Atypical initial presentations of Sjogren’s syndrome: a case series Authors Faiza Naeem Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. Salman Khurshid Imami Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. https://orcid.org/0000-0001-5140-6623 Saira Elaine Anwer Khan Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. Shabnam Batool Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. Muhammad Akmal Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. Yasir Mushtaq Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan. DOI: https://doi.org/10.47391/JPMA.11555 Keywords: Primary Sjogren Syndrome, Takayasu Arteritis, Medium Vessel Vasculitis, Palpable Purpura, Thrombocytopenia Abstract Primary Sjogren’s syndrome (pSS) typically presents with Sicca symptoms xerostomia and xeropthalmia. This study highlights atypical presentations of Primary Sjogren's syndrome posing diagnostic and therapeutic challenges. Four female patients (median age 30 years, IQR = 15.5) with atypical pSS features, confirmed by positive Anti-Ro antibodies and negative lupus or rheumatoid arthritis tests, were treated between February and August 2023. PSS presented as erythema nodosum, soft tissue swelling with medium-vessel vasculitis, palpable purpura, and severe thrombocytopenia in these cases prior to Sicca symptoms. Initial treatment included steroids and hydroxychloroquine. Azathioprine, Methotrexate, and Cyclophosphamide were added as steroid-sparing agents. Three patients achieved remission. One patient with co-existing pulmonary tuberculosis (TB) died. Atypical features can occur in SS, requiring vigilance for diagnosis and individualised treatment. Our series demonstrates the diverse atypical clinical features of Sjogren's syndrome, encompassing radiologic, histopathologic, laboratory abnormalities, and treatment guidelines, expanding the known spectrum of disease presentation and management. Keywords: Primary Sjogren’s syndrome, Takayasu arteritis, Medium vessel vasculitis, Palpable purpura, Thrombocytopenia. Downloads Full Text Article Published 2024-12-23 How to Cite Faiza Naeem, Imami, S. K., Saira Elaine Anwer Khan, Shabnam Batool, Muhammad Akmal, & Yasir Mushtaq. (2024). Atypical initial presentations of Sjogren’s syndrome: a case series. Journal of the Pakistan Medical Association, 75(1), 125–130. https://doi.org/10.47391/JPMA.11555 More Citation Formats ACM ACS APA ABNT Chicago Harvard IEEE MLA Turabian Vancouver Download Citation Endnote/Zotero/Mendeley (RIS) BibTeX Issue Vol. 75 No. 1 (2025): JANUARY Section CASE SERIES License Copyright (c) 2024 Journal of the Pakistan Medical Association This work is licensed under a Creative Commons Attribution 4.0 International License.