PRIMARY SJÖGREN’S SYNDROME: CLINICOPATHOLOGIC REVIEW OF 20 CASES USING THE ACR/EULAR CRITERIA

Primary Sjogren’s syndrome

Abstract

OBJECTIVE: Sjögren’s Syndrome (SS) is a systemic autoimmune 
disorder that may present a diagnostic challenge in low and middle
income settings. The American College of Rheumatologists (ACR) 
and European League against Rheumatism (EULAR) in 2016 
developed diagnostic criteria. However, a dearth of literature exists 
on the clinico-pathologic pattern and the diagnostic criteria used to 
diagnose primary Sjögren’s syndrome in Nigeria.  
METHODOLOGY: This is a single-center retrospective review of 
20 cases seen at the Oral Medicine clinic that underwent an 
evaluation for suspected primary Sjogren’s Syndrome (pSS). We 
report the clinical and histopathology of the 20 cases, and evaluated 
these cases based on the diagnostic criteria by the ACR/EULAR. 

RESULTS: The survey revealed a series of 20 cases. There were 14 
females and 6 males with F: M ratio of 2.3:1. The study participants 
were from 18 to 78 years, with a mean age of 46.9 (± 15.9). Of all 
the cases reviewed, about 35% (n=7) met the ACR/EULAR criteria, 
and oral symptoms of dry mouth, 19(95%), was the most common 
symptom seen. Symptoms of persistent dry eyes and a feeling of 
gritty sensations in the eyes were equally common symptoms in the 
ocular region in 9(45%). Atypical symptoms in form of cough and 
sore throat resulting from dry mucosa were also reported in 15% 
(n=3) of cases reviewed. 
CONCLUSION: Oral symptoms appear to be relatively common in 
the cases reviewed, while the ACR/EULAR criteria confirmed 
diagnosis in a third of the cases evaluated.