是由于心脏结节病。肺结节病的进展阶段与临床结果相关。在大约30%-80%的病例中,I期和II期有影像学缓解。第三阶段只有10%-40%的机会解决,而第四阶段的决议没有变化。高达40%的肺结节病患者进展到IV期疾病与肺实质纤维增生,支气管扩张伴肺门回缩和纤维囊性疾病。这些患者发生毛细血管前肺动脉高压的风险最高,这可能发生在这些患者的70%。毛细血管前肺动脉高压结节病患者可以对靶向肺动脉高压药物产生反应。IV期纤维细胞结节病伴有明显的肺生理损害,>20%的HRCT纤维化或毛细血管前肺动脉高压具有最高的死亡风险,在5年内可能>40%。对于有症状(咳嗽和呼吸困难)并伴有实质浸润和肺功能测试(PFT)异常的患者,一线治疗是口服糖皮质激素,例如泼尼松,典型的起始剂量为每天20-40mg,持续2周至2个月。如果出现症状,泼尼松可以在6-18个月内逐渐减少,肺活量测定,PFTs,和射线照片改善。可能需要长期使用泼尼松来稳定疾病。需要延长泼尼松≥10mg/天的患者或因糖皮质激素而产生不良反应的患者可以进行二线和三线治疗。二线和三线治疗包括免疫抑制剂(例如,甲氨蝶呤和硫唑嘌呤)和抗肿瘤坏死因子(TNF)药物;分别。正在探索晚期纤维囊性肺病的有效治疗方法。尽管有不同的治疗方法,根据结节病的分期,复发率从13%到75%不等,涉及的器官数量,社会经济地位,和地理。结论:结节病在5年随访期间的死亡率约为7%。不幸的是,10%-40%的结节病患者发展为进行性肺部疾病,结节病导致的死亡>60%是由于晚期心肺疾病。口服糖皮质激素是一线治疗,而甲氨蝶呤和硫唑嘌呤被认为是第二和抗TNF药物是三线治疗,单独使用或作为糖皮质激素保护剂用于有症状的肺外或肺结节病,胸部X光片浸润和PFT异常。根据所研究的人群,复发率从13%到75%不等。
    Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.
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  • 文章类型: Journal Article
    脂类坏死病(NL)是一种罕见的慢性肉芽肿性疾病,毛细血管扩张,棕红色斑块,萎缩性黄色中心容易溃疡,主要发生在胫骨上。在儿童中,NL非常罕见,但是对治疗的抵抗力,麻烦的化妆品外观,痛苦的溃疡,在长期持续的病变中,鳞状细胞癌的可能发展是治疗期间的挑战。我们的综述包括从1990年发表在PubMed上的29例年龄<18岁的患者的NL报告,EMBASE,Medline患者平均年龄为14.3岁,女性占2:1,糖尿病患病率高(80%)。数据显示,每天两次的强效局部类固醇是一线治疗。对于难治性病例,治疗可以改用他克莫司。溃疡受益于相位适应的伤口护理和抗炎医用敷料如医用蜂蜜。在难以治疗的溃疡病变中,可以考虑在局部或全身治疗中添加高压氧。难治性病例可以改用局部光化学疗法或TNF-α抑制剂全身治疗,全身性类固醇(优选非糖尿病患者),己酮可可碱,或者羟氯喹.儿童时期的脂类坏死病难以治疗,治疗失败率为40%。因此,建议通过患者登记处进行进一步研究.
    Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that manifests as sharply demarcated, telangiectatic, brownish-red plaques with atrophic yellowish centers prone to ulceration and occurs predominantly on the shins. In children, NL is extremely rare, but resistance to therapy, troublesome cosmetic appearance, painful ulcerations, and possible development of squamous cell carcinoma in long-persisting lesions are challenges during treatment. Our review includes 29 reports of NL in patients aged <18 years published from 1990 on PubMed, EMBASE, and Medline. The mean age of patients was 14.3 years, with a female predominance of 2 : 1 and a high prevalence of diabetes mellitus (80%). Data showed that potent topical steroids up to twice daily is the first-line treatment. For refractory cases, therapy can be switched to tacrolimus. Ulcerations benefit from phase-adapted wound care and anti-inflammatory medical dressings such as medical honey. Adding hyperbaric oxygenation to local or systemic therapy in difficult-to-treat ulcerated lesions can be considered. Refractory cases may be switched to topical photochemotherapy or systemic treatment with TNF-α inhibitors, systemic steroids (preferably in non-diabetic patients), pentoxifylline, or hydroxychloroquine. Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.
