ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2131

Longitudinal Cohort Study of Anti-PM/Scl Myositis Patients: Mild Muscle and Lung Involvement with Prominent Perivascular Inflammation

Rebecca De Lorenzo1, Iago Pinal-Fernandez2, Maria Casal-Dominguez2, Wilson Huang2, Jose Cesar Milisenda1, Cassie Parks2, Katherine Pak3, Cheilonda Johnson4, Eleni Tiniakou5, Jemima Albayda6, Julie J. Paik6, Sonye K. Danoff7, Lisa Christopher-Stine7, Andrea Corse8 and Andrew Mammen3,9, 1Muscle Disease Unit, NIAMS, NIH, Bethesda, MD, 2NIAMS, NIH, Bethesda, MD, 3National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 4Medicine/Pulmonology, Johns Hopkins University School of Medicine, Baltimore, MD, 5Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 6Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 7Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, 8Neurology, Johns Hopkins University, Baltimore, MD, 9Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: , , ,

Session Information

Date: Tuesday, November 7, 2017

Title: Muscle Biology, Myositis and Myopathies Poster

Session Type: ACR Poster Session C

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

Background/Purpose: To describe the clinical and serologic features, as well as the disease course and response to therapy of anti-PM/Scl patients.

Methods: All Johns Hopkins Myositis Center patients positive for anti-PM/Scl autoantibodies from 2002 to 2016 were included in the study and compared with other autoantibody-positive patients of the cohort. We studied the presence and rate of development of clinical features, the intensity of muscle and lung involvement, and the serologic and biopsy characteristics of these patients.

Results: 41 anti-PM/Scl patients were compared with 445 patients positive for other myositis autoantibodies. Muscle weakness was responsive to first-line immunosuppressants, similar in severity to that of patients with antisynthetase syndrome (AS) and dermatomyositis (DM) autoantibodies, and milder than in patients with immune-mediated necrotizing myopathy autoantibodies. Distal and deltoid weakness was more common in anti-PM/Scl patients than in other myositis subgroups. Muscle biopsies showed marked perivascular inflammation (79%) with scarce perifascicular atrophy (26%). ILD was detected in 61% of anti-PM/Scl patients, more frequently than in DM (13%) and less than in AS (80%), but was milder compared to AS. Extramuscular involvement was common and heterogeneous, with marked skin involvement. No major differences were detected between patients positive for both anti-PM/Scl-75 and anti-PM/Scl-100 and those positive for just one. The nucleolar ANA pattern was effective as a screening technique (sensitivity=94%, specificity=92%) to detect anti-PM/Scl autoantibodies in myositis patients. No increased cancer or mortality rates were detected in these patients.

Conclusion: Anti-PM/Scl myositis is characterized by a particular pattern of weakness, mild lung and muscle involvement, and marked extramuscular features.

Disclosure: R. De Lorenzo, None; I. Pinal-Fernandez, None; M. Casal-Dominguez, None; W. Huang, None; J. C. Milisenda, None; C. Parks, None; K. Pak, None; C. Johnson, None; E. Tiniakou, None; J. Albayda, None; J. J. Paik, None; S. K. Danoff, None; L. Christopher-Stine, OptionCare, 5,Mallinckrodt, 5,Inova Diagnostics, Inc., 7; A. Corse, None; A. Mammen, None.

To cite this abstract in AMA style:

De Lorenzo R, Pinal-Fernandez I, Casal-Dominguez M, Huang W, Milisenda JC, Parks C, Pak K, Johnson C, Tiniakou E, Albayda J, Paik JJ, Danoff SK, Christopher-Stine L, Corse A, Mammen A. Longitudinal Cohort Study of Anti-PM/Scl Myositis Patients: Mild Muscle and Lung Involvement with Prominent Perivascular Inflammation [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/longitudinal-cohort-study-of-anti-pmscl-myositis-patients-mild-muscle-and-lung-involvement-with-prominent-perivascular-inflammation/. Accessed .

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