Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review
TAFRO syndrome is a systemic inflammatory disorder of unknown etiology, and its diagnosis requires the exclusion of autoimmune diseases. A 42-year-old Japanese woman presented with TAFRO syndrome-like manifestations, but had undiagnosed limited-cutaneous systemic sclerosis preventing a definitive di...
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2025-03-01
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| Series: | Immunological Medicine |
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| Online Access: | https://www.tandfonline.com/doi/10.1080/25785826.2025.2481675 |
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| author | Hiroyuki Kawahara Satoshi Hara Noriko Iwaki Hiroko Ikeda Dai Inoue Ichiro Mizushima Hideki Nomura Yasufumi Masaki Yasunori Iwata Mitsuhiro Kawano |
| author_facet | Hiroyuki Kawahara Satoshi Hara Noriko Iwaki Hiroko Ikeda Dai Inoue Ichiro Mizushima Hideki Nomura Yasufumi Masaki Yasunori Iwata Mitsuhiro Kawano |
| author_sort | Hiroyuki Kawahara |
| collection | DOAJ |
| description | TAFRO syndrome is a systemic inflammatory disorder of unknown etiology, and its diagnosis requires the exclusion of autoimmune diseases. A 42-year-old Japanese woman presented with TAFRO syndrome-like manifestations, but had undiagnosed limited-cutaneous systemic sclerosis preventing a definitive diagnosis of TAFRO syndrome. However, her clinical course and pathological findings, including renal glomerular microangiopathy, were consistent with TAFRO syndrome. We performed a systematic review of the literature to evaluate how autoimmunity affects the clinical characteristics of TAFRO syndrome/idiopathic multicentric Castleman disease (iMCD)-TAFRO. We reviewed 95 reported cases of TAFRO syndrome/iMCD-TAFRO and found that at least 41 (43.6%) had various autoantibodies. In particular, the positive rates of anti-nuclear antibody, anti-SS-A antibody, anti-SS-B antibody, PA-IgG, and direct Coombs test were high. Furthermore, we identified 14 cases of autoimmune diseases with TAFRO syndrome-like manifestations. We compared the clinical characteristics of these 14 with those of the autoantibody-positive and -negative cases among the 95 cases of TAFRO syndrome/iMCD-TAFRO. Apart from sex ratio, we found no significant difference in clinical presentation, treatment, or outcome among the groups. In conclusion, TAFRO syndrome/iMCD-TAFRO often accompanies autoantibodies and shares many clinical characteristics with other autoimmune diseases. Clinicians should be aware that some autoimmune diseases mimic TAFRO syndrome/iMCD-TAFRO. |
| format | Article |
| id | doaj-art-017cecd3f9a0474291ef2647fd969bab |
| institution | DOAJ |
| issn | 2578-5826 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Immunological Medicine |
| spelling | doaj-art-017cecd3f9a0474291ef2647fd969bab2025-08-20T02:51:00ZengTaylor & Francis GroupImmunological Medicine2578-58262025-03-0111010.1080/25785826.2025.2481675Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature reviewHiroyuki Kawahara0Satoshi Hara1Noriko Iwaki2Hiroko Ikeda3Dai Inoue4Ichiro Mizushima5Hideki Nomura6Yasufumi Masaki7Yasunori Iwata8Mitsuhiro Kawano9Department of Nephrology and Rheumatology, Kanazawa University Hospital, Kanazawa, JapanDepartment of Nephrology and Rheumatology, Kanazawa University Hospital, Kanazawa, JapanDepartment of Hematology, National Cancer Center Hospital, Tokyo, JapanDepartment of Pathology, Kanazawa University Hospital, Kanazawa, JapanDepartment of Radiology, Kanazawa University Hospital, Kanazawa, JapanDepartment of Nephrology and Rheumatology, Kanazawa University Hospital, Kanazawa, JapanDepartment of General Medicine, Kanazawa University Hospital, Kanazawa, JapanDepartment of Hematology and Immunology, Kanazawa Medical University, Uchinada, Kahoku-gun, JapanDepartment of Nephrology and Rheumatology, Kanazawa University Hospital, Kanazawa, JapanDepartment of Hematology and Immunology, Kanazawa Medical University, Uchinada, Kahoku-gun, JapanTAFRO syndrome is a systemic inflammatory disorder of unknown etiology, and its diagnosis requires the exclusion of autoimmune diseases. A 42-year-old Japanese woman presented with TAFRO syndrome-like manifestations, but had undiagnosed limited-cutaneous systemic sclerosis preventing a definitive diagnosis of TAFRO syndrome. However, her clinical course and pathological findings, including renal glomerular microangiopathy, were consistent with TAFRO syndrome. We performed a systematic review of the literature to evaluate how autoimmunity affects the clinical characteristics of TAFRO syndrome/idiopathic multicentric Castleman disease (iMCD)-TAFRO. We reviewed 95 reported cases of TAFRO syndrome/iMCD-TAFRO and found that at least 41 (43.6%) had various autoantibodies. In particular, the positive rates of anti-nuclear antibody, anti-SS-A antibody, anti-SS-B antibody, PA-IgG, and direct Coombs test were high. Furthermore, we identified 14 cases of autoimmune diseases with TAFRO syndrome-like manifestations. We compared the clinical characteristics of these 14 with those of the autoantibody-positive and -negative cases among the 95 cases of TAFRO syndrome/iMCD-TAFRO. Apart from sex ratio, we found no significant difference in clinical presentation, treatment, or outcome among the groups. In conclusion, TAFRO syndrome/iMCD-TAFRO often accompanies autoantibodies and shares many clinical characteristics with other autoimmune diseases. Clinicians should be aware that some autoimmune diseases mimic TAFRO syndrome/iMCD-TAFRO.https://www.tandfonline.com/doi/10.1080/25785826.2025.2481675TAFRO syndromeiMCD-TAFROsystemic sclerosisautoantibodyautoimmunity |
| spellingShingle | Hiroyuki Kawahara Satoshi Hara Noriko Iwaki Hiroko Ikeda Dai Inoue Ichiro Mizushima Hideki Nomura Yasufumi Masaki Yasunori Iwata Mitsuhiro Kawano Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review Immunological Medicine TAFRO syndrome iMCD-TAFRO systemic sclerosis autoantibody autoimmunity |
| title | Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review |
| title_full | Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review |
| title_fullStr | Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review |
| title_full_unstemmed | Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review |
| title_short | Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review |
| title_sort | systemic sclerosis presenting tafro syndrome like manifestations including renal glomerular microangiopathy a case report and literature review |
| topic | TAFRO syndrome iMCD-TAFRO systemic sclerosis autoantibody autoimmunity |
| url | https://www.tandfonline.com/doi/10.1080/25785826.2025.2481675 |
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