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.
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 September 28, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/longitudinal-cohort-study-of-anti-pmscl-myositis-patients-mild-muscle-and-lung-involvement-with-prominent-perivascular-inflammation/