Chondritis

  • 文章类型: Case Reports
    复发性多软骨炎(RPC)是一种罕见的自身免疫性疾病,通常模仿复发性外耳炎。这种多系统疾病主要影响体内的软骨结构,耳廓是最常见的影响。RPC与炎症标志物和抗核抗体(ANA)升高有关,会导致软骨破坏.我们的病例是一名74岁的白人男性,有外周血管疾病(PVD)的病史,他在临床上反复发作,尽管使用了多种抗生素和非甾体抗炎药(NSAIDs),但右上耳疼痛肿胀14天。他在同一只耳朵患有慢性感觉神经性听力损失。在过去的七个月中,他多次出现相同的症状,并被诊断出患有外耳炎。他否认关节炎,疲劳,皮疹,磨损,过敏,创伤,或发烧。他开了抗菌药物,交替NSAIDs,和甲基强的松龙暂时缓解。他只服用他汀类药物,家族史平淡无奇。他发热,生命体征正常。在体检时,他没有急性痛苦,声音正常,但有弥漫性红斑,tender,肿胀的右耳耳廓和外管保留肺叶。其余的体检并不显著。实验室结果显示,C反应蛋白(CRP)升高100mg/L(正常范围:<3mg/L),红细胞沉降率(ESR)200mm/小时(正常范围:<20mm/小时)。ANA滴度为1:160,具有均匀的模式,但其他自身抗体呈阴性。在全血细胞计数(CBC)或综合代谢面板(CMP)上没有注意到危险信号,他的快速血浆反应素(RPR)试验为阴性。在这个病人身上,泼尼松每天60毫克开始作为单一疗法,和风湿病也被咨询。尽管进行了抗生素治疗,但由于反复和持续的上耳感染,患者仍寻求咨询,最终被诊断出患有罕见的疾病,称为复发性多软骨炎。经过这种治疗,耳廓软骨炎迅速好转。然后将类固醇剂量缓慢减少并维持在每天10mg以防止突然发作。随后,开始使用皮质类固醇后,炎症标志物下降到正常水平。
    Relapsing polychondritis (RPC) is a rare autoimmune condition that often mimics recurrent external otitis. This multisystemic disease primarily affects cartilaginous structures in the body, with the ear pinna being the most commonly impacted. RPC is associated with elevated inflammatory markers and antinuclear antibodies (ANA), and it can lead to chondral destruction. Our case is a 74-year-old Caucasian male with a history of peripheral vascular disease (PVD) who presented to the clinic with recurrent, painful swelling of the right upper ear for 14 days despite multiple antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs). He had chronic sensorineural hearing loss in the same ear. He was seen multiple times with identical symptoms in the last seven months and was diagnosed with otitis externa. He denied arthritis, fatigue, rash, abrasion, allergies, trauma, or fever. He was prescribed antimicrobials, alternating NSAIDs, and methylprednisolone with temporary relief. He is only on statins and has an unremarkable family history. He was afebrile with normal vital signs. On physical examination, he was not in acute distress and had a normal voice but had a diffusely erythematous, tender, swollen right ear pinna and external canal sparing the lobe. The rest of the physical examination was unremarkable. Laboratory results showed elevated C-reactive protein (CRP) of 100 mg/L (normal range: <3 mg/L) and erythrocyte sedimentation rate (ESR) of 200 mm/hour (normal range: <20 mm/hour). ANA titer is 1:160 with a homogenous pattern, but other autoantibodies were negative. No red flags were noted on the complete blood count (CBC) or comprehensive metabolic panel (CMP), and his rapid plasma reagin (RPR) test was negative. In this patient, prednisone 60 mg daily was initiated as monotherapy, and rheumatology was also consulted. The patient sought consultation due to recurrent and persistent upper ear infections despite antibiotic treatment and was ultimately diagnosed with a rare medical condition called relapsing polychondritis. Following this treatment, the auricular chondritis improved promptly. The steroid dosage was then slowly tapered and maintained at 10 mg daily to prevent flare-ups. Subsequently, after the initiation of corticosteroids, inflammatory markers trended down to normal levels.
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  • 文章类型: Journal Article
    VEXAS综合征是UBA1基因突变继发的罕见实体,位于X染色体上。这种突变产生,因此,造血干细胞上的特征性空泡。它的特点是多种自身炎症和血液学表现,有反应并最终依赖皮质类固醇治疗。在本出版物中,我们介绍了在我们医院诊断的2例病例系列,并对迄今为止已发表的证据进行了简短的文献回顾。
    VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far.
