Namas Rajaie, Elarabi Mohamed, Khan Saniya, Mubashir Asia, Memisoglu Esat, El-Kaissi Mahmoud, Joshi Abhay, Chapman Jeffrey, Jassim Imad, Khogali Hiba, Hassan Nada, Sabbour Hani, Saleh Khaled, Alnaqbi Khalid A, Zayat Ahmed S, Diab Sehriban, Awir Zyiad, Abu Taha Nehad, Ginawi Amel, Al Ansari Atheer, Rifaai Hazem, Alrawi Zaid, Al Dhaheri Afra, Ibrahim Gamal, Abogamal Ahmed, Al Shehhi Waleed, Teir Jamal, Khan Tahir, Musgrave Maisam, Hameed Beena, Khan Bhavna, Mosallam Nagwa, Hussien Nahla, Hussein Iman, Abdulelhamid Abeer, Ali Ahmed, Hannawi Suad, Al Izzi Mustafa, Badsha Humeira, Al Saleh Jamal
Division of Rheumatology, Department of Internal Medicine, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE.
Respiratory Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE.
J Scleroderma Relat Disord. 2023 Jun;8(2):137-150. doi: 10.1177/23971983221145788. Epub 2023 Jan 5.
Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group ( < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.
系统性硬化症是一种自身免疫性疾病，具有广泛的临床表现。登记系统有助于扩大对系统性硬化症的了解，并有助于患者护理和随访。本研究的目的是分析阿联酋系统性硬化症登记系统中一个大型队列中系统性硬化症的患病率，并找出不同亚组之间的显著异同。阿联酋所有硬皮病患者均纳入了这项多中心全国性回顾性分析。收集并分析了有关人口统计学、合并症、血清学特征、临床情况和治疗的数据，突出了所确定的最常见特征。共纳入了167名来自不同种族背景的系统性硬皮病患者。总体而言，54.5%（91/167）的患者被诊断为弥漫性皮肤型系统性硬化症，45.5%（76/167）为局限性皮肤型系统性硬化症。整个登记系统中系统性硬化症的患病率为每10万人1.66例，阿联酋患者为每10万人7.78例。几乎所有弥漫性皮肤型系统性硬化症和局限性皮肤型系统性硬化症组的患者免疫荧光抗核抗体检测均呈阳性。抗Scl-70抗体与弥漫性皮肤型系统性硬化症的相关性显著更高，而抗着丝点抗体与局限性皮肤型系统性硬化症组的相关性显著更高（<0.001）。就临床症状和器官受累情况而言，弥漫性皮肤型系统性硬化症患者的指端硬化、呼吸急促和指端溃疡比局限性皮肤型系统性硬化症亚型更为常见。毛细血管扩张在局限性皮肤型系统性硬化症组更为常见。此外，弥漫性皮肤型系统性硬化症患者的肺纤维化（间质性肺病）比局限性皮肤型系统性硬化症患者更多（70.5%对45.7%），肺动脉高压在局限性皮肤型系统性硬化症患者中的发生率是弥漫性皮肤型系统性硬化症患者的两倍。地方登记系统对于了解硬皮病的临床/血清学特征至关重要。本研究强调了提高疾病认识以及区分各种系统性硬化症亚组以实施针对患者的早期检测、更好管理和更高护理质量策略的重要性。
