Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis
Background: Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dys...
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SMC MEDIA SRL
2024-12-01
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| Series: | European Journal of Case Reports in Internal Medicine |
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| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/5026 |
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| author | Noura Alowais Noura Zamani Faisal Elbadawi Hassan Ahmed Pankhi Dutta Aesha Sharif |
| author_facet | Noura Alowais Noura Zamani Faisal Elbadawi Hassan Ahmed Pankhi Dutta Aesha Sharif |
| author_sort | Noura Alowais |
| collection | DOAJ |
| description | Background: Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger.
Case report: A 48-year-old female with an African ethnic background, diagnosed with SLE, presented with a 2-week history of high-grade fever, cough, and worsening arthralgia. She was initially admitted with suspected SLE flare secondary to community acquired pneumonia and was started on broad-spectrum antibiotics. Later during her hospital stay, she underwent extensive workup because of her clinical condition, as she had continuous spikes of fever, progressive cytopenia, accompanied with acute confusion state despite treatment. She was ultimately diagnosed with SLE flare complicated by MAS secondary to TBM in view of persistent fever, neuropsychiatric symptoms, hyperferritinemia and hypertriglyceridemia, supported by bone marrow aspiration findings, a tuberculosis (TB) positive contact and supporting cerebrospinal fluid analysis suggestive of TBM. She was immediately started on pulse doses of steroids and anti-tuberculosis medications in which significant clinical improvement was observed. She was discharged home, with continuity of care done at the outpatient department in addition to continuity of physical rehabilitation.
Conclusion: Given the wide range of clinical presentations and similarities between SLE and the life-threatening MAS, it is essential to maintain a high level of suspicion to establish the diagnosis and promptly start the necessary treatment to ensure better clinical and survival outcomes. |
| format | Article |
| id | doaj-art-1dd164cb097a4bada0364d7e11bcf8ec |
| institution | Kabale University |
| issn | 2284-2594 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj-art-1dd164cb097a4bada0364d7e11bcf8ec2025-01-07T13:40:28ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942024-12-0110.12890/2024_0050264561Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitisNoura Alowais0Noura Zamani1Faisal Elbadawi2Hassan Ahmed3Pankhi Dutta4Aesha Sharif5Internal Medicine, Dubai Health, Dubai, United Arab Emirates; Mohammed Bin Rashid University, Postgraduate Medical Education Division, Dubai, United Arab EmiratesRheumatology, Dubai Health, Dubai, United Arab EmiratesRheumatology, Dubai Health, Dubai, United Arab EmiratesMohammed Bin Rashid University, Postgraduate Medical Education Division, Dubai, United Arab Emirates; Rheumatology, Dubai Health, Dubai, United Arab EmiratesPathology, Dubai Health, Dubai, United Arab EmiratesInternal Medicine, Dubai Health, Dubai, United Arab Emirates; Mohammed Bin Rashid University, Postgraduate Medical Education Division, Dubai, United Arab EmiratesBackground: Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger. Case report: A 48-year-old female with an African ethnic background, diagnosed with SLE, presented with a 2-week history of high-grade fever, cough, and worsening arthralgia. She was initially admitted with suspected SLE flare secondary to community acquired pneumonia and was started on broad-spectrum antibiotics. Later during her hospital stay, she underwent extensive workup because of her clinical condition, as she had continuous spikes of fever, progressive cytopenia, accompanied with acute confusion state despite treatment. She was ultimately diagnosed with SLE flare complicated by MAS secondary to TBM in view of persistent fever, neuropsychiatric symptoms, hyperferritinemia and hypertriglyceridemia, supported by bone marrow aspiration findings, a tuberculosis (TB) positive contact and supporting cerebrospinal fluid analysis suggestive of TBM. She was immediately started on pulse doses of steroids and anti-tuberculosis medications in which significant clinical improvement was observed. She was discharged home, with continuity of care done at the outpatient department in addition to continuity of physical rehabilitation. Conclusion: Given the wide range of clinical presentations and similarities between SLE and the life-threatening MAS, it is essential to maintain a high level of suspicion to establish the diagnosis and promptly start the necessary treatment to ensure better clinical and survival outcomes.https://www.ejcrim.com/index.php/EJCRIM/article/view/5026hemophagocytic lymphohistiocytosis (hlh)macrophage activation syndrome (mas)systemic lupus erythematosus (sle)national early warning score (news) |
| spellingShingle | Noura Alowais Noura Zamani Faisal Elbadawi Hassan Ahmed Pankhi Dutta Aesha Sharif Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis European Journal of Case Reports in Internal Medicine hemophagocytic lymphohistiocytosis (hlh) macrophage activation syndrome (mas) systemic lupus erythematosus (sle) national early warning score (news) |
| title | Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| title_full | Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| title_fullStr | Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| title_full_unstemmed | Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| title_short | Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| title_sort | macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis |
| topic | hemophagocytic lymphohistiocytosis (hlh) macrophage activation syndrome (mas) systemic lupus erythematosus (sle) national early warning score (news) |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/5026 |
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