Macrophage activation syndrome in a Case of dermatomyositis overlapping syndrome with systemic lupus erythematosus: A case report
Bibliographic record
Abstract
Introduction Macrophage activation syndrome (MAS) is a rare but aggressive life-threatening auto-immune disease. It is characterized by fever, rash, splenomegaly, blood cytopenia, hypertriglyceridemia, high ferritin levels, liver insufficiency, coagulopathy and neurologic involvement. Persistent activation of inflammatory cells like macrophages, natural killer cells and cytotoxic lymphocytes can lead to a cytokine storm and multi organ damage. MAS is usually triggered by rheumatologic diseases and rarely in the presentation of a new connective disease like systemic lupus erythematosus (SLE). In addition to MAS, the auto-immune conditions of SLE can be associated with different overlapping syndromes notably dermatomyositis. Case description We present a 31-year-old male from a Latin-American background without pre-existent conditions who presented complains of sore throat, joint pain, fever and fatigue. He quickly developed a pancytopenia with increased liver and pancreatic enzymes. In the process of the investigation, he was treated with antibiotics and admitted intubated to the intensive care unit for a severe pneumonia. Result and conclusion We proceeded with a bone marrow biopsy which detected an active MAS. Regarding his muscle weakness, we also revealed an inflammatory myositis on a quadricep muscle biopsy. Further discovery of positive auto-antibodies (ANA and anti-DNA) showed the presence of a LED. We successfully treated his different auto-immune complications with high doses of prednisone, and intravenously immunoglobulins. After 2 months of his admission and 12 days passed in the intensive care unit, the patient returned home with minimal sequelae with a long term immunosuppressive treatment of prednisone, mycophenolate mofetil and hydroxychloroquine. Take-home message The early identification of the cause of MAS is crucial for the accurate management of this disease and preventing further multi organ complications. SLE has remains a complex condition that can present its first manifestations in a broad spectrum of auto-immune diseases.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.003 | 0.002 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.005 | 0.003 |
| Science and technology studies | 0.003 | 0.002 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.006 | 0.003 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".