Clinical Follow-up Predictors of Disease Pattern Change in Anti Jo-1 Positive Antisynthetase Syndrome: Results from a Multicenter, International and Retrospective Study

Franco Franceschini¹, Elena Bartoloni-Bocci², Santos Castañeda³, Laura Nuno⁴, Carlo Alberto Scirè⁵, Francisco Javier López-Longo⁶, Julia Martínez-Barrio⁷, Ilaria Cavazzana¹, Paolo Airò⁸, Javier Bachiller Corral⁹, Alberto Sifuentes Giraldo¹⁰, Rossella Neri¹¹, Simone Barsotti¹², Roberto Caporali¹³, Carlomaurizio Montecucco¹⁴, Marcello Govoni¹⁵, Renato La Corte¹⁵, Federica Furini¹⁵, Florenzo Iannone¹⁶, Margherita Giannini¹⁷, Enrico Fusaro¹⁸, Simone Parisi¹⁹, Giuseppe Paolazzi²⁰, Giovanni Barausse²¹, Raffaele Pellerito²², Alessandra Russo²², Lesley Ann Saketkoo²³, Norberto Ortego-Centeno²⁴, Luca Quartuccio²⁵, Christof Specker²⁶, Andreas Schwarting²⁷, Kostantinos Triantafyllias²⁸, Carlo Selmi²⁹, Fausto Salaffi³⁰, Marco Amedeo Cimmino³¹, Annamaria Iuliano³², Fabrizio Conti³³, Gianluigi Baiocchi³⁴, Elena Bravi³⁵, Anna Ghirardello³⁶, Trinitario Pina³⁷, Miguel A. Gonzalez-Gay³⁸, Lorenzo Cavagna³⁹ and AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group, ¹Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy, ²Department of Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, ³Rheumatology, H.U. La Princesa, Madrid, Spain, ⁴Rheumatology, Hospital Universitario La Paz, Madrid, Spain, ⁵Epidemiology Unit – Italian Society for Rheumatology (SIR), Milano, Italy, ⁶Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain, ⁷Servicio de Reumatologia, Hospital General Universitario Gregorio Marañón, Madrid, Spain, ⁸Rheumatology Unit, Spedali Civili of Brescia, Brescia, Italy, ⁹Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹⁰Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹¹Rheumatology Unit, University of Pisa, Italy, Pisa, Italy, ¹²Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy, ¹³University of Pavia and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy, ¹⁴Division of Rheumatology, University of Pavia, IRCCS Foundation Policlinico S. Matteo, Pavia, Italy, ¹⁵UOC Reumatologia, Azienda Ospedaliera Universitaria S. Anna, Ferrara, Italy, ¹⁶Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy, ¹⁷DIM, Rheumatology Unit, Bari, Italy, ¹⁸Department of Rheumatology, Città della Salute e della Scienza, Torino, Italy, ¹⁹Department of Rheumatology, Città Della Salute e della Scienza, Torino, Italy, ²⁰Rheumatology Unit, Santa Chiara Hospital, Trento, Italy, ²¹Rheumatology, Ospedale di Trento, Trento, Italy, ²²Division of Rheumatology, Mauriziano Hospital, Turin, Italy, ²³New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Tulane University Lung Center, New Orleans, LA, ²⁴Hospital San Cecilio. Granada, Granada, Spain, ²⁵S. Maria della Misericordia, University of Udine, Italy, Udine, Italy, ²⁶Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany, ²⁷Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany, ²⁸ACURA Rheumatology Center, Bad Kreuznach, Germany, ²⁹Internal Medicine- Unit of Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Rozzano, Italy, ³⁰Rheumatology Department, Polytechnic University of Marche, C. Urbani Hospital, Jesi,, Ancona, Italy, ³¹Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy, ³²Osp. San Camillo, Roma, Italy, ³³Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Rome, Italy, ³⁴Rheumatology Unit, Department of Internal Medicine, S.Maria Hospital –IRCCS, Reggio Emilia, Italy, ³⁵Internal Medicine, Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy, ³⁶Department of Medicine-DIMED, University of Padova, Padova, Italy, ³⁷Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Santander, Spain, ³⁸Reumatologia, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain, ³⁹Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: arthritis and myositis, Raynaud's phenomenon

Session Information

Date: Tuesday, November 10, 2015

Title: Muscle Biology, Myositis and Myopathies Poster II: Autoantibodies and Treatments in Inflammatory Myopathies

