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

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 and interstitial pneumonia. Despite a lack of overt skin sclerosis, nucleolar staining in our indirect immunofluorescence analysis nailfold capillaroscopy facilitated the diagnosis anti-PM/Scl antibody-positive systemic sclerosis. We observed persistent presence antibodies throughout clinical course, suggesting that her kidney disease scleroderma crisis. Anti-PM/Scl can be associated multiple organ diseases. Careful attention patient's antinuclear antibody pattern dermatological findings may help clarify etiology undiagnosed