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  • 文章类型: English Abstract
    复发性多软骨炎(RP)是一种罕见的多系统疾病,主要涉及细胞外基质。典型的表现是耳朵软骨炎,鼻子和气管以及小关节和大关节的不对称寡关节炎或多关节炎。还描述了各种其他涉及。RP的治疗取决于多种因素,例如,器官表现。糖皮质激素,实施免疫抑制剂和靶向治疗。在血清阴性的类风湿性关节炎或血管炎具有非典型病程的情况下,应考虑RP的症状。
    死亡复发多软骨炎(RP)模型。TypischeManifestationensicheineChondritisderOhren,NaseundderTrachea,KleinerundauchgröererGelenke.凡士林和贝特利贡根。DieTherapiederRPistindividualellabhängigvonverschiedenenFaktorenwie.B.Organizationen.EswerdenGlukokortikoide,随后,免疫抑制剂sowiegezielteTherapieneingesetzt。贝氏血清学风湿性关节炎非典型verlaufendenVaskulitidensollteandasKrankheitsbildderRPgedachtwerden。
    Relapsing polychondritis (RP) is a rare multisystemic disease predominantly involving the extracellular matrix. Typical manifestations are chondritis of the ears, nose and trachea as well as an asymmetrical oligoarthritis or polyarthritis of small and also larger joints. Various other involvements have also been described. The treatment of RP is individually dependent on a variety of factors, e.g., organ manifestations. Glucocorticoids, immunosuppressants and targeted treatment are implemented. In the case of seronegative rheumatoid arthritis or vasculitis with an atypical course the symptoms of RP should be taken into consideration.
    UNASSIGNED: Die Relapsing Polychondritis (RP) ist eine seltene Multisystemerkrankung mit vorwiegendem Befall der extrazellulären Matrix. Typische Manifestationen sich eine Chondritis der Ohren, Nase und der Trachea, außerdem eine asymmetrische Oligo- oder Polyarthritis kleiner und auch größerer Gelenke. Verschiedene andere Beteiligungen sind ebenso beschrieben. Die Therapie der RP ist individuell abhängig von verschiedenen Faktoren wie z. B. Organmanifestationen. Es werden Glukokortikoide, aber auch Immunsuppressiva sowie gezielte Therapien eingesetzt. Bei seronegativer rheumatoider Arthritis oder atypisch verlaufenden Vaskulitiden sollte an das Krankheitsbild der RP gedacht werden.
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  • 文章类型: Journal Article
    这项工作旨在表征猪耳和喉软骨炎爆发的临床病理表现。参观了养猪场,获得临床病史的地方,并进行了临床和尸检。在那些农场里,3%至4%的猪出现耳血肿,从托儿所开始,一直延伸到最后阶段。此外,一些肥育猪出现呼吸窘迫,最初表现为吸气性呼吸困难,与罕见的呼吸道喘鸣有关,并最终导致死亡。严重的,苗圃仔猪的耳朵增大了,在切割表面上,软骨碎片化,并伴有血凝块.在完成阶段,除了耳廓病变,会厌和关节软骨增厚和扭曲,部分堵塞了管腔。微观上,喉和耳软骨支离破碎,显示基质嗜碱性粒细胞的损失,周围有淋巴组织细胞炎症浸润,偶有多核巨细胞和纤维化。病变仅影响弹性软骨。肥育猪的疾病导致死亡率增加,并且是呼吸系统挑战的鉴别诊断。无法确定触发此情况的因素;但是,怀疑是营养协会。就作者所知,这是猪原发性耳和喉软骨炎的首次报道。
    This work aimed to characterize the clinic-pathological presentation of an outbreak of auricular and laryngeal chondritis in pigs. Visits were made to pig farms, where the clinical history was obtained, and clinical and postmortem examinations were performed. In those farms, 3% to 4% of pigs presented otohematomas, which started in the nursery and extended to the finishing phase. Moreover, some finishing pigs presented with respiratory distress, initially characterized as inspiratory dyspnea, associated by an uncommon respiratory stridor and culminating in death. Grossly, nursery piglets had enlarged ears, and on the cut surface, the cartilage was fragmented and associated with blood clots. In the finishing phase, in addition to auricular lesions, the epiglottis and arytenoid cartilages were thickened and distorted, which partially occluded the lumen. Microscopically, the laryngeal and auricular cartilages were fragmented, displayed a loss of matrix basophilia, and were surrounded by lymphohistiocytic inflammatory infiltrate, with occasional multinucleated giant cells and fibrosis. The lesions exclusively affected elastic cartilages. The disease in finishing pigs led to increased mortality and was a differential diagnosis to respiratory challenges. It was not possible to determine the factor that triggered this condition; however, a nutritional association is suspected. To the authors\' knowledge, this is the first report of primary auricular and laryngeal chondritis in pigs.
