Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation
BackgroundSystemic sclerosis (SSc) is a complex autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. Treatment with autologous hematopoietic cell transplantation (HCT) for progressive SSc has improved overall and event-free survival rates signi...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-11-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.925776/full |
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| author | D.A. Haverkort B.E. Kersten A. van Rhenen W.J.F.M. van der Velden M.C. Vonk |
| author_facet | D.A. Haverkort B.E. Kersten A. van Rhenen W.J.F.M. van der Velden M.C. Vonk |
| author_sort | D.A. Haverkort |
| collection | DOAJ |
| description | BackgroundSystemic sclerosis (SSc) is a complex autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. Treatment with autologous hematopoietic cell transplantation (HCT) for progressive SSc has improved overall and event-free survival rates significantly, but unfortunately disease progression after HCT is seen in a subset of patients. Data on the efficacy and safety of second HCT is scarce.CaseWe present a patient with diffuse cutaneous SSc and associated interstitial lung disease (ILD) who successfully underwent a second HCT for progressive disease five years after a first HCT. We describe changes in skin involvement and pulmonary involvement as well as the changes observed in sequential nailfold microcapillaroscopy (NCM), performed from first presentation up to this moment.ConclusionThis case adds to the current limited literature on efficacy and safety of a second HCT in SSc refractory cases. Furthermore it outlines the potential of HCT on amelioration of microvasculopathy in SSc. |
| first_indexed | 2024-04-13T12:10:38Z |
| format | Article |
| id | doaj.art-64d427324d7f4343a4c3d9d2fc5cfa53 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-13T12:10:38Z |
| publishDate | 2022-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-64d427324d7f4343a4c3d9d2fc5cfa532022-12-22T02:47:30ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-11-011310.3389/fimmu.2022.925776925776Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculationD.A. Haverkort0B.E. Kersten1A. van Rhenen2W.J.F.M. van der Velden3M.C. Vonk4Department of Rheumatology, Radboud University Medical Center, Nijmegen, NetherlandsDepartment of Rheumatology, Radboud University Medical Center, Nijmegen, NetherlandsDepartment of Hematology, University Medical Center, Utrecht, NetherlandsDepartment of Hematology, Radboud University Medical Center, Nijmegen, NetherlandsDepartment of Rheumatology, Radboud University Medical Center, Nijmegen, NetherlandsBackgroundSystemic sclerosis (SSc) is a complex autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. Treatment with autologous hematopoietic cell transplantation (HCT) for progressive SSc has improved overall and event-free survival rates significantly, but unfortunately disease progression after HCT is seen in a subset of patients. Data on the efficacy and safety of second HCT is scarce.CaseWe present a patient with diffuse cutaneous SSc and associated interstitial lung disease (ILD) who successfully underwent a second HCT for progressive disease five years after a first HCT. We describe changes in skin involvement and pulmonary involvement as well as the changes observed in sequential nailfold microcapillaroscopy (NCM), performed from first presentation up to this moment.ConclusionThis case adds to the current limited literature on efficacy and safety of a second HCT in SSc refractory cases. Furthermore it outlines the potential of HCT on amelioration of microvasculopathy in SSc.https://www.frontiersin.org/articles/10.3389/fimmu.2022.925776/fullsystemic sclerosisstem cell transplant (SCT)nailfod capillaroscopyRodnan skin scoreinterstitial lung diseasemicrovascular abnormalities |
| spellingShingle | D.A. Haverkort B.E. Kersten A. van Rhenen W.J.F.M. van der Velden M.C. Vonk Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation Frontiers in Immunology systemic sclerosis stem cell transplant (SCT) nailfod capillaroscopy Rodnan skin score interstitial lung disease microvascular abnormalities |
| title | Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation |
| title_full | Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation |
| title_fullStr | Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation |
| title_full_unstemmed | Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation |
| title_short | Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation |
| title_sort | case report a successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis with positive effect on skin involvement pulmonary function and microcirculation |
| topic | systemic sclerosis stem cell transplant (SCT) nailfod capillaroscopy Rodnan skin score interstitial lung disease microvascular abnormalities |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.925776/full |
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