Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody
Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to...
Saved in:
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2020-01-01
|
| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2020/1926908 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832554334058971136 |
|---|---|
| author | Yusho Ishii Hiroshi Fujii Koichiro Sugimura Tsuyoshi Shirai Yosuke Hoshi Yoko Fujita Yuko Shirota Tomonori Ishii Hiroaki Shimokawa Hideo Harigae |
| author_facet | Yusho Ishii Hiroshi Fujii Koichiro Sugimura Tsuyoshi Shirai Yosuke Hoshi Yoko Fujita Yuko Shirota Tomonori Ishii Hiroaki Shimokawa Hideo Harigae |
| author_sort | Yusho Ishii |
| collection | DOAJ |
| description | Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to be highly associated with SSc-associated pulmonary arterial hypertension (SSc-PAH). In general, several vasodilators are used as therapeutic drugs for SSc-PAH, whereas immunosuppressive therapies are not. Here, we report the case of a 62-year-old female with anticentriole antibody-positive SSc-PAH treated with immunosuppressants and vasodilators. She presented with two-year exertional dyspnea and was diagnosed with PAH and SSc owing to the centriole staining pattern and other symptoms without digital sclerosis. Oral vasodilators were initially administered but were not sufficiently effective on dyspnea. Immunosuppressants such as prednisolone and cyclophosphamide were started. Both of them improved mean pulmonary arterial pressure and 6-minute walk distance, and the anticentriole antibody also disappeared. In this case, SSc-PAH with anticentriole antibody was properly diagnosed and immunosuppressants and vasodilators improved the hemodynamics of PAH with anticentriole antibody and stably maintained it and, in addition, reduced the titer of anticentriole antibody. This indicates that anticentriole antibody might represent a good responsive group to therapies among subgroups of patients with SSc-PAH. |
| format | Article |
| id | doaj-art-02fbc66984bb44ad864fdbc931bbfc1f |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-02fbc66984bb44ad864fdbc931bbfc1f2025-02-03T05:51:46ZengWileyCase Reports in Rheumatology2090-68892090-68972020-01-01202010.1155/2020/19269081926908Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole AntibodyYusho Ishii0Hiroshi Fujii1Koichiro Sugimura2Tsuyoshi Shirai3Yosuke Hoshi4Yoko Fujita5Yuko Shirota6Tomonori Ishii7Hiroaki Shimokawa8Hideo Harigae9Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanClinical Research, Innovation and Education Center, Tohoku University Hospital, Sendai, Miyagi, JapanDepartment of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanDepartment of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, JapanSystemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to be highly associated with SSc-associated pulmonary arterial hypertension (SSc-PAH). In general, several vasodilators are used as therapeutic drugs for SSc-PAH, whereas immunosuppressive therapies are not. Here, we report the case of a 62-year-old female with anticentriole antibody-positive SSc-PAH treated with immunosuppressants and vasodilators. She presented with two-year exertional dyspnea and was diagnosed with PAH and SSc owing to the centriole staining pattern and other symptoms without digital sclerosis. Oral vasodilators were initially administered but were not sufficiently effective on dyspnea. Immunosuppressants such as prednisolone and cyclophosphamide were started. Both of them improved mean pulmonary arterial pressure and 6-minute walk distance, and the anticentriole antibody also disappeared. In this case, SSc-PAH with anticentriole antibody was properly diagnosed and immunosuppressants and vasodilators improved the hemodynamics of PAH with anticentriole antibody and stably maintained it and, in addition, reduced the titer of anticentriole antibody. This indicates that anticentriole antibody might represent a good responsive group to therapies among subgroups of patients with SSc-PAH.http://dx.doi.org/10.1155/2020/1926908 |
| spellingShingle | Yusho Ishii Hiroshi Fujii Koichiro Sugimura Tsuyoshi Shirai Yosuke Hoshi Yoko Fujita Yuko Shirota Tomonori Ishii Hiroaki Shimokawa Hideo Harigae Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody Case Reports in Rheumatology |
| title | Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody |
| title_full | Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody |
| title_fullStr | Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody |
| title_full_unstemmed | Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody |
| title_short | Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody |
| title_sort | successful treatment of pulmonary arterial hypertension in systemic sclerosis with anticentriole antibody |
| url | http://dx.doi.org/10.1155/2020/1926908 |
| work_keys_str_mv | AT yushoishii successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT hiroshifujii successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT koichirosugimura successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT tsuyoshishirai successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT yosukehoshi successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT yokofujita successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT yukoshirota successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT tomonoriishii successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT hiroakishimokawa successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody AT hideoharigae successfultreatmentofpulmonaryarterialhypertensioninsystemicsclerosiswithanticentrioleantibody |