1. Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement
- Author
-
Chie Saito, Naoko Okiyama, Masahiro Yokosawa, Kei Nagai, Kunihiro Yamagata, Hirayasu Kai, Akiko Fujita, Yuya Kondo, Megumi Watanabe, Sae Inoue, Tatsuya Shimizu, Naoki Morito, Ryota Ishii, Tetsuya Kawamura, Joichi Usui, and Shuzo Kaneko
- Subjects
Adult ,Pathology ,medicine.medical_specialty ,Thrombotic microangiopathy ,Anti-nuclear antibody ,systemic sclerosis ,anti-PM/Scl antibody ,Scleroderma Renal Crisis ,Case Report ,Disease ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Internal Medicine ,medicine ,Humans ,Scleroderma, Systemic ,biology ,business.industry ,General Medicine ,medicine.disease ,scleroderma renal crisis ,thrombotic microangiopathy ,Antibodies, Antinuclear ,Etiology ,biology.protein ,Organ involvement ,Female ,030211 gastroenterology & hepatology ,Antibody ,Lung Diseases, Interstitial ,business ,Kidney disease - 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.
- Published
- 2021