ArticleThe American journal of pathology1999
Involvement of soluble CD95 in Churg-Strauss syndrome.
Article in The American journal of pathology, 1999. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 66 citations in OpenAlex.
- EGPA at Diagnosis: A Comprehensive Single-Center Profile of Laboratory Findings and Clinical Evidence.International journal of molecular sciences · 2026Article
- Novel Targets for Drug Use in Eosinophilic Granulomatosis With Polyangiitis.Frontiers in medicine · 2021Review
- Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology.Frontiers in medicine · 2021Review
- Pulmonary vasculature and critical asthma syndromes: a comprehensive review.Clinical reviews in allergy & immunology · 2015Review
- Eosinophils in vasculitis: characteristics and roles in pathogenesis.Nature reviews. Rheumatology · 2014Review
- Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).Arthritis research & therapy · 2013Article
- Decreases in the numbers of peripheral blood regulatory T cells, and increases in the levels of memory and activated B cells, in patients with active eosinophilic granulomatosis and polyangiitis.Journal of clinical immunology · 2013Article
- Cutting edge issues in the Churg-Strauss syndrome.Clinical reviews in allergy & immunology · 2013Review
- [Update Churg-Strauss syndrome].Zeitschrift fur Rheumatologie · 2012Article
- Anti-neutrophil cytoplasmic antibody pathogenesis in small-vessel vasculitis: an update.The American journal of pathology · 2009Review
- [Hypereosinophilic syndrome and Churg-Strauss syndrome: is it clinically relevant to differentiate these syndromes?].Der Internist · 2008Review
- Churg-strauss syndrome in the pediatric age group.The World Allergy Organization journal · 2008Article
- Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides.Clinical and experimental immunology · 2005Review
- Dendritic cells overexpressing Fas-ligand induce pulmonary vasculitis in mice.Clinical and experimental immunology · 2004Article
- Perspective on future therapy of vasculitis.Current rheumatology reports · 2000Review
- Article
- Heterogeneity and individualized therapy for eosinophilic granulomatosis with polyangiitis.Therapeutic advances in respiratory diseaseReview
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Deficiency of CD95 (Apo-1/Fas)-mediated apoptosis has recently been found in some autoimmune lymphoproliferative disorders due to inherited mutations of the CD95 gene. In this study, impairment of CD95 ligand-mediated killing of lymphocytes and eosinophils in Churg-Strauss Syndrome (CSS), which was a result of variation of CD95 receptor isoform expression, is demonstrated. Compared to those from healthy individuals, peripheral blood lymphocytes from eight CSS patients exhibit a switch from the membrane-bound CD95 receptor expression to its soluble splice variant, which protects from CD95L-mediated apoptosis. In five out of seven CSS patients recurrent oligoclonal T cell expansions were found, all using a Vbeta-gene from the Vbeta21 family associated with similar CDR3 motifs, indicating the predominance of T cell clones of a similar specificity in the CSS patients. In two of them, the effect of immunosuppressive therapy was studied. In both cases aberrant overexpression of the soluble CD95 receptor isoform and deviations from normal TCR Vbeta-gene usage normalized in parallel with the clinical improvement. Furthermore, soluble CD95 was identified as a survival factor for eosinophils rescuing eosinophils from apoptosis in the absence of growth factors in vitro. Given the role of eosinophils as effector cells in CSS, these findings suggest that soluble CD95 may be mechanistically involved in the disease.
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