Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement

Tatsuya Shimizu; Chie Saito; Megumi Watanabe; Ryota Ishii; Tetsuya Kawamura; Kei Nagai; Akiko Fujita; Shuzo Kaneko; Hirayasu Kai; Naoki Morito; Joichi Usui; Masahiro Yokosawa; Yuya Kondo; Sae Inoue; Naoko Okiyama; Kunihiro Yamagata

doi:10.2169/internalmedicine.5665-20

Tatsuya Shimizu, Chie Saito, Megumi Watanabe, Ryota Ishii, Tetsuya Kawamura, Kei Nagai, Akiko Fujita, Shuzo Kaneko, Hirayasu Kai, Naoki Morito, Joichi Usui, Masahiro Yokosawa, Yuya Kondo, Sae Inoue, Naoko Okiyama, Kunihiro Yamagata

Author information

Tatsuya Shimizu
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Chie Saito
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Megumi Watanabe
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Ryota Ishii
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Tetsuya Kawamura
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Kei Nagai
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Akiko Fujita
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Shuzo Kaneko
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Hirayasu Kai
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Naoki Morito
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Joichi Usui
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
Masahiro Yokosawa
Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Japan
Yuya Kondo
Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Japan
Sae Inoue
Department of Dermatology, Faculty of Medicine, University of Tsukuba, Japan
Naoko Okiyama
Department of Dermatology, Faculty of Medicine, University of Tsukuba, Japan
Kunihiro Yamagata
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan

Keywords: anti-PM/Scl antibody, systemic sclerosis, scleroderma renal crisis, thrombotic microangiopathy

JOURNALS OPEN ACCESS Advance online publication

Article ID: 5665-20

DOI https://doi.org/10.2169/internalmedicine.5665-20

Details

Abstract

A 40-year-old Japanese woman developed malignant-phase hypertension complicated by thrombotic microangiopathy, progressing to end-stage renal disease. Five years later, she was diagnosed with pulmonary arterial hypertension and interstitial pneumonia. Despite a lack of overt skin sclerosis, nucleolar staining in our indirect immunofluorescence analysis and nailfold capillaroscopy facilitated the diagnosis of anti-PM/Scl antibody-positive systemic sclerosis. We observed the persistent presence of anti-PM/Scl antibodies throughout the clinical course, suggesting that her kidney disease was scleroderma renal crisis. Anti-PM/Scl antibodies can be associated with multiple organ diseases. Careful attention to a patient's antinuclear antibody pattern and dermatological findings may help clarify the etiology of undiagnosed diseases.

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