Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
Clinical Features and Survival in Anti-PL-7 Autoantibody Positive Myositis Patients From a Single Tertiary Care Center.
Aggarwal1, Rohit, Koontz2, Diane, Fertig2, Noreen, Oddis2, Chester V., Agudelo-Hernandez1, Arcadio
To describe the demographic, clinical features and survival in a cohort of anti-PL-7 autoantibody positive myositis patients from a single tertiary care center
All PL-7 (+) patients with their first evaluation between 19852009 were included regardless of connective tissue disease (CTD) diagnosis. Anti-PL-7 was detected using RNA and protein immunoprecipitation techniques. ANA and cytoplasmic pattern was determined by indirect immunoflourescence (IIF) using Hep2 cell. All data were collected from the registry database and medical record. Mortality data was determined using National Death Index or Social Security Death Index, and medical record review. Survival analysis was done using Kaplan Meier plots.
We identified 25 PL-7 (+) patients in our cohort. Eighteen (72%) were female and 22 (88%) Caucasian with a mean (± SD) age of 55.1 (15.4) years at diagnosis. The initial CTD symptom occurred at a mean age of 49.1 (18.2), and their first evaluation at our center was at age 57.0 (13.3) with a diagnosis delay of 6.0 (10.2) years [median (IQR) = 1.08 (0.56.8) years]. Only 13 (52%) patients were ANA positive, whereas 23 (92%) had cytoplasmic pattern on IIF (7 speckled; 6 diffuse; 10 both diffuse and speckled). The initial diagnoses included dermatomyositis (DM) (n=7), polymyositis (n=5), systemic sclerosis (n=1), overlap (n=7), UCTD/unknown (n=5). The most common initial CTD symptoms were Raynauds (n=9), arthralgia/arthritis (n=5), followed by muscle weakness (n=2), rash (n=2) and dyspnea (n=2). ILD was detected in 92% (23/25) of patients and 20% (5/25) had pulmonary hypertension (PHT) (3 primary/2 secondary). Most patients (19/23) developed pulmonary involvement before an established CTD diagnosis with nearly 50% having pulmonary symptoms > 6 months before the CTD diagnosis. The mean time of follow up was 4.9 (4.1) years with the cohort developing the following clinical features at some time during their course: fatigue 100%, Raynauds 84%, dyspnea 72% (n=18; 12 moderate to severe, 6 on home O2), myalgia 48%, fever 44%, arthritis 44%, DM rash 36%, dysphagia 32%,telangectasia 32%, puffy fingers 32%,, pleurisy 16%, and mechanics hands in 5 of 8 evaluated specifically for it. Proximal muscle weakness was seen in 76% (19/25) of the cohort but nearly 50% (n=12) had mild/subtle weakness. Nineteen (76%) patients had CK elevations with a median peak CK 7 times the upper limit of normal [IQR 3.716.5]. The median (IQR) survival time in years for the entire cohort was poor at 6.42 (4.221.6) years. The 5 and 10 year unadjusted cumulative survival rates were: 66.5% (95% CI 41.882.6) and 30.8% (95% CI 11.752.4), respectively. Overall, 14 (56%) patients died during the study period with an average age at death of 59.2 (13.9) years. The most common cause of death was pulmonary in 11/14 (78.6%) patients (8 with fibrosis and 3 with PHT).
We describe detailed clinical features and survival of a large anti-PL-7 (+) cohort. We emphasize the importance of considering this autoantibody in patients presenting with both pulmonary symptoms and other CTD features even in the absence of clinically overt myositis. This subset of myositis is also associated with a high mortality rate.
To cite this abstract, please use the following information:
Aggarwal, Rohit, Koontz, Diane, Fertig, Noreen, Oddis, Chester V., Agudelo-Hernandez, Arcadio; Clinical Features and Survival in Anti-PL-7 Autoantibody Positive Myositis Patients From a Single Tertiary Care Center. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :229