Diffuse cutaneous systemic sclerosis

  • 文章类型: Case Reports
    许多肺部疾病,如吸入性肺炎和急性呼吸窘迫综合征(ARDS),可能是由于胃或口咽内容物进入下呼吸道而引起的。ARDS是一种弥漫性肺损伤,其特征是突然发作的广泛肺部炎症伴随着多器官系统的衰竭。系统性硬化症是一种罕见的结缔组织疾病,表现为皮肤增厚,其病因尚不清楚。在大多数系统性硬化症病例中,在食管远端三分之一处观察到食管管腔扩张。这种扩张主要归因于该区域平滑肌纤维的更丰富。这里,我们介绍1例70岁女性患者,临床诊断为弥漫性系统性硬化症,符合2013年欧洲抗风湿病联盟/美国风湿病学会分类标准.她有食道扩张症,食道内腔直径在上部测量,中间,和下胸段食管2.5厘米,2.5cm,和3.5厘米,分别。由于吸入性肺炎引起的ARDS,患者被送往重症监护病房(ICU)。我们的患者在入住ICU时并发ARDS继发于吸入性肺炎主要是由于食管扩张和反流。积极的抗反流药物治疗和床抬高可能有助于防止误吸引起的肺损伤。食管并发症在此类患者中很常见,并且可能对预后和生活质量产生重大影响。定期就医是必要的,以识别和管理任何潜在的问题。
    Numerous pulmonary conditions, such as aspiration pneumonia and acute respiratory distress syndrome (ARDS), may result from aspiration of gastric or oropharyngeal contents passing into the lower respiratory tract. ARDS is a type of diffuse lung injury that is distinguished by the abrupt onset of extensive pulmonary inflammation accompanied by the failure of multiple organ systems. Systemic sclerosis is an uncommon connective tissue disorder that presents with skin thickening, the etiology of which remains unknown. Esophageal luminal dilatation is observed in the distal third of the esophagus in most cases of systemic sclerosis. This dilatation is primarily attributed to the greater abundance of smooth muscle fibers in this area. Here, we present the case of a 70-year-old female patient who was diagnosed clinically with diffuse systemic sclerosis and fulfilled the 2013 European League Against Rheumatism/American College of Rheumatology classification criteria. She had esophageal dilatation, with an esophageal luminal diameter measured at the upper, middle, and lower thoracic esophagus of 2.5 cm, 2.5 cm, and 3.5 cm, respectively. The patient was admitted to the intensive care unit (ICU) due to ARDS from aspiration pneumonia. Our patient\'s complicated condition at the time of ICU admission with ARDS secondary to aspiration pneumonia was primarily due to esophageal dilatation and reflux. Aggressive anti-reflux pharmacotherapy and bed elevation may be beneficial in preventing pulmonary injury caused by aspiration. Esophageal complications are common in such patients and can have a substantial impact on the prognosis and quality of life. Regular medical attention is necessary to identify and manage any potential issues.
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  • 文章类型: Case Reports
    腕管综合征(CTS)是一种经常遇到的压迫性神经病,通常通过手术治疗。这里,我们介绍了一例罕见的病例,一例74岁女性在CTS手术后迅速出现皮肤硬化.这种情况最初被误诊为复杂的区域疼痛综合征。然而,自从她的皮肤状况进展后,她被转诊到风湿病科。随后的评估证实了弥漫性皮肤系统性硬化症的诊断,伴有间质性肺病.霉酚酸酯治疗并没有显着改变间质性肺阴影,但导致皮肤硬化的轻微改善。对于意外的术后症状患者,考虑风湿性疾病的可能性至关重要。
    Carpal tunnel syndrome (CTS) is a frequently encountered compressive neuropathy that is often treated surgically. Here, we present an unusual case of a 74-year-old female who developed a rapid emergence of skin sclerosis following CTS surgery. The condition was initially misdiagnosed as complex regional pain syndrome. However, since her skin condition progressed, she was referred to the rheumatology department. Subsequent evaluations confirmed the diagnosis of diffuse cutaneous systemic sclerosis, accompanied by interstitial lung disease. Treatment with mycophenolate mofetil did not notably alter the interstitial lung shadows but led to minor improvement in skin sclerosis. It is crucial to consider the possibility of rheumatic diseases in patients with unexpected postoperative symptoms.
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  • 文章类型: Journal Article
    弥漫性皮肤系统性硬化症可能发生在育龄妇女中。即使在出现与硬化症相关的症状之前,该人群的怀孕也与不良产科和产妇结局的风险显着增加有关。我们报告了一例30岁的弥漫性皮肤系统性硬化症妇女的妊娠管理和结局。她的怀孕过程很好,并得到了包括产科医生和风湿病学家在内的团体咨询的协助。阴道分娩是患者的首选,因为她的下腹部皮肤有不规则的皮肤紧绷。她接受了引产和脊髓-硬膜外联合镇痛,并成功交付。重要的是,这些怀孕需要计划,在可能的情况下,以便有机会提前为妇女及其伴侣提供咨询,并降低任何风险。这些怀孕应该被认为是高风险的,他们需要密切的产前监测和良好的监督专家多学科团队在高风险怀孕方面经验丰富。皮肤系统性硬化症患者的分娩管理具有挑战性,分娩镇痛阴道分娩是剖宫产的另一种选择。
    Diffuse cutaneous systemic sclerosis may occur in women of childbearing age. Pregnancies in this population are associated with a markedly increased risk of adverse obstetric and maternal outcomes even before the onset of symptoms related to sclerosis. We report a case involving the management and outcome of pregnancy in a 30-year-old woman with diffuse cutaneous systemic sclerosis. The course of her pregnancy was good and was assisted by a group consultation including obstetricians and rheumatologists. Vaginal delivery was the patient\'s preferred choice because she had irregular skin tightness in her lower abdominal skin. She underwent induction of labor and combined spinal-epidural analgesia, and successfully delivered. Importantly, these pregnancies need to be planned, where possible, to allow the opportunity to counsel women and their partners in advance and to decrease any risks. These pregnancies should be considered high risk, and they require close antenatal monitoring and good supervision from an expert multidisciplinary team experienced in high-risk pregnancies. The management of delivery for patients with cutaneous systemic sclerosis is challenging, and vaginal delivery with labor analgesia is an alternative option to cesarean section.
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  • 文章类型: Case Reports
    一名69岁的日本男子出现了高血压危象。肾组织学显示恶性肾硬化,包括洋葱皮图案,小动脉从小动脉到小叶间动脉的纤维蛋白样坏死。免疫学研究表明抗RNA聚合酶(RNAP)III抗体阳性,皮肤活检诊断为局限性皮肤系统性硬化症(LcSSc)是引起硬皮病肾危象(SRC)的潜在疾病。血管紧张素覆盖酶(ACE)抑制剂治疗和钙拮抗剂对他的肾脏疾病有效。尽管在弥漫性皮肤SSc(DcSSc)患者中已经报道了SRC和抗RNAPIII抗体之间的关联,该病例表明,通过检测抗RNAPIII抗体,也可以在LcSSc患者中诊断出SRC伴高血压急症伴恶性肾硬化。
    A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Angiotensin covering enzyme (ACE) inhibitor therapy and calcium antagonist were effective for his renal condition. Although an association between SRC and anti-RNAP III antibody has already been reported in patients with diffuse cutaneous SSc (Dc SSc), this case indicates that SRC with hypetensive emergency with malignant nephrosclerosis can also be diagnosed on patients with Lc SSc patients by the examination of anti-RNAP III antibody.
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  • 文章类型: Case Reports
    We report the case of a 58-year-old man who had an ulcer on the right middle finger that was cured by surgery 4 years before consultation with our department. A few years after the surgery, he noticed recurrence of the ulcer and sclerosis of the skin. At the initial examination, skin sclerosis was observed from the fingers to the upper arms and from the feet to the thighs. Pitting scars on the fingertips and punctured hemorrhages of the nail-fold capillaries were also present. Gastroscopy showed slight reflex esophagitis. Laboratory findings were positive for antinuclear antibody (ANA; 1:640) with a speckled and discrete speckled pattern. Anti-topoisomerase I (anti-topo I) antibody and anti-RNA polymerase III were negative, but anti-centromere antibody was positive in an enzyme-linked immunosorbent assay. Anti-Ku antibody was positive in an immunoprecipitation assay using extracts of the leukemia cell line K562. Therefore, the patient was diagnosed with diffuse cutaneous systemic sclerosis with anti-Ku and anti-centromere antibodies. Treatment with an oral antiplatelet agent, vitamin E, a proton pump inhibitor, and i.v. lipoprostaglandin E1 were started. Subsequently, there has been repeated recurrence of finger ulcers, but no muscle involvement has been detected since his first visit. This is the first reported case of systemic sclerosis with anti-Ku and anti-centromere antibodies.
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