ArticleMedicine2025
Autoimmune-associated hemophagocytic syndrome complicated by anti-MDA5 antibody-positive dermatomyositis successfully treated with immunosuppressive therapy: A case report.
Article in Medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
0 citing papers in PubMed.
No citing paper in PubMed yet.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
rationaleAnti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is frequently complicated by rapidly progressive interstitial lung disease and may present with life-threatening hemophagocytic syndrome, a critical but often under-recognized complication. Prompt diagnosis and intervention are essential to improve patient outcomes. PATIENT CONCERNS: A 38-year-old Japanese woman presented with fever, arthralgia, erythema, and elevated liver enzymes. Physical examination revealed chilblain-like erythema, Gottron and reverse Gottron signs, and tenderness in the thighs. DIAGNOSES: Blood tests revealed pancytopenia and elevated soluble interleukin-2 receptor and ferritin levels. Bone marrow aspiration showed macrophage activation and hemophagocytosis. Imaging revealed ground-glass opacities in both lower lung lobes and bilateral thigh myositis. The presence of anti-MDA5 antibodies led to the diagnosis of anti-MDA5-positive dermatomyositis complicated by autoimmune-related hemophagocytic syndrome. Liver biopsy suggested cytokine storm-mediated hepatocyte damage and macrophage infiltration.
interventionsThe patient received steroid pulse therapy, tacrolimus, and intravenous cyclophosphamide. OUTCOMES: Pancytopenia and liver dysfunction improved significantly following treatment. The patient was transitioned to outpatient care. After 4 years of follow-up, during which she received 6 courses of cyclophosphamide, steroids, and tacrolimus were successfully tapered off, and she remained in remission without recurrence. LESSONS: This case underscores the importance of early recognition of hemophagocytic syndrome in patients with anti-MDA5-positive dermatomyositis. Prompt bone marrow evaluation and aggressive immunosuppressive therapy may lead to favorable long-term outcomes. Further studies are warranted to establish standardized treatment strategies for autoimmune-related hemophagocytic syndrome.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.