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  • 文章类型: Case Reports
    液泡,E1酶,X-linked,自身炎症,体细胞(VEXAS)综合征是一种新型的自身炎症实体,其诊断由造血祖细胞中UBA1X连锁基因的体细胞突变定义。临床表现是异质的,因为它们的范围从自身炎症症状到潜在的血液系统疾病如骨髓增生异常综合征的存在。对VEXAS治疗的反应非常差,迄今为止,所采用的治疗策略仅部分有效.然而,最近描述的接受Janus激酶抑制剂(JAK-I)治疗的VEXAS受试者队列证明,这些药物可有效治疗与该疾病相关的几种表现。在这里,我们进行了简短的文献综述,包括采用JAK-I作为治疗VEXAS患者的有希望的策略的队列和单个病例.随后,我们在VEXAS中描述了我们使用JAK-I的经验,说明第一种情况,根据我们的知识,一名65岁的男性成功使用选择性JAK-1抑制剂filgotinib治疗。
    Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a novel described autoinflammatory entity for which the diagnosis is defined by somatic mutations of the UBA1 X-linked gene in hematopoietic progenitor cells. The clinical manifestations are heterogeneous since they range from autoinflammatory symptoms to the presence of underlying hematologic disorders such as myelodysplastic syndromes. Response to treatment in VEXAS is very poor and to date, the therapeutic strategies adopted are only partially effective. However, recently described cohorts of subjects with VEXAS treated with Janus kinase inhibitors (JAK-I) proved that these drugs can be effective in the treatment of several manifestations related to the disease. Herein, we carried out a brief literature review that includes cohorts and single cases in which JAK-I were adopted as a promising strategy to manage VEXAS patients. Subsequently, we described our experience with JAK-I in VEXAS, illustrating the first case, to our knowledge, of a 65-year-old man who was successfully treated with the selective JAK-1 inhibitor filgotinib.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    未经证实:化脓性软骨炎是耳朵烧伤的潜在毁灭性并发症。感染和炎症可以液化软骨,导致难以治疗的显著美学畸形。本文回顾了已发表的预防烧伤后软骨炎的措施。
    未经评估:对截至2020年9月的所有可用文献进行了全面搜索,根据PRISMA指南,用于评估烧伤后软骨炎预防措施的研究。随机对照试验(RCT),队列研究,病例对照研究,病例报告和系列有资格纳入.
    未经评估:共10项研究,包括一项RCT和九项回顾性观察分析,包括在内,合并1369名耳朵烧伤患者。最常见的干预措施是避免压力(70%),每日清洁(60%),局部使用醋酸马菲尼特(60%)和靶向清创术(30%)。包括避免压力的一揽子措施是最有效的,所有这些都达到了软骨炎的发病率<6%。
    UNASSIGNED:低水平但强有力的公开证据表明,重要的治疗原则包括通过减压预防,有针对性的清创,预防性局部抗生素,局部防腐和避免干燥。
    UNASSIGNED: Suppurative chondritis is a potentially devastating complication of burns to the ear. The infection and inflammation can liquify cartilage, leading to significant aesthetic deformities which are difficult to treat. This article reviews published measures for preventing post-burn chondritis.
    UNASSIGNED: A comprehensive search of all available literature up to September 2020 was performed, according to PRISMA guidelines, for studies assessing preventive measures for post-burn chondritis. Randomised controlled trials (RCT), cohort studies, case-control studies, case reports and series were eligible for inclusion.
    UNASSIGNED: A total of 10 studies, including one RCT and nine retrospective observational analyses, were included, incorporating 1369 patients with burns to the ear. The most common interventions were pressure avoidance (70%), daily cleansing (60%), topical mafenide acetate (60%) and targeted debridement (30%). Packages of measures which included pressure avoidance were the most effective, all of which achieved a chondritis incidence of <6%.
    UNASSIGNED: Low-level but strong published evidence suggests that important treatment principles include prevention by pressure relief, targeted debridement, prophylactic local antibiotics, local antisepsis and the avoidance of desiccation.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    Trapeziometacarpal joint ligament reconstruction is a surgical option in the early stages of thumb basal joint arthritis when the joint is painful and unstable without chondropathy. Arthroscopy is invaluable here to ensuring the joint surfaces are intact, which is often underestimated by radiography. The Eaton-Littler procedure using a distally pedicled flexor carpi radialis slip has been studied the most in this context. This reconstruction provides pain relief while slowing the rapid development of osteoarthritis.
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