PO071 | WHEN SJÖGREN’S DISEASE MIMICS TTP
Abstract
Introduction. Sjögren’s disease is a systemic autoimmune disorder primarily affecting salivary and lacrimal glands, leading to hypofunction. It may occur as a primary condition or in association with other autoimmune diseases, including systemic lupus erythematosus (15%), rheumatoid arthritis (20%), and systemic sclerosis (30%). Key biomarkers include anti-SSA/Ro (most specific), anti-SSB/La, ANA, and rheumatoid factor. Diagnosis relies on a weighted score incorporating symptoms, serology, glandular involvement, Schirmer’s test, and salivary flow (1). Although xerophthalmia and xerostomia are common, the disease may involve multiple organs, with complication such as interstitial lung disease and lymphoma, impacting prognosis. Treatment is mainly symptomatic, with immunosuppressive therapy reserved for systemic involvement. Hematologic abnormalities, including cytopenias, may occur and rarely mimic thrombotic thrombocytopenic purpura (TTP), posing diagnostic challenges. Case Report. A 52-year-old man from Ivory Coast was referred to our Internal Medicine Unit with suspected TTP for therapeutic plasma exchange (PEX). He had presented to another Emergency Department with worsening performance status, hypotension, diffuse xerosis, severe thrombocytopenia (14,000/mm³), and hemolytic anemia (Hb 6.9 g/dL, LDH 430 U/L), without schistocytes and with normal renal function. Neurological evaluation and brain CT were normal. Based on a PLASMIC score of 7 and reduced ADAMTS13 activity (7%) measured at the referring hospital, corticosteroids and caplacizumab were started. On admission, no neurological, cardiac, or renal involvement was detected. PEX was initiated. However, after two sessions, platelet count remained ~12,000/mm³ and ADAMTS13 activity normalized (45%), leading to discontinuation of TTP-directed therapy. Repeat testing confirmed ADAMTS13 >40%, with persistent absence of schistocytes and normal haptoglobin. Further workup showed positive direct Coombs test (IgG), ANA 1:640, and high anti-SSA/SSB titers, consistent with Sjögren’s disease. Intravenous immunoglobulins (2 g/kg over five days) led to progressive improvement, with platelets >50,000/mm³ and hemoglobin recovery after transfusion. Despite a negative Schirmer’s test, systemic involvement was documented, including submandibular gland edema on CT, diffuse lymphadenopathy, and interstitial lung disease (possible NSIP/UIP pattern) with reduced DLCO (28%). Bone marrow biopsy and and PET imaging excluded lymphoproliferative disease. After cutaneous and hematological improvement (Hb 9.7 g/dL, platelets 125,000/mm³) he was discharged on tapering prednisone with multidisciplinary follow-up. Discussion and Conclusions. This case highlights Sjögren’s disease presenting with severe hematologic involvement mimicking TTP. Initial findings (thrombocytopenia, hemolytic anemia, reduced ADAMTS13) supported TTP, but normalization of ADAMTS13 without platelet response argued against it. Autoimmune cytopenias, though uncommon in Sjögren’s disease, include hemolytic anemia and immune thrombocytopenia (2). Differentiation from thrombotic microangiopathies is essential, as management differs. In this case, response to immunoglobulins supported an autoimmune mechanism. Transient ADAMTS13 reduction may be nonspecific. Integration of clinical evolution and treatment response is crucial to avoid misdiagnosis and inappropriate therapy. 1. Ramos-Casals M, EULAR, Ann Rheum Dis. 2020 2. Liu C, Front Immunol. 2026
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BibTeX
@article{castoldi2026po071,
title = {{PO071 | WHEN SJÖGREN’S DISEASE MIMICS TTP}},
author = {Alice Castoldi},
journal = {Bleeding, Thrombosis and Vascular Biology},
year = {2026},
month = oct,
url = {https://www.btvb.org/btvb/article/view/887},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - PO071 | WHEN SJÖGREN’S DISEASE MIMICS TTP
AU - Alice Castoldi
PY - 2026
DA - 2026/10/26
JO - Bleeding, Thrombosis and Vascular Biology
UR - https://www.btvb.org/btvb/article/view/887
AB - Introduction. Sjögren’s disease is a systemic autoimmune disorder primarily affecting salivary and lacrimal glands, leading to hypofunction. It may occur as a primary condition or in association with other autoimmune diseases, including systemic lupus erythematosus (15%), rheumatoid arthritis (20%), and systemic sclerosis (30%). Key biomarkers include anti-SSA/Ro (most specific), anti-SSB/La, ANA, and rheumatoid factor. Diagnosis relies on a weighted score incorporating symptoms, serology, glandular involvement, Schirmer’s test, and salivary flow (1). Although xerophthalmia and xerostomia are common, the disease may involve multiple organs, with complication such as interstitial lung disease and lymphoma, impacting prognosis. Treatment is mainly symptomatic, with immunosuppressive therapy reserved for systemic involvement. Hematologic abnormalities, including cytopenias, may occur and rarely mimic thrombotic thrombocytopenic purpura (TTP), posing diagnostic challenges. Case Report. A 52-year-old man from Ivory Coast was referred to our Internal Medicine Unit with suspected TTP for therapeutic plasma exchange (PEX). He had presented to another Emergency Department with worsening performance status, hypotension, diffuse xerosis, severe thrombocytopenia (14,000/mm³), and hemolytic anemia (Hb 6.9 g/dL, LDH 430 U/L), without schistocytes and with normal renal function. Neurological evaluation and brain CT were normal. Based on a PLASMIC score of 7 and reduced ADAMTS13 activity (7%) measured at the referring hospital, corticosteroids and caplacizumab were started. On admission, no neurological, cardiac, or renal involvement was detected. PEX was initiated. However, after two sessions, platelet count remained ~12,000/mm³ and ADAMTS13 activity normalized (45%), leading to discontinuation of TTP-directed therapy. Repeat testing confirmed ADAMTS13 >40%, with persistent absence of schistocytes and normal haptoglobin. Further workup showed positive direct Coombs test (IgG), ANA 1:640, and high anti-SSA/SSB titers, consistent with Sjögren’s disease. Intravenous immunoglobulins (2 g/kg over five days) led to progressive improvement, with platelets >50,000/mm³ and hemoglobin recovery after transfusion. Despite a negative Schirmer’s test, systemic involvement was documented, including submandibular gland edema on CT, diffuse lymphadenopathy, and interstitial lung disease (possible NSIP/UIP pattern) with reduced DLCO (28%). Bone marrow biopsy and and PET imaging excluded lymphoproliferative disease. After cutaneous and hematological improvement (Hb 9.7 g/dL, platelets 125,000/mm³) he was discharged on tapering prednisone with multidisciplinary follow-up. Discussion and Conclusions. This case highlights Sjögren’s disease presenting with severe hematologic involvement mimicking TTP. Initial findings (thrombocytopenia, hemolytic anemia, reduced ADAMTS13) supported TTP, but normalization of ADAMTS13 without platelet response argued against it. Autoimmune cytopenias, though uncommon in Sjögren’s disease, include hemolytic anemia and immune thrombocytopenia (2). Differentiation from thrombotic microangiopathies is essential, as management differs. In this case, response to immunoglobulins supported an autoimmune mechanism. Transient ADAMTS13 reduction may be nonspecific. Integration of clinical evolution and treatment response is crucial to avoid misdiagnosis and inappropriate therapy. 1. Ramos-Casals M, EULAR, Ann Rheum Dis. 2020 2. Liu C, Front Immunol. 2026
SN - 2785-5309
ER -