Scleroderma Renal Crisis-Associated Thrombotic Microangiopathy Preceding Skin Sclerosis in Diffuse Cutaneous Systemic Sclerosis: A Case Report
Abstract
A prompt and accurate diagnosis is essential because the management of thrombotic microangiopathy (TMA) depends on its underlying etiology. We report a case of diffuse cutaneous systemic sclerosis (dcSSc) in which scleroderma renal crisis-associated TMA preceded the development of overt skin sclerosis. A 53-year-old woman presented with TMA and severe renal dysfunction. She tested positive for anti-RNA polymerase III antibodies and subsequently developed skin sclerosis, leading to a diagnosis of dcSSc. Angiotensin-converting enzyme inhibitor therapy resulted in a clinical improvement in the patient. Therefore, systemic sclerosis should be considered in the differential diagnosis of TMA, even in the absence of skin manifestations at presentation.
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BibTeX
@article{kobayashi2026scleroderma,
title = {{Scleroderma Renal Crisis-Associated Thrombotic Microangiopathy Preceding Skin Sclerosis in Diffuse Cutaneous Systemic Sclerosis: A Case Report}},
author = {Tomoki Kobayashi and Ken Goda and Kodai Yamamoto and Yohei Kanzawa and Mitsumasa Okano and Keiko Hatazawa and Kazunori Otsui and Kazuhiko Sakaguchi},
journal = {Internal Medicine},
year = {2026},
month = sep,
doi = {10.2169/internalmedicine.8085-26},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_8085-26/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Scleroderma Renal Crisis-Associated Thrombotic Microangiopathy Preceding Skin Sclerosis in Diffuse Cutaneous Systemic Sclerosis: A Case Report
AU - Tomoki Kobayashi
AU - Ken Goda
AU - Kodai Yamamoto
AU - Yohei Kanzawa
AU - Mitsumasa Okano
AU - Keiko Hatazawa
AU - Kazunori Otsui
AU - Kazuhiko Sakaguchi
PY - 2026
DA - 2026/09/29
JO - Internal Medicine
DO - 10.2169/internalmedicine.8085-26
UR - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_8085-26/_article
AB - A prompt and accurate diagnosis is essential because the management of thrombotic microangiopathy (TMA) depends on its underlying etiology. We report a case of diffuse cutaneous systemic sclerosis (dcSSc) in which scleroderma renal crisis-associated TMA preceded the development of overt skin sclerosis. A 53-year-old woman presented with TMA and severe renal dysfunction. She tested positive for anti-RNA polymerase III antibodies and subsequently developed skin sclerosis, leading to a diagnosis of dcSSc. Angiotensin-converting enzyme inhibitor therapy resulted in a clinical improvement in the patient. Therefore, systemic sclerosis should be considered in the differential diagnosis of TMA, even in the absence of skin manifestations at presentation.
SN - 1349-7235
SN - 0918-2918
ER -