Felty’s syndrome
Felty’s syndrome was first described in 1924 by the US-American physician Augustus Roi Felty as a triad of rheumatoid arthritis, splenomegaly and leucopenia. Even nearly 100 years later, this rare syndrome is still paralleled by diagnostic and therapeutic challenges and its pathogenesis is incomplet...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-10-01
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| Series: | Frontiers in Medicine |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2023.1238405/full |
| _version_ | 1797658608895262720 |
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| author | Christoph Wegscheider Christoph Wegscheider Vera Ferincz Vera Ferincz Karin Schöls Karin Schöls Andreas Maieron Andreas Maieron |
| author_facet | Christoph Wegscheider Christoph Wegscheider Vera Ferincz Vera Ferincz Karin Schöls Karin Schöls Andreas Maieron Andreas Maieron |
| author_sort | Christoph Wegscheider |
| collection | DOAJ |
| description | Felty’s syndrome was first described in 1924 by the US-American physician Augustus Roi Felty as a triad of rheumatoid arthritis, splenomegaly and leucopenia. Even nearly 100 years later, this rare syndrome is still paralleled by diagnostic and therapeutic challenges and its pathogenesis is incompletely understood. Neutropenia with potentially life-threatening infections is the main problem and several pathomechanisms like Fas-mediated apoptosis, anti-neutrophil antibodies, anti-G-CSF antibodies, neutrophil consumption in the context of NETosis and suppression of granulopoiesis by T-LGLs have been suggested. Felty’s syndrome has various differential diagnoses as splenomegaly and cytopenia are common features of different infectious diseases, malignancies and autoimmune disorders. Additionally, benign clonal T-/NK-LGL lymphocytosis is increasingly noticed in Felty’s syndrome, which further complicates diagnosis. Today’s treatment options are still sparse and are largely based on case reports and small case series. Methotrexate is the mainstay of therapy, followed by rituximab, but there is less evidence for alternatives in the case of adverse reactions or failure of these drugs. This article gives an updated review about Felty’s syndrome including its pathogenesis and treatment options. |
| first_indexed | 2024-03-11T18:01:37Z |
| format | Article |
| id | doaj.art-e8cf8b192e9b428a8e7f42be45711d42 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-03-11T18:01:37Z |
| publishDate | 2023-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-e8cf8b192e9b428a8e7f42be45711d422023-10-17T08:51:09ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2023-10-011010.3389/fmed.2023.12384051238405Felty’s syndromeChristoph Wegscheider0Christoph Wegscheider1Vera Ferincz2Vera Ferincz3Karin Schöls4Karin Schöls5Andreas Maieron6Andreas Maieron7Karl Landsteiner University of Health Sciences, Krems, AustriaDivision of Internal Medicine, University Hospital St. Pölten, St. Pölten, AustriaKarl Landsteiner University of Health Sciences, Krems, AustriaDivision of Internal Medicine, University Hospital St. Pölten, St. Pölten, AustriaKarl Landsteiner University of Health Sciences, Krems, AustriaDivision of Internal Medicine, University Hospital St. Pölten, St. Pölten, AustriaKarl Landsteiner University of Health Sciences, Krems, AustriaDivision of Internal Medicine, University Hospital St. Pölten, St. Pölten, AustriaFelty’s syndrome was first described in 1924 by the US-American physician Augustus Roi Felty as a triad of rheumatoid arthritis, splenomegaly and leucopenia. Even nearly 100 years later, this rare syndrome is still paralleled by diagnostic and therapeutic challenges and its pathogenesis is incompletely understood. Neutropenia with potentially life-threatening infections is the main problem and several pathomechanisms like Fas-mediated apoptosis, anti-neutrophil antibodies, anti-G-CSF antibodies, neutrophil consumption in the context of NETosis and suppression of granulopoiesis by T-LGLs have been suggested. Felty’s syndrome has various differential diagnoses as splenomegaly and cytopenia are common features of different infectious diseases, malignancies and autoimmune disorders. Additionally, benign clonal T-/NK-LGL lymphocytosis is increasingly noticed in Felty’s syndrome, which further complicates diagnosis. Today’s treatment options are still sparse and are largely based on case reports and small case series. Methotrexate is the mainstay of therapy, followed by rituximab, but there is less evidence for alternatives in the case of adverse reactions or failure of these drugs. This article gives an updated review about Felty’s syndrome including its pathogenesis and treatment options.https://www.frontiersin.org/articles/10.3389/fmed.2023.1238405/fullFelty syndromerheumatoid arthritisT-LGLrituximabJAK inhibitor |
| spellingShingle | Christoph Wegscheider Christoph Wegscheider Vera Ferincz Vera Ferincz Karin Schöls Karin Schöls Andreas Maieron Andreas Maieron Felty’s syndrome Frontiers in Medicine Felty syndrome rheumatoid arthritis T-LGL rituximab JAK inhibitor |
| title | Felty’s syndrome |
| title_full | Felty’s syndrome |
| title_fullStr | Felty’s syndrome |
| title_full_unstemmed | Felty’s syndrome |
| title_short | Felty’s syndrome |
| title_sort | felty s syndrome |
| topic | Felty syndrome rheumatoid arthritis T-LGL rituximab JAK inhibitor |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2023.1238405/full |
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