Abstract
Background:Primary SjÖgren´s Syndrome (PSS) is an autoimmune disease characterized by sicca symptoms, autoantibodies and lymphocytic infiltrate in exocrine glands.Classification and diagnosis of PSS is performed by applying 2002 AECG, 2012 ACR or 2016 ACR-EULAR criteria.Time of evolution of symptoms has been associated with different clinical, serological and pathology findings.Objectives:Document sicca profiles and compare findings according to time of evolution of symptoms.Methods:A descriptive retrospective observational study was performed in 116 patients with sicca symptoms in a Rheumatology center in México.Patients were separated according to time of evolution of sicca symptoms in ≤1 year, ≤2 years, ≤3 years, and ≥3 years. Clinical, serological, and histopathology features were assessed at each established time.Time from symptom onset to diagnosis was reported in median and interquartil range (IQR); age at symptom onset and age at diagnosis was expressed in mean and standard deviation (SD).Patients were classified as PSS if they fullfilled the 2002 AECG or 2016 ACR-EULAR criteria. Serology of Rheumatoid factor (RF), ANA, Anti-Ro, and Anti-La were documented.Description of MSGB was performed reporting presence of foci, lobules, atrophy, adipose tissue infiltration and ductal dilatation.Results:One hundred and sixteen sicca patients had a complete profile and 97 (83.62%) fulfilled AECG 2002 SSP criteria. Eighty-two (70.6%) patients with an ocular staining score were able to classify as PSS using the 2016 ACR-EULAR criteria.Of the sicca cohort, 112 (96.6%) were female. The mean age of symptom onset was 48.4 (SD 13.11) years, and the mean age at diagnosis was 53.33 (SD 12.43) years. The median time from symptom onset to diagnosis was 36 (IQR 12-84) months.Fullfilment the 2002 AECG PSS classification criteria according to duration of sicca symptoms is described in Table 1.Table 1.Classification PSS criteria according to time of evolution of sicca symptoms<12 months<24 months<36 months>37 monthsOral symptoms28%40%48%89%Ocular symptoms26%39%47%93%Altered Salivary flow rate test21%32%41%81%Altered Schirmer test19%28%38%75%Positive Anti Ro13%19%26%47%Positive Anti La3%6%10%16%≥1 foci score/4mm214%24%34%67%2002 AECG Criteria*22%34%44%84%*AECG-American European Consensus Group0%25%50%75%100%Extra-classification criteria such as severe oral symptoms (choking, cracker cookie) were more common with prolonged symptom duration. Figure 1Figure 1.Clinical, serologic and histopathologic findings according to sicca symptom durationConclusion:The probability and capability of fulfilling PSS criteria is time dependent, this should be taken into consideration when evaluating patients referring sicca symptoms. Longer symptom duration was associated with more severe clinical, serological and histopathology profiles.
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