1. Anti-survival motor neuron complex antibodies as a novel biomarker for pulmonary arterial hypertension and interstitial lung disease in mixed connective tissue disease.
- Author
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Todoroki, Yasuyuki, Satoh, Minoru, Kubo, Satoshi, Kosaka, Shunpei, Fukuyo, Shunsuke, Nakatsuka, Keisuke, Saito, Kazuyoshi, Tanaka, Shin, Nakayamada, Shingo, and Tanaka, Yoshiya
- Subjects
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RESEARCH funding , *MYOSITIS , *AUTOANTIBODIES , *MOTOR neurons , *SCIENTIFIC observation , *INTERSTITIAL lung diseases , *SYSTEMIC lupus erythematosus , *SYMPTOMS , *DESCRIPTIVE statistics , *CONNECTIVE tissue diseases , *SYSTEMIC scleroderma , *RESEARCH , *PULMONARY arterial hypertension , *BIOMARKERS , *PRECIPITIN tests , *DISEASE complications - Abstract
Objective The presence of anti-U1 RNP antibodies (Abs) is critical for diagnosing MCTD. The aim of this study is to evaluate the clinical relevance of anti-survival motor neuron (SMN) complex Abs, which often coexist with anti-U1 RNP Abs. Methods A total of 158 newly diagnosed consecutive cases of SLE, SSc or MCTD with anti-U1 RNP Abs were enrolled in this multicentre observational study between April 2014 and August 2022. Serum anti-SMN complex Abs were screened by immunoprecipitation of 35S-methionine-labelled cell extracts, and associations between anti-SMN complex Abs positivity and clinical characteristics were analysed. Results Anti-SMN complex Abs were detected in 36% of MCTD patients, which was significantly higher than that in SLE (8%) or SSc (12%). Among MCTD patients classified based on the combination of the clinical features of SLE, SSc and idiopathic inflammatory myopathies, anti-SMN complex Abs showed the highest prevalence in a subset with clinical features of all three components. Anti-SMN complex Abs–positive MCTD had a higher prevalence of pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), which are related to poor prognosis, than negative patients. Moreover, all three cases of death within 1 year of the treatment were positive for anti-SMN complex Abs. Conclusions Anti-SMN complex Abs is the first biomarker of a typical subset of MCTD which bears organ damages such as PAH and ILD. [ABSTRACT FROM AUTHOR]
- Published
- 2024
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