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Research ArticleOriginal Article
Open Access

Disease phenotype and diagnostic delay in Saudi patients with primary Sjögren’s syndrome

An exploratory cross-sectional study

Mohammed A. Omair, Bashaer S. AlQahtani, Esam H. AlHamad, Yusra A. Tashkandy, Nashwa S. Othman, Khalid A. AlShahrani, Muthurajan P. Paramasivam, Fahidah AlEnzi, Rabih Halwani and Maha H. Daghestani
Saudi Medical Journal April 2021, 42 (4) 405-410; DOI: https://doi.org/10.15537/smj.2021.42.4.20200767
Mohammed A. Omair
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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  • For correspondence: [email protected]
Bashaer S. AlQahtani
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Esam H. AlHamad
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Yusra A. Tashkandy
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Nashwa S. Othman
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Khalid A. AlShahrani
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Muthurajan P. Paramasivam
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Fahidah AlEnzi
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Rabih Halwani
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Maha H. Daghestani
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
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Article Figures & Data

Figures

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    Figure 1

    - Characteristics of minor salivary gland biopsy results based on the focus score (n=24).

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    Figure 2

    - Frequency of extra-glandular manifestations (n=41).

Tables

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    Table 1

    - Demographics characteristics of patients included in this study.

    Demographicsn=41Rheumatology clinicPulmonary clinicP-value
    Female gender (%)32 (78)13 (93)19 (70)0.099
    Age at diagnosis (years)54.8 ± 13.446.9 ± 10.959±12.9<0.001
    Age at study inclusion (years)58.76 ± 12.752.9 ± 11.961.8 ± 12.20.003
    Disease duration (years)4.6 ± 2.36 ± 2.53.9 ± 1.80.003
    Time from symptom onset to diagnosis (years)
    Mean2.2 ± 2.4   
    Range1-11   
    Outcome measures
    Mean EULAR Sjogren’s Syndrome Disease Activity Index9.95 ± 7.736.43 ± 9.4111.78±6.130.100
    Mean EULAR Sjogren’s Syndrome Patient Reported Outcome5.17 ± 2.45.095 ± 2.7005.205±2.2390.386
    Mean Patient Global Assessment5.2 ± 2.374.786 ± 2.7235.423±2.1760.864
    Mean Physician Global Assessment4.46 ± 2.063.786 ± 2.4554.815±1.7770.879
    Antibody profile; n (%)
    ANA32 (78.0)13 (93)19 (70)0.099
    Anti-SSA19 (46.3)12 (86)7 (26)<0.001
    Anti-SSB12 (29.3)9 (64)3 (11)<0.001
    Rheumatoid factor10 (24.4)11 (79)8 (30)0.168
    Presence of extra-glandular manifestations36 (87.8)9 (64)27 (100)0.001
    • EULAR: The European League Against Rheumatism, SSA: Sjogren’s syndrome A antibody,SSB: Sjogren’s syndrome B antibody,

    • View popup
    Table 2

    - Master table of all cases included in the study.

    AgeAge at disease onsetGenderANASSASSBRFLip biopsyPulmo-naryRenalCuta-neousArthritisPNSCyto-peniaCNSPGAPhGAESSDAIESSPRI for drynessESSPRI for fatigueESSPRI for painESSPRI
    1816Female1111000000006706835.7
    3429Female1001110010002271010.7
    3932Female11131100100055128756.7
    4337Female1001110010005375555
    4340Female11011100100065175575.7
    4538Female11133011001022149304
    4942Male1113300000003103322.7
    5046Female110030000000107010101010
    5047Female10011100100043125112.3
    5248Female11113011101012200121
    5347Female1100300010007608255
    5348Female1000100001005205704
    5445Female1111300010104126403.3
    5446Female110011001000471987108.3
    5548Female00001100000047102512.7
    5550Female11103000100057271088.3
    5753Female01003000000063041097.7
    5753Female11101100100088125876.7
    5857Female0000110010005598587
    5856Female1000110010005475755.7
    6055Female1111300000001209134.3
    6050Female11113000010054274655
    6158Female11003100000074106897.7
    6159Female00001101100055205555
    6157Female1100110000005575354.3
    6359Female000001001000963410888.7
    6461Female10001100000086105997.7
    6554Female1110300100003265001.7
    6665Male1000110010001077610108.7
    6764Male00001100100053126524.3
    6964Female100030001111971997108.7
    6966Male0000110010003774724.3
    7066Male10001100100053180553.3
    7167Male1000110000001172000.7
    7168Male10001100100033122433
    7170Male10000100100067145665.7
    7370Male0000110010006575655.3
    7573Female1111110010004376635
    7574Female0000110010007575797
    7773Female1000110010105182333.2
    8379Female1000110010009690574
    • ANA: antinuclear antibody, SSA: Sjogren’s syndrome A antibody, SSB: Sjogren’s syndrome B antibody, RF: rheumatoid factor, PNS: peripheral nervous system, CNS: central nervous system, PGA: patient global assessment, PhGA: physician global assessment, ESSDAI: EULAR Sjogren’s syndrome disease activity score, ESSPRI: EULAR Sjogren’s syndrome patient reported index

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Saudi Medical Journal: 42 (4)
Saudi Medical Journal
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Disease phenotype and diagnostic delay in Saudi patients with primary Sjögren’s syndrome
Mohammed A. Omair, Bashaer S. AlQahtani, Esam H. AlHamad, Yusra A. Tashkandy, Nashwa S. Othman, Khalid A. AlShahrani, Muthurajan P. Paramasivam, Fahidah AlEnzi, Rabih Halwani, Maha H. Daghestani
Saudi Medical Journal Apr 2021, 42 (4) 405-410; DOI: 10.15537/smj.2021.42.4.20200767

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Disease phenotype and diagnostic delay in Saudi patients with primary Sjögren’s syndrome
Mohammed A. Omair, Bashaer S. AlQahtani, Esam H. AlHamad, Yusra A. Tashkandy, Nashwa S. Othman, Khalid A. AlShahrani, Muthurajan P. Paramasivam, Fahidah AlEnzi, Rabih Halwani, Maha H. Daghestani
Saudi Medical Journal Apr 2021, 42 (4) 405-410; DOI: 10.15537/smj.2021.42.4.20200767
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