Abstract
BackgroundSystemic sclerosis (SSc) has been suggested to cause exocrine pancreatic dysfunction. However, a case-control-based autopsy study failed to associate systemic sclerosis with any pancreatic histopathology. The primary objective of this study was to examine the exocrine pancreatic function in consecutive SSc patients in relation to an age- and sex-matched control group. A secondary objective was to relate exocrine pancreatic function to radiological, laboratory, and clinical SSc characteristics.MethodsOne hundred twelve consecutive patients fulfilling the 2013 American Congress of Rheumatology/European League Against Rheumatism criteria for SSc and 52 control subjects were matched for sex and age. Exocrine pancreatic function was assessed by ELISA-based measurement of fecal elastase, and levels ≤ 200 μg/g were considered pathological, i.e., representing exocrine pancreatic insufficiency. Patients were characterized regarding SSc manifestations including gastrointestinal and hepatobiliary function, by use of laboratory and clinical examinations. Pancreas parenchyma characteristics were evaluated by high-resolution computer tomography (HRCT).ResultsA similar proportion of subjects exhibited pathological levels of fecal elastase among SSc patients (6/112; 5.4%) and control subjects (3/52; 5.8%). Patients with fecal elastase ≤ 200 μg/g did not differ from other SSc patients with respect to laboratory and clinical characteristics, including malnutrition. SSc subjects with low levels of fecal elastase displayed significantly lower pancreas attenuation on HRCT examinations compared to the control subjects.ConclusionsIn this study encompassing 112 consecutive SSc patients and 52 matched control subjects, we were unable to associate systemic sclerosis with clinically significant exocrine pancreatic dysfunction.
Highlights
Systemic sclerosis (SSc) is a heterogeneous systemic disease characterized by the development of autoimmunity, vasculopathy, and multiorganic fibrosis
Clinical characteristics SSc was defined according to the 2013 American Congress of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria, and patients were subdivided into diffuse cutaneous SSc and limited cutaneous SSc [12, 13]
We identified an age-dependent variation in pancreas attenuation both in the SSc subjects (r = − 0.39, p = 0.041) and the control subjects (r = − 0.45, p = 0.044)
Summary
Systemic sclerosis (SSc) is a heterogeneous systemic disease characterized by the development of autoimmunity, vasculopathy, and multiorganic fibrosis. The measurement of fecal elastase (FE) has been established as a reliable method to screen for clinically significant EPI with a reported sensitivity above 90% [8]. Assessment of exocrine pancreatic function by FE measurement has been recommended in the evaluation of SSc-associated malnutrition and steatorrhea [9, 10]. Systemic sclerosis (SSc) has been suggested to cause exocrine pancreatic dysfunction. A case-control-based autopsy study failed to associate systemic sclerosis with any pancreatic histopathology. The primary objective of this study was to examine the exocrine pancreatic function in consecutive SSc patients in relation to an age- and sex-matched control group. A secondary objective was to relate exocrine pancreatic function to radiological, laboratory, and clinical SSc characteristics
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