Publication: Influence of the age at diagnosis in the disease expression of primary sjögren syndrome. analysis of 12,753 patients from the sjögren big data consortium
Loading...
Files
Date
2021-01-01
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Abstract
Objective. To analyse how the main
components of the disease phenotype
(sicca symptoms, diagnostic tests, immunological markers and systemic disease)
can be driven by the age at diagnosis of
primary Sjögren’s syndrome (pSS).
Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the
2002/2016 classification criteria for pSS.
The age at diagnosis was defined as the
time when the attending physician confirmed fulfilment of the criteria. Patients
were clustered according to age at diagnosis. 50 clusters with more than 100
observations (from 27 to 76 years) were
used to study the influence of the age at
diagnosis in the disease expression.
Results. There was a consistent increase
in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed
at the youngest ages and >95% in those
diagnosed at the oldest ages. The smooth
curves that best fitted a linear model
were the frequency of dry mouth (adjusted R2
0.87) and the frequency of abnormal oral tests (adjusted R2
0.72). Therefore, for each 1-year increase in the age
at diagnosis, the frequency of dry mouth
increased by 0.13%, and the frequency of
abnormal oral diagnostic tests by 0.11%.
There was a consistent year-by-year
decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the
linear models, for each 1-year increase
in the age at diagnosis, the frequency
of a positive result decreased by 0.57%
(for anti-Ro antibodies), 0.47% (for RF)
and 0.42% (for anti-La antibodies). The
ESSDAI domains which showed a more
consistent decrease were glandular and
lymph node involvement (for each 1-year
increase in the age at diagnosis, the frequency of activity decreased by 0.18%),
and constitutional, cutaneous, and haematological involvements (the frequency
decreased by 0.09% for each 1-year
increase). In contrast, other domains
showed an ascending pattern, especially
pulmonary involvement (for each 1-year
increase in the age at diagnosis, the frequency of activity increased by 0.22%),
and peripheral nerve involvement (the
frequency increased by 0.09% for each
1-year increase).
Conclusion. The influence of the age at
diagnosis on the key phenotypic features
of pSS is strong, and should be considered critical not only for designing a
personalised diagnostic approach, but
also to be carefully considered when analysing the results of diagnostic tests and
immunological parameters, and when
internal organ involvement is suspected
at diagnosis
Description
Keywords
Sjögren’s syndrome, age, disease phenotype, immunological markers
Citation
Retamozo S., Acar-Denizli N., Horváth I. F., Ng W., Rasmussen A., Dong X., Li X., Baldini C., Olsson P., Priori R., et al., "Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium.", Clinical and experimental rheumatology, cilt.39, sa.6, ss.166-174, 2021