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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http://dx.doi.org/10.2169/internalmedicine.5665-20 | DOI Listing |
Neuromuscul Disord
September 2024
Department of Neurology, Mayo Clinic-Rochester, Rochester, MN, USA. Electronic address:
We describe two anti-3‑hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) antibody-positive patients with treatment-responsive ophthalmoparesis. Patient 1 was a 53-year-old male with progressive proximal limb weakness, dysphagia, ptosis, and diplopia over 6 weeks and creatine kinase (CK) of 3,512 units/L. Patient 2 was a 55-year-old female with progressive proximal weakness, dysarthria, ptosis, diplopia, and dyspnea over 2 weeks with CK of 31,998 units/L.
View Article and Find Full Text PDFInt J Rheum Dis
March 2023
Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan.
Front Med (Lausanne)
January 2022
Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
Objective: This study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients.
Method: We reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up.
Results: A total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.
J Dermatol
February 2022
Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan.
A 37-year-old Japanese man with a 3-year history of diffuse cutaneous systemic sclerosis was admitted to our hospital with high fever, arthralgia, myalgia, and muscle weakness. A physical examination revealed facial erythema, Gottron's sign, and mechanic's hands in addition to skin sclerosis. Laboratory data revealed significantly elevated levels of creatine kinase and decreased complement.
View Article and Find Full Text PDFMod Rheumatol Case Rep
January 2022
The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyusyu, Fukuoka, Japan.
Anti-PM/Scl antibodies are associated with the overlap syndrome of systemic sclerosis and dermatomyositis/polymyositis (SSc-DM/PM), and are found in 50% of SSc-DM/PM cases in Europe and the USA, whereas they are rare in Japan. We report a case of an 80-year-old Japanese female with SSc-amyopathic dermatomyositis overlap syndrome, who developed scleroderma renal crisis, a complication of SSc. She had positive antinuclear antibodies in a discrete-speckled and nucleolar pattern and anti-centromere antibodies and anti-PM/Scl antibodies were confirmed by enzyme-linked immunosorbent assay and immunoprecipitation, respectively.
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