ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1256

Clinical and Temporal Characterization of Anti-Jo-1 Positive Anti-Synthetase Syndrome: Preliminary Results of an International Multicentre Study

Lorenzo Cavagna1, Miguel A González-Gay2, Santos Castañeda-Sanz3, Franco Franceschini4, Paolo Airo5, Ilaria Cavazzana6, Laura Nuno7, Trinitario Pina Murcia2, Francisco Javier Lopez Longo8, Norberto Ortego-Centeno9, Rossella Neri10, Simone Barsotti10, Enrico Fusaro11, Simone Parisi12, Giuseppe Paolazzi13, Giovanni Barausse13, Luca Quartuccio14, Elena Bartoloni-Bocci15, Carlo Selmi16, Carlo Alberto Scirè17, Elena Bravi18, Javier Bachiller Corral19, Lesley Ann Saketkoo20, Gianluigi Bajocchi21, Raffaele Pellerito22, Marcello Govoni23, Andreas Schwarting24, Christof Specker25, Carlomaurizio Montecucco1 and Roberto Caporali26, 1Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy, 2Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain, 3Rheumatology, Hospital Universitario de La Princesa. Madrid. Spain, Madrid, Spain, 4AO Spedali Civili, Rheumatology and Clinical Immunology Unit, Brescia, Italy, 5Rheumatology and Clinical Immunology, Spedali Civili, AO Spedali Civili, Brescia, Italy, 6Rheumatology and Clinical Immunology Unit, AO Spedali Civili, Brescia, Italy, 7Rheumatology, Hospital Universitario La Paz, Madrid, Spain, 8Rheumatology, Hospital Gregorio Marañón. Madrid, Madrid, Spain, 9Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain, 10Rheumatology Unit, University of Pisa, Pisa, Italy, 11Department of Rheumatology, Azienda Ospedaliera Universitaria Città della Salute e della Scienza di Torino, Turin, Italy, 12Rheumatology, Azienda Ospedaliera Città Della Salute e della Scienza, Turin, Italy, 13Rheumatology, Santa Chiara Hospital, Trento, Italy, 14Rheumatology, DSMB, University Hospital Santa Maria della Misericordia, Udine, Italy, 15Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, 16Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Italy, 17Italian Society for Rheumatology, Milan, Italy, 18Internal Medicine, Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy, 19Rheumatology, Hospital Ramon y Cajal, Madrid, Spain, 20Scleroderma and Sarcoidosis Patient Care and Research Center, Rheumatology and Pulmonary Medicine, Louisiana State University Health Sciences Center, New Orleans, LA, 21Department of Internal Medicine, Rheumatology Unit, Arcispedale S Maria Nuova. IRCCS, Reggio Emilia, Italy, 22Rheumatology, Ospedale Mauriziano, Turin, Italy, 23Clinical and Experimental Medicine, Section of Rheumatology, University of Ferrara, Ferrara, Italy, 24Rheumatology, University Hospital Johannes-Gutenberg, Mainz, Germany, 25Rheumazentrum Rhein-Ruhr e.V., St. Josef Krankenhaus (Kliniken Essen Süd), Universitätsklinikum Essen, Esssen, Germany, 26Rheumatology, Division of Rheumatology, University of Pavia, IRCCS Policlinico San Matteo, Pavia, Italy

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: , ,

Session Information

Title: Muscle Biology, Myositis and Myopathies: Myositis Autoantibodies and Disease Phenotype

Session Type: Abstract Submissions (ACR)

Background/Purpose: anti Jo-1 antibodies are the main marker of the antisynthetase syndrome (As), a connective tissue disease chiefly characterized by arthritis (A), myositis (M) and interstitial lung disease (I). These manifestations may occur concomitantly but incomplete clinical pictures have been described. Aim of this study is to further characterize clinical presentation and course of anti Jo-1 positive As

Methods: data from multicentre, multinational series of patients diagnosed with anti Jo-1 positive As were retrospectively collected and analyzed. Anti Jo-1 antibodies were tested in all cases by commercially available ELISA techniques

Results: 146 patients were included (Table 1). Median follow up was 91 months (IQR 40.25-153.75). At the onset, a complete clinical picture (AMI) was present in 26 patients (18%). Seventy-two patients (49%) had monosymptomatic presentation (A 36, 24.5%; M 20, 14%; I 16, 11%), 19 AM (13.5%), 19 MI (13.5%) and 10 AI (7%). Arthritis was the most frequent feature at the onset, the only detectable in 24.5% of patients. Many patients meet the 2010 ACR/EULAR classification criteria for rheumatoid arthritis (RA). Rheumatoid factor, anti citrullinated peptide antibodies and RA typical radiographic erosions were not a rare finding. Time from clinical onset to diagnosis was longer in incomplete forms (p<0.001). During the follow up, 69 patients (57.5%) developed additional features (median time to progression: 17 months, IQR 7.5-43.5). Progression rate was significantly higher in patients with single feature onset (57/72) with respect to those with two features (12/48) (p<0.001). At the last follow-up, 69 patients (47%) had AMI, 23 (15.5%) AM, 23 (15.5%) MI, 16 (11%) AI, 8 (5.5%) I, 4 (3%) A and 3 (2%) M. Twenty-five patients (17.5%) died, 7 (5%) due to As progression. First line treatment was based on corticosteroids (140 patients, 96%) and immunosuppressants (112, 77%; hydroxychloroquine, HCQ, 30; methotrexate, MTX, 24; IV cyclophosphamide, CYC, 20; cyclosporine, Cys, 20; azathioprine, AZA, 17; mycophenolate mofetil, MMF, 4; tacrolimus, TC, 3; other not listed). Globally, MTX was prescribed in 58 patients (40%, 22 withdrawn for side effects/ineffectiveness), Cys in 47 (32%, 10), AZA 36 (24.5%, 21), CYC 34 (23%, 23), HCQ 34 (23%, 21), MMF in 17 (11.5%, in 5), TC in 9 (6%, 5)

Conclusion: the diagnosis of anti Jo1 positive As may be delayed in incomplete forms, which are the vast majority at presentation. Joint involvement may be very similar to RA, adding further challenges to differential diagnosis. Clinical picture evolution is common in patients presenting with incomplete forms and it may occur even after several years from disease onset. Treatment appears heterogeneous and with high drop-out rates in both first and second line. This suggests the need of better strategies supported by specific recommendations

 

Disclosure:

L. Cavagna,
None;

M. A. González-Gay,
None;

S. Castañeda-Sanz,
None;

F. Franceschini,
None;

P. Airo,
None;

I. Cavazzana,
None;

L. Nuno,
None;

T. Pina Murcia,
None;

F. J. Lopez Longo,
None;

N. Ortego-Centeno,
None;

R. Neri,
None;

S. Barsotti,
None;

E. Fusaro,
None;

S. Parisi,
None;

G. Paolazzi,
None;

G. Barausse,
None;

L. Quartuccio,
None;

E. Bartoloni-Bocci,
None;

C. Selmi,
None;

C. A. Scirè,
None;

E. Bravi,
None;

J. Bachiller Corral,
None;

L. A. Saketkoo,
None;

G. Bajocchi,
None;

R. Pellerito,
None;

M. Govoni,
None;

A. Schwarting,
None;

C. Specker,
None;

C. Montecucco,
None;

R. Caporali,
None.

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