ArticleRheumatology and therapy2021
Sjögren's Syndrome Associated With Thrombotic Thrombocytopenic Purpura: A Case-Based Review.
Article in Rheumatology and therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 9 citations in OpenAlex.
- Risk of venous, arterial, and rare thrombotic events in adults with Sjögren's disease: a systematic review and meta-analysis.Reumatologia · 2026Review
- Thrombotic thrombocytopenic purpura and macrophage activation syndrome secondary to Sjögren's syndrome: a case report.Frontiers in immunology · 2026Article
- Raising Suspicion of Thrombotic Thrombocytopenic Purpura in a Patient With a Pre-existing Autoimmune Disease.Cureus · 2025Article
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- An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren's Syndrome Induced by a COVID-19 Immune Response.The Neurohospitalist · 2022Article
- Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report.Hematology reports · 2022Article
- Sequential Immune Thrombocytopenia (ITP) and Thrombotic Thrombocytopenic Purpura (TTP) in an Elderly Male Patient with Primary Sjogren's Syndrome: When in Doubt, Use the PLASMIC Score.Case reports in medicine · 2021Article
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Authors and funding
2 authors at 2 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo review all published cases of the rare association between thrombotic thrombocytopenic purpura (TTP) and Sjögren's syndrome (SS). The authors report an additional case of this unique association.
methodsSystematic review of the literature and a case report. The database were articles published in PubMed/MEDLINE, Web of Science, LILACS, and SciELO, registered from 1966 to August 2020. The DESH terms were "Sjögren's syndrome" and "thrombotic thrombocytopenic purpura," without language limitation.
resultsMost patients were female (88%), and the age varied from 30 to 75 years old. Concerning the sequence of disease appearance, SS followed by TTP was seen in seven articles, TTP and SS in three, and simultaneous appearance of both diseases in three studies. Primary SS was observed in 16 patients, and secondary SS was detected in two cases: dermatomyositis and rheumatoid arthritis. Anemia was the most common TTP manifestation, followed by thrombocytopenia, fever, consciousness alteration, renal impairment, and schistocytes' appearance on a blood smear. Treatment involved plasmapheresis, plasma exchange, rituximab, glucocorticoid, and cyclophosphamide. A good outcome was noted in most studies; few patients died.
conclusionsTTP is a rare manifestation associated with SS. After the TTP diagnosis, plasmapheresis and/or plasma exchange should be immediately implemented.
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