ReviewClinical rheumatology2015
Macrophage activation syndrome in the course of monogenic autoinflammatory disorders.
Review in Clinical rheumatology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers, 2 of them syntheses that pooled it.
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
44 citing papers in PubMed, 2 syntheses or guidelines pooled it, 73 citations in OpenAlex.
- Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.Frontiers in immunology · 2024Pooled it
- Anakinra treatment in macrophage activation syndrome: a single center experience and systemic review of literature.Clinical rheumatology · 2018Pooled it
- An animal model of NLRC4-associated autoinflammation and infantile enterocolitis reveals novel therapeutic strategies.Cellular & molecular immunology · 2025Article
- Lack of HLH in FMF.Pediatric rheumatology online journal · 2025Article
- Patients with Adult-Onset Still's Disease in Germany: A Retrospective Analysis of Clinical Characteristics and Treatment Practices Ahead of the Release of the German Recommendations.Journal of clinical medicine · 2025Article
- Emergencies in inflammatory rheumatic diseases.Rheumatology international · 2024Review
- New discoveries in the genetics and genomics of systemic juvenile idiopathic arthritis.Expert review of clinical immunology · 2024Review
- Macrophage Activation Syndrome in the Setting of Rheumatic Diseases.Advances in experimental medicine and biology · 2024Review
- Genetics of Acquired Cytokine Storm Syndromes : Secondary HLH Genetics.Advances in experimental medicine and biology · 2024Review
- The History of Macrophage Activation Syndrome in Autoimmune Diseases.Advances in experimental medicine and biology · 2024Review
- The tumor microenvironment shows a hierarchy of cell-cell interactions dominated by fibroblasts.Nature communications · 2023Article
- Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department's Analysis of 14 Cases.Clinical medicine insights. Case reports · 2023Review
- Review
- The everchanging framework of autoinflammation.Internal and emergency medicine · 2021Review
- Lenalidomide-Associated Hemophagocytic Lymphohistiocytosis With Plasma Cell Phagocytosis.Cureus · 2021Article
- Overview of the rarest causes of fever in newborns: handy hints for the neonatologist.Journal of perinatology : official journal of the California Perinatal Association · 2021Review
- COVID-19 in Autoinflammatory Diseases with Immunosuppressive Treatment.Journal of clinical medicine · 2021Article
- Blocking IL-1 to prevent respiratory failure in COVID-19.Critical care (London, England) · 2020Review
- The genetics of macrophage activation syndrome.Genes and immunity · 2020Review
- Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management.Paediatric drugs · 2020Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
An overwhelming activation of cytotoxic T cells and well-differentiated macrophages leading to systemic overload of inflammatory mediators characterizes the so-called macrophage activation syndrome (MAS); this potentially life-threatening clinical entity may derive from several genetic defects involved in granule-mediated cytotoxicity but has been largely observed in patients with juvenile idiopathic arthritis, many rheumatologic diseases, infections, and malignancies. The occurrence of MAS in the natural history or as the revealing clue of monogenic autoinflammatory disorders (AIDs), rare conditions caused by disrupted innate immunity pathways with overblown release of proinflammatory cytokines, has been only reported in few isolated patients with cryopyrin-associated periodic syndrome, mevalonate kinase deficiency, familial Mediterranean fever, and tumor necrosis factor receptor-associated periodic syndrome since 2001. All these patients displayed various clinical, laboratory, and histopathologic features of MAS and have often required intensive care support. Only one patient has died due to MAS. Defective cytotoxic cell function was documented in a minority of patients. Corticosteroids were the first-line treatment, but anakinra was clinically effective in three refractory cases. Even if MAS and AIDs share multiple clinical features as well as heterogeneous pathogenetic scenes and a potential response to anti-interleukin-1 targeted therapies, MAS requires a prompt specific recognition in the course of AIDs due to its profound severity and high mortality rate.
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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.