MACROPHAGE ACTIVATION SYNDROME SECONDARY TO SJÖGREN’S SYNDROME: A RARE MULTISYSTEMIC CASE
- 1Universidade de Brasília (UnB), Brasília, DF, Brazil
- 2Hospital DF Star, Brasília, DF, Brazil
Abstract
Introduction: Macrophage activation syndrome (MAS) is a rare and potentially fatal hyperinflammatory disorder caused by excessive macrophage and T-cell activation with a mortality rate of 20% to 53%. It may be primary or secondary to infections, malignancies, or autoimmune diseases. Its presentation may mimic other conditions. MAS associated with Sjögren’s syndrome (SS) is exceptionally rare. We report a case of MAS secondary to SS with multisystemic and neurological involvement. Case Report: A 44-year-old female with recently diagnosed SS and Hashimoto thyroiditis was admitted with fatigue, arthralgia, worsening weakness and 5 days of fever (38–39°C). Previous testing showed ANA 1:640, strongly positive anti-Ro/SSA, and elevated rheumatoid factor. Screening for dengue, COVID-19 and influenza was negative. After hospitalization she developed altered consciousness, diffuse rash, renal and liver dysfunction, coagulopathy, and multiorgan involvement. Laboratory findings included leukopenia (3,100/mm 3 ), thrombocytopenia (87,000/mm 3 ), ferritin 81,765 ng/mL, fibrinogen 61–109 mg/dL, triglycerides 380 mg/dL, AST 892 U/L, LDH 7,169 U/L, and creatinine 4.26 mg/dL. Hepatomegaly and pleural effusion were evidenced by tomography. Infection and autoimmune flare were initially considered, but the overall pattern suggested MAS. Bone marrow evaluation showed hemophagocytosis and brain MRI was compatible with metabolic leukoencephalopathy and possible posterior reversible encephalopathy syndrome. Treatment included corticosteroid pulsotherapy, intravenous immunoglobulin, broad-spectrum antimicrobials, rituximab, anakinra, ruxolitinib, intrathecal chemotherapy and intensive support. Despite severe complications including acute renal failure, tracheostomy, muscular hematomas and retinal hemorrhage the patient recovered, with improvement in liver and renal function and recovery from hematologic and neurologic manifestations. At discharge, she was alert, oriented and undergoing tracheostomy occlusion. Maintenance therapy included prednisone taper, hydroxychloroquine, mycophenolate mofetil, and rituximab every 6-months. Discussion: MAS associated with Sjögren’s syndrome is exceptionally rare and may be underrecognized because its manifestations overlap with infection and autoimmune disease activity. In a systematic review of 421 patients with rheumatic disease-associated hemophagocytic syndrome, only 3 cases were related to SS. This rarity reinforces the need to consider MAS in SS patients with rapidly progressive hyperinflammation and multiorgan dysfunction. Since no standardized therapy exists for autoimmune disease-associated MAS, treatment must be individualized according to severity, suspected triggers and clinical response. In the reported case disease progression despite initial corticosteroids and intravenous immunoglobulin required escalation to rituximab, anakinra and ruxolitinib, reflecting a multitarget approach to the cytokine cascade in a severe disease. The subsequent hematologic, renal, hepatic, and neurologic improvement supports the importance of early recognition, prompt treatment escalation and continuous infection surveillance during immunosuppression.
