Sandmeier Bettina, Jäger Veronika K, Nagy Gabriella, Carreira Patricia E, Tzankov Alexandar, Widuchowska Malgorzata, Antic Milos, Distler Oliver, Reichert Helena, Distler Jörg H W, Walker Ulrich A, Hügle Thomas
Department of Rheumatology, University Hospital Basel, Switzerland.
Department of Rheumatology and Immunology, University of Pécs, Hungary.
Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S75-9. Epub 2015 Sep 1.
Subclinical organ pathology occurs regularly in systemic sclerosis (SSc) and affects correct prognosis as well as treatment choices. We aimed to evaluate autopsy data for organ involvement with subsequent correlation to clinical data in order to assess discrepancies in pathological and clinical findings in SSc.
A standardised autopsy questionnaire from diseased patients registered in the European Scleroderma Trials and Research group (EUSTAR) cohort was analysed on cause of death and various manifestations in different organ systems. Clinical data obtained from the EUSTAR database of the corresponding patients including cause of death and disease manifestations of lung, heart, kidney, gastrointestinal, skin or musculoskeletal organ involvement were retrospectively analysed and compared to autopsy data.
11 patients (6 women, 5 male) aged between 23 and 84 were included. Cause of death defined by pathologist and clinician were identical in 9/11 cases. In 8 individuals, cause of death was related to heart and lung pathologies. Heart and lung involvement (both 10/11) were the most frequently detected organ involvement at autopsy. Here, myocardial fibrosis occurred in 66% and lung fibrosis in 50% of the patients. Clinically, diastolic function abnormalities (6/11), conduction block (4/11), reduced DCLO (6/11) and dyspnea (8/11) were the most prevalent cardiopulmonary findings. For heart and renal involvement we found higher prevalence in autopsy than by clinical diagnosis. Especially myocardial fibrosis and renal arteriosclerosis were only obtained by autopsy in several individuals.
Clinical diagnostic procedures are limited in detection of end-organ damage, especially for cardiac involvement. All the more post mortem examinations are needed for quality verification of clinical diagnosis and might help as to better understand the disease processes as well as to improve patient care.
亚临床器官病理学在系统性硬化症（SSc）中经常出现，影响正确的预后以及治疗选择。我们旨在评估器官受累的尸检数据，并将其与临床数据进行后续关联，以评估SSc病理和临床发现之间的差异。
对欧洲硬皮病试验与研究组（EUSTAR）队列中登记的患病患者的标准化尸检问卷进行分析，内容包括死亡原因和不同器官系统的各种表现。回顾性分析从相应患者的EUSTAR数据库中获得的临床数据，包括死亡原因以及肺、心脏、肾脏、胃肠道、皮肤或肌肉骨骼器官受累的疾病表现，并与尸检数据进行比较。
纳入了11名年龄在23至84岁之间的患者（6名女性，5名男性）。病理学家和临床医生确定的死亡原因在9/11的病例中相同。在8名个体中，死亡原因与心脏和肺部病变有关。心脏和肺部受累（均为10/11）是尸检时最常检测到的器官受累情况。其中，66%的患者出现心肌纤维化，50%的患者出现肺纤维化。临床上，舒张功能异常（6/11）、传导阻滞（4/11）、弥散性肺一氧化碳（DCLO）降低（6/11）和呼吸困难（8/11）是最常见的心肺表现。对于心脏和肾脏受累，我们发现尸检中的患病率高于临床诊断。特别是心肌纤维化和肾动脉硬化仅在数名个体的尸检中发现。
临床诊断程序在检测终末器官损伤方面存在局限性，尤其是对于心脏受累。因此，更需要进行尸检以验证临床诊断的质量，这可能有助于更好地理解疾病过程并改善患者护理。