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  • 文章类型: Journal Article
    结节病是一种多器官的肉芽肿性疾病,肺和淋巴系统是身体最常受影响的部位。它于1877年首次报道,自那时以来一直困扰着临床医生和科学家。因为结节病是一种排除性诊断,它要求医生排除所有可能的诊断。大多数患者保持无症状,这使得疾病长时间不被注意。多年后,这种疾病可以在目睹患者有症状或患有器官衰竭后被诊断出来。它可能会影响任何性别的中年人,通常其临床特征与结核病相关。在免疫学和组织病理学检查中,它简单地揭示了非干酪性肉芽肿。糖皮质激素现在仍然是标准药物。必须做进一步的研究才能知道确切的发病机制,结节病的早期发现和治疗方案的改进。当前的综述文章简要介绍了病因,临床特征,升级的诊断方法,如生物标志物检测和有组织的治疗计划,以治疗结节病。
    Sarcoidosis is a granulomatous disorder of multiple organs, with lungs and lymphatic systems being the most frequently affected sites of the body. It was first reported in 1877 and has continued to engross both clinicians and scientists since that time. Because sarcoidosis being a diagnosis of exclusion, it demands the physician to rule out all the possible diagnosis. Most of the patients remain asymptomatic and this makes the disease remain unnoticed for a prolonged period. Later after years, the disease could be diagnosed after witnessing the patient being symptomatic or suffering from organ failures. It could affect middle aged people of any sexes, often its clinical features correlate with tuberculosis. On immunological and histopathological examination, it reveals noncaseating granuloma in simple terms. Glucocorticoids remain the standard drug now and then. Further research has to be done to know the exact pathogenesis, early detection and betterment in treatment plan of sarcoidosis. The current review article gives a brief knowledge about etiopathogenesis, Clinical features, upgraded diagnostic methods such as biomarkers detection and the organized treatment plan to treat sarcoidosis.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    BACKGROUND: Sarcoidosis and spondyloarthritis (SpA) have been regularly associated. Bone iliac granulomas have also been described. We propose herein a systematic review of rheumatologic axial manifestations of sarcoidosis.
    METHODS: PubMed and the Cochrane Library were used to conduct this systematic literature review. Case reports and cross-sectional studies were reviewed according to Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines.
    RESULTS: A total of 41 articles were eligible. Three cross-sectional studies on the association between SpA and sarcoidosis showed a prevalence of sacroiliitis and SpA ranging from 12.9 to 44.8% and 12.9 to 48.3% in inflammatory back pain (IBP) subgroups, respectively. However, the IBP definitions and sacroiliac joint (SIJ) imaging modalities (X-rays or magnetic resonance imaging) were heterogeneous, and X-ray was mainly used for sacroiliitis diagnosis (in 78% of cases). Thirty-one case-report articles of the sarcoidosis-sacroiliitis association were identified, representing 35 patients. ASAS criteria for SpA were met in half of cases (16/32) and 46% (12/26) had HLA B27 positivity. Sarcoidosis occurred after sacroiliac symptoms in 47% of cases. In the seven case-report articles with granulomatous sacroiliac bone involvement, unilateral involvement seemed higher than in the sarcoidosis-sacroiliitis group.
    CONCLUSIONS: Literature analysis found a good evidence of the association between SpA and sarcoidosis, and special attention should be given to patients reporting IBP. Unilateral sacroiliitis may raise suspicion of granulomatous bone involvement, distinct from sacroiliitis. Imaging modalities used to study the SIJ in patients with sarcoidosis have been heterogeneous and further investigation is needed.
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  • 文章类型: Case Reports
    Chronic granulomatous invasive fungal sinusitis (CGIFS) is a peculiar disease of the paranasal sinuses due to its rarity, patient subset, and disease course. We describe 7 cases of histopathologically confirmed CGIFS with different treatment plans and varying outcomes. Of particular note was that one of these patients developed allergic fungal rhinosinusitis after complete resolution of his primary invasive disease, a finding that has never been reported in the literature. Another patient had an atypical fungal species (Aspergillus nidulans) on fungal stain and culture, while one immunodeficient patient had a large intracerebral disease component and died after 2 months of treatment. We also present a review of the pertinent literature investigating this rare disease.
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  • 文章类型: Case Reports
    Wegener\'s granulomatosis, first characterized as a clinical syndrome in 1936, is a rare form of vasculitis of the small- and medium-sized blood vessels affecting mainly the upper and lower respiratory tracts as well as the kidneys. This review article describes the case of a 45-year-old man who developed aortic regurgitation and third degree heart block secondary to Wegener\'s granulomatosis. He subsequently presented to our institution 3 years later with pulmonary hemorrhage. The ensuing literature review reveals that historically clinical cardiac involvement in Wegener\'s has been rare, particularly with valvular lesions. However, recent literature has shown an increase in reported cases of cardiac valvular lesions in Wegener\'s. The presented case report along with recently reported case reports highlight the morbidity and mortality associated with such cardiac lesions. We believe routine cardiovascular supervision including echocardiography and electrocardiograms are important for the screening and monitoring of patients with Wegener\'s granulomatosis.
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  • 文章类型: Journal Article
    Palisaded granulomatous dermatitis is an uncommon pathologic condition potentially associated with several disorders. These include drugs, inflammatory bowel disease, multiple myelomas, rheumatoid arthritis, and systemic lupus erythematosus. An illustrative case of a man with palisaded granulomatous dermatitis who subsequently developed ulcerative colitis is described, and the characteristics of other individuals with ulcerative colitis-associated palisaded granulomatous dermatitis are reviewed. PubMed was used to search the following terms: palisaded, interstitial, granulomatous, dermatitis, ulcerative colitis, and neutrophilic. Papers were obtained and references were reviewed. Ulcerative colitis-associated palisaded granulomatous dermatitis is uncommon. Palisaded granulomatous dermatitis-associated ulcerative colitis has been reported in four individuals. The palisaded granulomatous dermatitis appeared from six years prior to diagnosis to 19 years following diagnosis of the patient\'s gastrointestinal disease. In addition to individual and grouped papular lesions on the elbows, the morphology of palisaded granulomatous dermatitis can also present as indurated linear plaques overlying the metacarpophalangeal (MCP) joints and proximal fingers.
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