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BibTeX
@article{amorim2026macrophage,
title = {{MACROPHAGE ACTIVATION SYNDROME SECONDARY TO SJÖGREN’S SYNDROME: A RARE MULTISYSTEMIC CASE}},
author = {Maria Eduarda SANTOS DE AMORIM and Eduarda RODRIGUES MARTINS and Lívia PAIVA XAVIER and Maria Gabriela BARBOSA RODRIGUES and Pedro DA CUNHA NOGUEIRA and Vinícius QUIRINO FERREIRA E SILVA and Verena PORTELA CARREIRO and Andreia Cristina BALBINO and Fernando SÉRGIO BLUMM FERREIRA and Flávia DIAS XAVIER},
journal = {Hematology, Transfusion and Cell Therapy},
year = {2026},
month = oct,
doi = {10.1016/j.htct.2026.108504},
url = {https://doi.org/10.1016/j.htct.2026.108504},
note = {CC BY},
}
RIS
TY - JOUR
TI - MACROPHAGE ACTIVATION SYNDROME SECONDARY TO SJÖGREN’S SYNDROME: A RARE MULTISYSTEMIC CASE
AU - Maria Eduarda SANTOS DE AMORIM
AU - Eduarda RODRIGUES MARTINS
AU - Lívia PAIVA XAVIER
AU - Maria Gabriela BARBOSA RODRIGUES
AU - Pedro DA CUNHA NOGUEIRA
AU - Vinícius QUIRINO FERREIRA E SILVA
AU - Verena PORTELA CARREIRO
AU - Andreia Cristina BALBINO
AU - Fernando SÉRGIO BLUMM FERREIRA
AU - Flávia DIAS XAVIER
PY - 2026
DA - 2026/10/23
JO - Hematology, Transfusion and Cell Therapy
DO - 10.1016/j.htct.2026.108504
UR - https://doi.org/10.1016/j.htct.2026.108504
AB - Introduction: Macrophage activation syndrome (MAS) is a rare and potentially fatal hyperinflammatory disorder caused by excessive macrophage and T-cell activation with a mortality rate of 20% to 53%. It may be primary or secondary to infections, malignancies, or autoimmune diseases. Its presentation may mimic other conditions. MAS associated with Sjögren’s syndrome (SS) is exceptionally rare. We report a case of MAS secondary to SS with multisystemic and neurological involvement. Case Report: A 44-year-old female with recently diagnosed SS and Hashimoto thyroiditis was admitted with fatigue, arthralgia, worsening weakness and 5 days of fever (38–39°C). Previous testing showed ANA 1:640, strongly positive anti-Ro/SSA, and elevated rheumatoid factor. Screening for dengue, COVID-19 and influenza was negative. After hospitalization she developed altered consciousness, diffuse rash, renal and liver dysfunction, coagulopathy, and multiorgan involvement. Laboratory findings included leukopenia (3,100/mm 3 ), thrombocytopenia (87,000/mm 3 ), ferritin 81,765 ng/mL, fibrinogen 61–109 mg/dL, triglycerides 380 mg/dL, AST 892 U/L, LDH 7,169 U/L, and creatinine 4.26 mg/dL. Hepatomegaly and pleural effusion were evidenced by tomography. Infection and autoimmune flare were initially considered, but the overall pattern suggested MAS. Bone marrow evaluation showed hemophagocytosis and brain MRI was compatible with metabolic leukoencephalopathy and possible posterior reversible encephalopathy syndrome. Treatment included corticosteroid pulsotherapy, intravenous immunoglobulin, broad-spectrum antimicrobials, rituximab, anakinra, ruxolitinib, intrathecal chemotherapy and intensive support. Despite severe complications including acute renal failure, tracheostomy, muscular hematomas and retinal hemorrhage the patient recovered, with improvement in liver and renal function and recovery from hematologic and neurologic manifestations. At discharge, she was alert, oriented and undergoing tracheostomy occlusion. Maintenance therapy included prednisone taper, hydroxychloroquine, mycophenolate mofetil, and rituximab every 6-months. Discussion: MAS associated with Sjögren’s syndrome is exceptionally rare and may be underrecognized because its manifestations overlap with infection and autoimmune disease activity. In a systematic review of 421 patients with rheumatic disease-associated hemophagocytic syndrome, only 3 cases were related to SS. This rarity reinforces the need to consider MAS in SS patients with rapidly progressive hyperinflammation and multiorgan dysfunction. Since no standardized therapy exists for autoimmune disease-associated MAS, treatment must be individualized according to severity, suspected triggers and clinical response. In the reported case disease progression despite initial corticosteroids and intravenous immunoglobulin required escalation to rituximab, anakinra and ruxolitinib, reflecting a multitarget approach to the cytokine cascade in a severe disease. The subsequent hematologic, renal, hepatic, and neurologic improvement supports the importance of early recognition, prompt treatment escalation and continuous infection surveillance during immunosuppression.
SN - 2531-1379
SN - 2531-1387
ER -