ArticleOrphanet journal of rare diseases2019
Clinical and positron emission tomography responses to long-term high-dose interferon-α treatment among patients with Erdheim-Chester disease.
Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 24 citations in OpenAlex.
- An Overlap of Erdheim-Chester Disease and Rosai-Dorfman Disease With Isolated Bone Lesions Presenting a Diagnostic Challenge: A Case Report.Clinical case reports · 2026Article
- The xanthogranuloma family of histiocytic neoplasms in the molecular era: Erdheim-Chester disease and beyond.Hematology. American Society of Hematology. Education Program · 2025Review
- An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement.Internal medicine (Tokyo, Japan) · 2025Article
- Clinical Spectrum and Outcome of Kidney Involvement in Non-Langerhans Histiocytosis.Kidney international reports · 2025Article
- The clinical spectrum and prognostic factors of Erdheim-Chester disease and mixed Langerhans cell histiocytosis and Erdheim-Chester disease.Annals of hematology · 2023Article
- [Mutation pedigree and treatment selection of Erdheim-Chester disease].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2023Article
- Erdheim-Chester disease: A multisystem disease case illustration with rare manifestations and treatment challenges.Clinical case reports · 2023Article
- Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature.BMC gastroenterology · 2022Review
- Intermediate-dose cytarabine is an effective therapy for adults with non-Langerhans cell histiocytosis.Orphanet journal of rare diseases · 2022Article
- Article
- [Efficacy and safety of vemurafenib in the treatment of BRAF(V600E)-mutated Erdheim-Chester disease].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2021Article
- [Erdheim-Chester Disease presenting with exophthalmos, impaired vision, and retroperitoneal fibrosis: a case report and literature review].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2021Review
- Bone mineral density and bone microarchitecture in a cohort of patients with Erdheim-Chester Disease.Orphanet journal of rare diseases · 2020Article
- Clinical presentation, imaging and response to interferon-alpha therapy in Erdheim-Chester disease: case-based review.Rheumatology international · 2020Review
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundErdheim-Chester disease (ECD) is a rare multi-systemic form of histiocytosis. Treatment with BRAF inhibitors has markedly improved outcomes of ECD; however, this targeted therapy is expensive (estimated annual cost is $50,000). Since estimated annual cost of interferon-α (IFN-α) is only approximately $1600 in China, we retrospectively evaluated the long-term therapeutic efficacy of IFN-α and the value of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) as an assessment method among 32 ECD patients who received high dose IFN-α therapy at Peking Union Medical College Hospital.
resultsThe median age at diagnosis was 48 years (range, 6-66 years). The median duration of treatment was 18.5 months (range, 1-51 months). The overall clinical response rates were 80.0%, including 33.3% complete response, 36.7% partial response and 10.0% stable disease. Thirty-one patients underwent a total of 81 scans by FDG-PET. Seventeen patients had serial FDG-PET results, nine patients had experienced a partial metabolic response at the last follow-up. The median reduction of ratios between the most active target lesion standardized uptake value (SUV) and liver SUV from baseline to last FDG-PET scan was 61.4% (range, 8.8-86.6%). Eight of thirteen patients who experienced continuous clinical improvement during follow-up had at least one target lesion SUV increased by FDG-PET which decreased in subsequent scans without changing treatment strategy. The estimated 3-year progression-free survival (PFS) and overall survival (OS) were 64.1 and 84.5%, respectively. Central nervous system (CNS) involvement was the only predictor for poor PFS and OS.
conclusionsHigh-dose IFN-α treatment is a cost-effective option, especially for patients without CNS involvement. Single target lesion SUV elevation according to FDG-PET do not accurately demonstrate disease progression, but serial FDG-PET imaging effectively discriminate treatment response.
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