Recurrent Immune-Mediated Thrombotic Thrombocytopenic Purpura in the Setting of Suspected Sjögren's Syndrome.
- 1Division of Rheumatology, Department of Medicine, TidalHealth, Salisbury, USA.
Abstract
A 70-year-old African American female with a past history of relapsing/remitting thrombotic thrombocytopenic purpura (TTP), hypertensive retinopathy, and breast cancer was evaluated in the rheumatology clinic for a possible autoimmune rheumatic disease. Earlier, she had followed up with a community rheumatologist who diagnosed her with systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). She was being treated with hydroxychloroquine, but this was discontinued after her hospitalization for a TTP flare. Investigations revealed the presence of anti-nuclear antibodies (ANA) and anti-SSA antibodies. Anti-double-stranded DNA (dsDNA) and anti-Smith antibodies were not present. Her main complaints were dry eyes and dry mouth, and there were no rashes, mouth sores, alopecia, serositis or polyarthritis. Although she had a positive ANA, the available clinical and serologic documentation did not establish sufficient additional criteria to fulfill the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria for SLE. Her relapsing TTP was treated with multiple courses of rituximab and plasmapheresis with ADAMTS13 level monitoring. Mycophenolate mofetil (MMF) was added as a steroid-sparing immunosuppressive agent in the setting of recurrent immune-mediated TTP, ongoing corticosteroid exposure, and concomitant autoimmune features suggestive of Sjögren disease. Thereafter, the patient remained stable without another documented TTP relapse during follow-up. This temporal association does not establish that MMF prevented TTP relapse. This case report emphasizes an interesting association between suspected Sjögren disease and recurrent TTP and highlights the diagnostic and therapeutic challenges of managing recurrent TTP in the setting of systemic autoimmune features.
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BibTeX
@article{amare2026recurrent,
title = {{Recurrent Immune-Mediated Thrombotic Thrombocytopenic Purpura in the Setting of Suspected Sjögren's Syndrome.}},
author = {Biruk Amare and Sajid Ehsan and Imran Yousaf and Deepak Gupta and Hannah Rupert},
journal = {Cureus},
year = {2026},
month = sep,
doi = {10.7759/cureus.117022},
url = {https://europepmc.org/article/MED/42948852},
note = {CC BY},
}
RIS
TY - JOUR
TI - Recurrent Immune-Mediated Thrombotic Thrombocytopenic Purpura in the Setting of Suspected Sjögren's Syndrome.
AU - Biruk Amare
AU - Sajid Ehsan
AU - Imran Yousaf
AU - Deepak Gupta
AU - Hannah Rupert
PY - 2026
DA - 2026/09/27
JO - Cureus
DO - 10.7759/cureus.117022
UR - https://europepmc.org/article/MED/42948852
AB - A 70-year-old African American female with a past history of relapsing/remitting thrombotic thrombocytopenic purpura (TTP), hypertensive retinopathy, and breast cancer was evaluated in the rheumatology clinic for a possible autoimmune rheumatic disease. Earlier, she had followed up with a community rheumatologist who diagnosed her with systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). She was being treated with hydroxychloroquine, but this was discontinued after her hospitalization for a TTP flare. Investigations revealed the presence of anti-nuclear antibodies (ANA) and anti-SSA antibodies. Anti-double-stranded DNA (dsDNA) and anti-Smith antibodies were not present. Her main complaints were dry eyes and dry mouth, and there were no rashes, mouth sores, alopecia, serositis or polyarthritis. Although she had a positive ANA, the available clinical and serologic documentation did not establish sufficient additional criteria to fulfill the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria for SLE. Her relapsing TTP was treated with multiple courses of rituximab and plasmapheresis with ADAMTS13 level monitoring. Mycophenolate mofetil (MMF) was added as a steroid-sparing immunosuppressive agent in the setting of recurrent immune-mediated TTP, ongoing corticosteroid exposure, and concomitant autoimmune features suggestive of Sjögren disease. Thereafter, the patient remained stable without another documented TTP relapse during follow-up. This temporal association does not establish that MMF prevented TTP relapse. This case report emphasizes an interesting association between suspected Sjögren disease and recurrent TTP and highlights the diagnostic and therapeutic challenges of managing recurrent TTP in the setting of systemic autoimmune features.
SN - 2168-8184
ER -