Autopsy versus clinical findings in patients with systemic sclerosis in a case series from patients of the EUSTAR database

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 find...

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Published in:	Clinical and experimental rheumatology Vol. 33; no. 4 Suppl 91; pp. S75 - S79
Main Authors:	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
Format:	Journal Article
Language:	English
Published:	Italy 01-07-2015
Subjects:	Adult Aged Aged, 80 and over Autopsy Cause of Death Databases, Factual Disease Progression Europe - epidemiology Female Gastrointestinal Tract - pathology Humans Kidney - pathology Lung - pathology Male Middle Aged Musculoskeletal System - pathology Myocardium - pathology Predictive Value of Tests Prevalence Retrospective Studies Scleroderma, Systemic - complications Scleroderma, Systemic - diagnosis Scleroderma, Systemic - mortality Scleroderma, Systemic - pathology Surveys and Questionnaires Young Adult Europe
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Summary:	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.
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ISSN:	0392-856X