 Original Article Published: 

04 January 2020

Evaluation of cognitive function in systemic

sclerosis patients: a pilot study
 Eman M. Khedr, 
 Noha Abo El Fetoh, 
 Rania M. Gamal, 
 Mona H. Elzohri, 
 Nashwa Mostafa A. Azoz & 
 Daniel E. Furst 

Abstract

Objective
To investigate cognitive dysfunction in adult patients with systemic sclerosis
(SSc) who had no known clinical neurological manifestations and to relate it
with other disease severity parameters.

Methods
In the present study, 20 SSc consecutive female patients, who met the 2013
American College of Rheumatology SSc criteria, were compared with 20
healthy age–, gender-, and educational status–matched volunteer hospital
workers. Mean age and duration of illness were 41.8 ± 12.52 and 6.9 ± 
5.4 years respectively. Mini-Mental State Examination (MMSE), Wechsler
Adult Intelligence scale (WAIS-III), and P300 component of event-related
potentials (ERPs) were used to evaluate cognitive function in SS subjectively
and objectively respectively.

Results
Sixty-five percent (13 out of 20) of SSc patients had MMSE score < 25, and
cognitive impairment. Despite the lack of clinically apparent neurological
manifestations, SSc patients had significantly low MMSE score, high
Deterioration Index (DI), and prolonged P300 latency compared with that of
the control group (P = 0.0001; 0.010 and 0.008 respectively). A significant
positive association was found between (DI) and the Medsger severity vascular
score (r = 0.518; P = 0.012).There were few differences between limited and
diffuse SSc.
Conclusions
To our knowledge, few studies highlighted that subclinical cognitive
impairment can occur in the course of SSc disease. Early diagnosis of cognitive
impairment should be investigated either subjectively (using psychometrics
tests as MMSE or WAIS-III) or objectively using P300 evoked related
potentials. Medsger severity vascular score seems to be closely related to
cognitive impairment.

Key points
• Cognitive impairment can be associated with Medsger Vascular severity score and the duration of illness.
• Further larger studies will be needed to estimate the effect of disease activity on cognitive function, to further
delineate the differences between limited and diffuse SSc in this area, and to understand the underlying
pathophysiological mechanisms causing cognitive impairment in patients with SSc.
• To investigate impaired cognitive function in patients with SSc, even in the absence of clinically apparent
neurological and vascular disease.