• 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, and Kunihiro Yamagata.
• Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan.
• Intern. Med. 2021 Apr 1; 60 (7): 1101-1107.

AbstractA 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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