Myositis Patients with Anti-U1-RNP and Anti-Ro52 Autoantibodies Are at Risk of Pericarditis, Glomerulonephritis, and Pulmonary Hypertension

Maria Casal-Dominguez¹, Iago Pinal-Fernandez², Andrea Corse³, Julie J. Paik², Jemima Albayda², Livia Casciola-Rosen⁴, Cheilonda Johnson⁵, Sonye K. Danoff⁶, Lisa Christopher-Stine⁶, Eleni Tiniakou⁴ and Andrew Mammen⁷, ¹Muscle Diseases Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases. National Institutes of Health, Bethesda, MD, ²Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ³Neurology, Johns Hopkins University, Baltimore, MD, ⁴Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁵Medicine/Pulmonology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁶Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, ⁷National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Autoantibodies and inflammatory myositis

Session Information

Date: Sunday, October 21, 2018

Title: Muscle Biology, Myositis and Myopathies Poster I: Clinical Features and Disease Course

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

To define the clinical phenotype of myositis patients with anti-U1- ribonucleoprotein (RNP) autoantibodies.

Methods:

The clinical features of 20 anti-U1RNP-positive patients were assessed and compared to those of 132 antisynthetase syndrome (ASyS), 178 dermatomyositis (DM), and 135 immune-mediated necrotizing myopathy (IMNM) patients.

Results:

Twenty (4.6%) of 437 patients tested were anti-U1-RNP-positive; these were younger (~37 years) and more likely to be black (60%) than ASyS, DM, or IMNM patients. Muscle weakness, arthritis, interstitial lung disease (ILD), and DM-specific skin features eventually developed in 80%, 60%, 45%, and 60% of anti-U1-RNP-positive patients, respectively. Four of 7 (57%) anti-U1-RNP-positive patients had a necrotizing muscle biopsy. ILD was more severe in anti-U1-RNP-positive patients than in DM or IMNM. Glomerulonephritis and pericarditis occurred in 25% and 40% of anti-U1-RNP-positive patients, respectively, but rarely in the other groups. Pulmonary hypertension (PH) developed in 25% of anti-U1-RNP-positive, 20% of ASyS, and in <3% of DM and IMNM patients. Interestingly, glomerulonephritis, pericarditis and PH only occurred in anti-U1- RNP-positive patients with co-existing anti-Ro52 autoantibodies.

Conclusion:

Muscle weakness and arthritis are the most common clinical features in anti- U1-RNP-positive patients; when present, ILD is severe. Pericarditis, glomerulonephritis and PH are relatively common in anti-U1-RNP-positive patients, and only developed in those anti-U1-RNP-positive patients with co-existing anti-Ro52 autoantibodies.

Disclosure: M. Casal-Dominguez, None; I. Pinal-Fernandez, None; A. Corse, None; J. J. Paik, None; J. Albayda, None; L. Casciola-Rosen, None; C. Johnson, None; S. K. Danoff, None; L. Christopher-Stine, None; E. Tiniakou, None; A. Mammen, None.

To cite this abstract in AMA style:

Casal-Dominguez M, Pinal-Fernandez I, Corse A, Paik JJ, Albayda J, Casciola-Rosen L, Johnson C, Danoff SK, Christopher-Stine L, Tiniakou E, Mammen A. Myositis Patients with Anti-U1-RNP and Anti-Ro52 Autoantibodies Are at Risk of Pericarditis, Glomerulonephritis, and Pulmonary Hypertension [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/myositis-patients-with-anti-u1-rnp-and-anti-ro52-autoantibodies-are-at-risk-of-pericarditis-glomerulonephritis-and-pulmonary-hypertension/. Accessed .

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