ReviewLeukemia2020
Erdheim-Chester disease: a rapidly evolving disease model.
Review in Leukemia, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
What it found
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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
38 citing papers in PubMed, 70 citations in OpenAlex.
- Article
- Targeted Therapy in Erdheim-Chester Disease: A Case Report and Review of the Literature.Cureus · 2026Article
- Kidney Transplantation in Patients With Erdheim-Chester Disease.Kidney international reports · 2026Article
- Case Report: Ureteral obstruction reveals rare underlying fibro-inflammatory, infiltrative, and proliferative disorders: report of four cases.Frontiers in immunology · 2026Article
- Perioperative multidisciplinary rescue of a patient with Erdheim - Chester disease and multi-system involvement: a case report.Frontiers in medicine · 2026Article
- Unraveling the molecular landscape of Erdheim-Chester disease: new insights from methylome and transcriptome integration.Leukemia · 2025Article
- Article
- Juvenile xanthogranuloma as a potential early manifestation of neurofibromatosis type 1: A case report.Medicine · 2025Article
- Article
- Reactive Histiocytic Proliferation in Rheumatoid Arthritis Mimicking Erdheim-Chester Disease.Cureus · 2025Article
- Recent advances in therapeutic strategies of Erdheim-Chester disease.Naunyn-Schmiedeberg's archives of pharmacology · 2025Review
- MEK inhibitors are effective in Erdheim-Chester disease regardless of MAPK pathway mutations.Leukemia · 2025Article
- Treatment holidays in patients with Erdheim-Chester disease receiving vemurafenib: a prospective pilot study.Blood advances · 2025Article
- IgG4-related disease and other fibro-inflammatory conditions.Nature reviews. Rheumatology · 2025Review
- A patient with Eradheim-Chester disease presenting with progressive cystic lung lesions and confirmed pulmonary artery hypertension: a case report.BMC pulmonary medicine · 2025Article
- Risk of Second Cancer in Erdheim-Chester Disease.JAMA oncology · 2025Article
- Predominantly multiple focal non-cystic renal lesions: an imaging approach.Abdominal radiology (New York) · 2025Review
- Erdheim-Chester Disease in Adults: 11 Cases from a Canadian Rare Diseases Program.Clinical hematology international · 2025Article
- A Rare Case of Isolated Central Nervous System Neoplasm With Histiocytic Features.Brain tumor research and treatment · 2025Article
- A rare case of Erdheim Chester disease.Radiology case reports · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a putative neoplastic and inflammatory nature. The disease is driven by mutations in proto-oncogenes such as BRAF and MEK, while immune-mediated mechanisms contribute to disease development and progression. The clinical presentation of ECD is highly heterogeneous, ranging from smouldering unifocal forms to multiorgan life-threatening disease. Almost any organ can be involved, but the most common lesions include long-bone involvement, retroperitoneal fibrosis, interstitial lung disease, pericardial and myocardial infiltration, CNS, retro-orbital, and large-vessel involvement. These manifestations may mimic those of neoplastic and systemic immune-mediated diseases. Overlap with these conditions represents an emerging challenge for the clinician. A variety of treatments are efficacious for ECD, targeting both the MAPK-pathway and the immune-mediated pathomechanisms. The traditional approach is based on immunomodulatory agents (interferon-α), but recent alternatives-including anti-cytokine therapies (IL1- and TNFα-blockers) and immunosuppressants (mTOR-inhibitors)-showed promising results. However, since the detection of MAPK pathway activation in most patients and the dramatic efficacy of BRAF and MEK inhibitors, these targeted treatments represent the first-line approach in patients with severe disease forms. High rates of radiologic responses do not often mean clinical remission, especially for CNS involvement, which often results in chronic disability. This review will outline the main clinical features of ECD, with emphasis on the emerging challenges in pathogenesis and management, and on the role of recent targeted approaches.
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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.