Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Predictors of Survival in Patients with Anti-Synthetase Autoantibodies.

Aggarwal5,  Rohit, Cassidy1,  Elaine A., Fertig4,  Noreen, Lucas4,  Mary, Ascherman2,  Dana P., Oddis3,  Chester V.

Children's Hospital Pittsburg, Pittsburgh, PA
University of Pittsburgh, Pittsburgh, PA
University of Pittsburgh, Pittsburgh, PA
University of Pittsburgh
University of Pittsburgh Medical Center, Pittsburgh, PA

Purpose:

To evaluate baseline clinical, laboratory, serologic, radiographic and functional predictors of survival in patients (pts) with anti-synthetase autantibodies (anti-synAb) from a single tertiary care center.

Methods:

All anti-synAb+ pts from a single referral center between1985–2009 were included regardless of their initial or subsequent connective tissue disease (CTD) diagnosis. All data were collected from the registry database and medical record. Current status and cause of death was determined by query of the National Death Index or Social Security Death Index, and record review. For lung survival an endpoint of lung transplant or death was used. Each baseline characteristic was evaluated for prediction of overall and lung survival using Cox-proportional hazard in a univariate fashion. Only clinical data available at first visit was used in the model.

Results:

Of the 3880 CTD pts in the registry from 1985–2009, 202 (5.2%) were anti-synAb+[122 Jo-1; 80 non Jo-1 (35 PL-12; 25 PL-7; 9 EJ; 6 KS; 5 OJ)]. Eighty-nine (44%) had PM, 44 (22%) DM, and 69 (34%) had undifferentiated or overlap CTD. Demographic features predicting survival included age at diagnosis and symptom onset, and a diagnosis delay (Table 1). PM pts had worse survival. Among other variables only arthralgia, pleurisy, dermatomyositis V neck or trunk rash, and telangiectasia had worse survival while Gottron papules had better survival. Raynaud, sicca symptoms, dyspnea, fever, muscle weakness, dysphagia, puffy fingers, mechanic hands, heliotrope rash, physician global assessment of active disease, capillary microscopy, digital abnormality, and calcinosis did not predict survival. Among lab and serologic parameters only non-Jo1 anti-synAb positivity, particularly anti-PL-7 and anti-EJ predicted worse survival, while SSA antibody was protective. ANA positivity, muscle enzymes, hemoglobin, platelet and ESR had no impact on survival. The % predicted FVC and DLCO along with pulmonary hypertension (PHT) by echocardiography and secondary PHT at baseline predicted survival. High resolution CT (HRCT) evidence of pulmonary fibrosis did not predict survival as most pts had some evidence of fibrosis by routine chest radiography or HRCT.

Table 1. Univariate predictors of overall and lung survival in a synthetase cohort

 Overall survival Hazzard ratio (C.I.)p valueLung survival Hazard ratio (C.I.)p value
Demographic
Gender (male)0.82 (0.47–1.44)0.570.82 (0.48–1.43)0.47
Ethnicity (AA)0.92 (0.43–1.96)0.871.0 (0.49–2.04)0.98
Age at diagnosis (yrs)1.03 (101–1.05)<0.0011.03 (1.01–1.05)<0.001
Age at symptoms (yrs)1.02 (1.0–1.04)0.071.02 (1.0–1.03)0.01
Diagnosis delay (yrs)1.03 (1.0–1.06)0.041.01 (1.0–1.01)0.008
Clinically history
Clinical diagnosisPM: 2.4 (1.08–5.53)0.032PM: 2.73 (1.21–6.15)PM: 0.015
   UCTD 2.54 (1.11–5.84)UCTD 0.27
Inflammatory arthralgia1.84 (1.08–3.150.021.78 (1.08–2.95)0.02
V neck trunk rash2.9 (1.14–7.32)0.022.64 (1.05–.6.65)0.04
Gottron papules0.33 (0.14–0.78)0.010.32 (0.14–0.71)0.005
Telangiectasia2.6 (1.4–4.8)0.0032.93 (1.64–5.25)<0.001
Pleurisy1.88 (1.02–3.48)0.041.97 (1.06–3.63)0.03
All non-Jo11.67 (1.05–2.68)0.031.64 (1.03–2.63)0.04
Anti-PL-72.45 (1.31–4.56)0.0052.55 (1.37–4.73)0.003
Anti-EJ3.29 (1.27–8.49)0.013.14 (1.22–8.09)0.01
SSA (n = 81)0.11 (0.15–0.85)0.040.10 (0.13–0.76)0.03 Pulmonary Function Testing Other
%FVC 10.97 (0.96–0.99)0.030.97 (0.95–0.98)0.001
%DLCO0.96 (0.94–0.98)0.0060.96 (0.94–0.99)0.006
Elevated PAP3.1 (1.3–70)0.0063.2 (1.53–6.74)0.002
Secondary PHT3.46 (1.65–7.27)0.0013.34 (1.59–7.0)0.001
O2 requirement at baseline2.43 (1.38–4.26)0.0022.42 (1.37–4.21)0.002
CI = confidence interval, AA = African American, PAP = Pulmonary artery pressure

Conclusion:

The myositis clinical subset (i.e. PM), anti-synAb positivity, pulmonary function parameters, secondary PAH, joint involvement, pleurisy, various DM rashes and telangiectasia help predict overall survival and lung survival in anti-synAb+ pts. Some findings can be explained on the basis of their known myositis subset associations, such as Gottron papules in DM (which has a better prognosis than PM) and telangiectasias in UCTD (worse survival due to non-Jo-1 association). However, others require further study to confirm their association with survival (e.g. SSA positivity).

To cite this abstract, please use the following information:
Aggarwal, Rohit, Cassidy, Elaine A., Fertig, Noreen, Lucas, Mary, Ascherman, Dana P., Oddis, Chester V.; Predictors of Survival in Patients with Anti-Synthetase Autoantibodies. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :930
DOI: 10.1002/art.28698

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