中新网北京8月14日电 (记者 韦香惠) 想象一下，一个人，没有正常的面纹、鼻翼、嘴唇也特别薄，就像戴着一张“面具”，遮住了五官。这是一种什么样的折磨？硬皮病，就是这样一种听起来就让人心生畏惧的罕见病，以其独特的“面具脸”症状，让无数患者饱受身心双重折磨。
硬皮病病因尚不明确，截至目前，国际上仍没有针对硬皮病的较全面的流行病学资料，许多医生甚至没有听过硬皮病的名字。然而，硬皮病的生命危险极高，由于胶原纤维等细胞外基质沉积，会导致局限性或弥漫性皮肤增厚和纤维化，一旦影响心脏、肺等内脏器官，凶险万分，是死亡率最高的结缔组织病之一。
不少硬皮病患者还会出现自身免疫疾病、肿瘤，或者因为肢端溃疡引发全身败血症。“有一些患者，由于病情加重，我们甚至都没有来得及收到病房，患者在急诊就走了。”穆荣向中新健康介绍，那位十年前去世的患者在当时住院时已经出现了严重的肺纤维化，即使使用呼吸机辅助治疗，效果也不理想，病情突然加重，直至最后离世。
穆荣表示，硬皮病患者不仅要承受疾病带来的痛苦，由于对容貌造成了不可逆转的伤害，许多患者还会遭遇他人的非议，带来心理上的打击。
为了提高硬皮病的早期诊断率和治疗效果，穆荣设立了国内第一个硬皮病专病门诊。该门诊致力于为患者提供专业、全面的诊断和治疗服务，并通过早期发现硬皮病，对改善硬皮病患者的生活质量至关重要。在穆荣诊治的硬皮病患者中，约有90%在发病初期会出现雷诺现象。通过这一早期症状，医生可以及早识别硬皮病的风险，并采取相应的预防措施。
穆荣的专病门诊不仅为患者提供了专业的医疗服务，还提供了心理支持和社会支持，帮助患者及其家庭更好地应对疾病的挑战。通过这一门诊，患者能够获得更加精准的治疗方案，从而提高生活质量，减少疾病对健康的影响。
英语如下：
Rare Disease Specialist Outpatient Clinic: Unveiling the Lives Hidden Behind Masks
Keywords: Rare Disease, Specialty Outpatient Clinic, Founder
Beijing, August 14th, 2023 (Xinhua News Agency) – Imagine a person without normal facial features, with thin lips and nostrils, resembling a face covered by a “mask,” concealing their features. What kind of torment is this? Dermatomyositis is a rare disease that strikes fear upon hearing its name, characterized by its unique “mask face” symptom, causing countless patients to suffer from both physical and psychological torments.
The cause of dermatomyositis is still not fully understood, and to date, there is still no comprehensive epidemiological data available internationally for the disease. Many doctors have never heard of dermatomyositis. However, the life-threatening nature of dermatomyositis is extremely high, as it leads to thickening and fibrosis of the skin in a localized or systemic manner due to the deposition of collagen fibers and other extracellular matrix components. Once it affects internal organs such as the heart and lungs, the situation is extremely critical, making it one of the most fatal connective tissue diseases.
Many patients with dermatomyositis also suffer from autoimmune diseases, tumors, or systemic sepsis caused by ulcers on the extremities. “There are patients whose conditions worsened so rapidly that we didn’t even have time to admit them to the ward; they passed away in the emergency room.” Mu Rong told Xinhua Health, referring to a patient who passed away ten years ago and was already suffering from severe pulmonary fibrosis upon admission, despite the use of ventilators for support, the condition suddenly deteriorated and the patient eventually passed away.
Mu Rong stated that patients with dermatomyositis not only have to endure the pain caused by the disease but also face criticism from others due to the irreversible damage to their appearance, leading to psychological blows.
To improve the early diagnosis rate and treatment efficacy of dermatomyositis, Mu Rong established the first specialty outpatient clinic for dermatomyositis in the country. The clinic is dedicated to providing professional and comprehensive diagnostic and treatment services for patients, and early detection of dermatomyositis is crucial for improving the quality of life of patients with the disease. Among the patients Mu Rong has treated, about 90% experience Raynaud’s phenomenon in the early stages of the disease. Through this early symptom, doctors can identify the risk of dermatomyositis early and take preventive measures accordingly.
Mu Rong’s specialty outpatient clinic not only provides professional medical services but also offers psychological support and social support to help patients and their families better cope with the challenges of the disease. Through this clinic, patients can receive more precise treatment plans, thereby improving their quality of life and reducing the impact of the disease on their health.
【来源】http://www.chinanews.com/life/2024/08-14/10268559.shtml
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