The diagnostic challenge of patients with anti-U1-RNP antibodies

Elhani, Inès; Khoy, Kathy; Mariotte, D.; Comby, E.; Marcelli, Christian; Audemard-Verger, Alexandra; Boutémy, Jonathan; Maigné, Gwénola; Silva, Nicolas Martin; Aouba, Achille; Boysson, Hubert de

doi:10.1007/s00296-022-05161-w

Cited by 9 publications

(7 citation statements)

References 50 publications

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“…Regarding diagnosis of MCTD, most physicians would agree that it should be considered in an anti-U1-RNP-positive patient presenting with Raynaud's phenomenon, 'puffy hands' and at least two of the following clinical features: arthritis, myositis, leucopenia, esophageal dysmotility, pleuritis, pericarditis, interstitial lung disease or pulmonary arterial hypertension. 9 Overall, diagnosing MCTD in clinical practice is an issue of pattern recognition and clinical decision.…”

Section: Discussionmentioning

confidence: 99%

Dysphagia as an Uncommon Presentation of Mixed Connective Tissue Disorder: A Case Report

Adhikari,

Kayastha,

Kayastha

et al. 2023

JCMC

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Mixed Connective Tissue Disorder is an autoimmune condition characterized by features of multiple connective tissue diseases. Dysphagia is a rare presentation of Mixed Connective Tissue Disorder and is not commonly reported. A unique and challenging case report to find out the cause of dysphagia as a Mixed Connective Tissue Disorder in a patient at a tertiary care Centre is presented. Early recognition, accurate diagnosis and appropriate management are crucial for optimal outcomes in such cases.

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Section: Discussionmentioning

confidence: 99%

Dysphagia as an Uncommon Presentation of Mixed Connective Tissue Disorder: A Case Report

Adhikari,

Kayastha,

Kayastha

et al. 2023

JCMC

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“…Anti-RNP (ribonuclear protein) antibodies are prevalent in a myriad of systemic diseases such as myositis, mixed connective tissue disease, systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis ( 96 ). A study aiming to characterize the pulmonary manifestations among patients with anti-RNP antibodies found that out of a total of 544 patients, ~25% had ILD with NSIP being the predominant radiological finding followed by UIP.…”

Section: Myositis Overlap: Anti Pm/scl Anti-ku Anti-rnpmentioning

confidence: 99%

Myositis interstitial lung disease and autoantibodies

Chaudhry

Christopher‐Stine

2023

Front. Med.

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The aim of this review is to examine and evaluate published literature associated with idiopathic inflammatory myopathies (IIM) and interstitial lung disease (ILD) based on myositis specific autoantibodies (MSA) and the potential clinical significance of each autoantibody subtype for the practicing clinician. The review is a comprehensive search of literature published in PubMed from the year 2005 and onward coinciding with the surge in the discovery of new MSAs. Additionally, we comment on recommended multidisciplinary longitudinal care practices for patients with IIM-ILD with regard to imaging and other testing. Treatment is not covered in this review.

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“…5 Anti-ribonucleoprotein (RNP) antibodies target proteins included in the small nuclear RNP (snRNP) complex; their presence was described to be systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or mixed connective tissue disease (MCTD). [6][7][8] Despite its relatively frequent prevalence in MCTD and SLE, IIM patients with anti-U1 ribonucleoprotein (U1RNP) antibodies have been described. It was previously shown that muscle weakness was present at the time of disease onset in all myositis patients with anti-U1RNP involving proximal limb muscles, and almost 90% of those patients had weakness in proximal muscles that was frequently severe.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy

Yang

Jiang

et al. 2023

Therapeutic Advances in Musculoskeletal

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Background: Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied. Objective: We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis. Design: This is a retrospective cohort study. Methods: We reviewed the clinical data of myositis patients with anti-U1RNP antibodies and compared them to those with anti-signal recognition particle (SRP) and hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-associated immune-mediated necrotizing myopathy (IMNM). Results: A total of 30 adult cases were identified; median age was 47.5 years and 24 (80%) were females, and 12 patients did not coexist with myositis-specific antibodies (MSAs) (isolated anti-U1RNP). The serum creatine kinase (CK) was significantly higher in patients with isolated anti-U1RNP (2182.5 U/L versus 289 U/L, p = 0.01), and the number of patients with CK > 2000 U/L was higher compared to that in anti-U1RNP antibody patients coexisting with MSAs (66.7% versus 16.7%, p = 0.009). The prevalence of IMNM in patients’ muscle pathology with isolated anti-U1RNP was significantly higher (75%, p = 0.003). Skin rashes were less common in isolated anti-U1RNP group ( p < 0.05). Of the 25 individuals with available pulmonary high-resolution CT (HRCT), 14 (56%) were diagnosed with interstitial lung disease (ILD). The incidence of muscular weakness, dysphagia, or levels of CK was not different between the isolated anti-U1RNP antibody individuals and the anti-HMGCR or SRP-IMNM groups ( p > 0.05). But the frequency of Raynaud’s phenomenon, arthritis, and membrane attack complex (MAC) deposits in myositis patients with isolated anti-U1RNP antibodies were higher than in anti-HMGCR and SRP-IMNM (all p < 0.005). There was no difference between anti-U1RNP patients with and without Ro-52 ( p > 0.05). Isolated anti-U1RNP individuals showed marked improvements in muscle strength, and the remission rate in 1 and 2 years was significantly higher than that in anti-HMGCR and SRP-IMNM ( p < 0.05). Conclusions: The clinical and pathological features of myositis patients with isolated anti-U1RNP antibodies were similar to IMNM. Arthritis and ILD are the most common extramuscular clinical features. Most respond well to treatment and have a good prognosis.

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The diagnostic challenge of patients with anti-U1-RNP antibodies

Cited by 9 publications

References 50 publications

Dysphagia as an Uncommon Presentation of Mixed Connective Tissue Disorder: A Case Report

Dysphagia as an Uncommon Presentation of Mixed Connective Tissue Disorder: A Case Report

Myositis interstitial lung disease and autoantibodies

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy

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