Session Type: ACR Poster Session C

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

Background/Purpose: arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of antisynthetase syndrome (ASSD). Even if reported in up to 90% of cases, concomitant onset of these manifestations uncommon. A recent study has shown that 60% of anti Jo-1 positive ASSD presenting without the complete triad may develop other manifestations in a very wide range of time. At baseline, the only predictor of subsequent disease pattern evolution is the onset with just one of the classic triad findings, whereas the occurrence of other ASSD typical but less frequently observed manifestations, such as Raynaud’s phenomenon (RP), fever or mechanic’s hands (MH), does not add further information. The aim of this multicenter, international, retrospective study is to assess whether the appearance of RP, fever or MH (defined as accompanying features) during the follow-up may predict the subsequent occurrence of the baseline absent classic triad manifestations

Methods: anti Jo-1 positive patients presenting with no more than 2 classic triad findings (for a practical purpose defined as incomplete ASSD) were included in the analysis. Clinical characteristics and different clusters of disease manifestations were retrospectively collected and analyzed

Results: we identified 170 patients (126 females, 44 males) with incomplete ASSD. The median age at disease onset was 53 years (IQR 42-64), median follow-up 88 months (IQR 48-155). Ninety-nine patients (58%) developed new classic triad manifestations and 41 (24%) new accompanying features, especially in those patients in whom isolated arthritis was the first manifestation (p=0.0049). In these 41 patients, the subsequent ex-novo appearance of classic triad features was statistically increased (p=0.0170) and it was always concomitant or subsequent to the development of accompanying features. Furthermore, these patients had more frequently all triad manifestations (p=0.0004). The clinical characteristics of patients and statistical significances are reported in table 1 and 2

Conclusion: in anti Jo-1 positive patients with incomplete ASSD the occurrence of RP, fever or MH during the follow-up may suggest the subsequent occurrence of new classic triad manifestations

References

Cavagna L, et al. Ann Rheum Dis 2015;74(Suppl2): 601
Cavagna L, et al. Arthritis Rheum 2014;66(Suppl):550

	New accompanying features		p
	Yes	Not	p
Patients number (%)	41 (24)	129 (76)	–
Female sex (% of subset)	30 (73)	96 (74.5)	0.964
Median age in years at disease onset (IQR)	54 (42-64)	49 (40.5-60.5)	0.240*
Median follow-up in months (IQR)	100 (61-167)	84 (43.5-153)	0.365*
Baseline triad manifestations
Isolated arthritis (% of subset)	20 (49)	31 (24)	0.0049
Isolated myositis (% of subset)	6 (14.5)	22 (17)	0.970
Isolated ILD (% of subset)	3 (7)	22 (17)	0.200
Arthritis and myositis (% of subset)	6 (14.5)	21 (16.5)	0.995
Arthritis and ILD (% of subset)	2 (5)	12 (9)	0.570
Myositis and ILD (% of subset)	4 (10)	21 (16.5)	0.440
Final triad manifestations
Isolated arthritis (% of subset)	1 (2.5)	4 (3)	0.775
Isolated myositis (% of subset)	0 (0)	5 (4)	0.454
Isolated ILD (% of subset)	1 (2.5)	14 (11)	0.180
Arthritis and myositis (% of subset)	3 (7.5)	22 (17)	0.200
Arthritis and ILD (% of subset)	4 (10)	19 (15)	0.583
Myositis and ILD (% of subset)	6 (15)	25 (19)	0.650
Arthritis, myositis and ILD (% of subset)	26 (62.5)	40 (31)	0.0004
New development of classic triad manifestations (% of subset)	33 (80.5)	66 (51)	0.0170

Table 1: overtime disease pattern changes of classic triad manifestations (eg arthritis, myositis and ILD) according to the occurrence or not of accompanying features (eg Raynaud’s phenomenon, fever, mechanic’s hands) during the follow-up. Legend: ILD: interstitial lung disease. *Independent Sample T test (if equal variances) or Welch-test (if unequal variances). Others: Chi-square test.

	Disease onset	Last follow-up	p*
Total Raynaud’s phenomenon (%)	37 (22)	59 (35)	0.011
Total Mechanic’s hands (%)	31 (18)	50 (29)	0.022
Total Fever (%)	36 (21)	57 (34)	0.015
Isolated Raynaud’s phenomenon (%)	26 (15)	26 (15)	0.880
Isolated Mechanic’s hands (%)	16 (9)	17 (10)	1.000
Isolated Fever (%)	29 (17)	22 (13)	0.362
Raynaud’s phenomenon and Mechanic’s hands (%)	4 (2)	9 (5)	0.680
Raynaud’s phenomenon and Fever (%)	2 (1)	11 (6.5)	0.0237
Fever and mechanic’s hands (%)	6 (4)	11 (6.5)	0.320
Raynaud’s phenomenon, fever and mechanic’s hands (%)	5 (3)	13 (8)	0.090
None (%)	82 (48)	61 (36)	0.0250

Table 2: prevalence and cluster of Raynaud’s phenomenon, mechanic’s hands and fever at baseline and at last available follow-up. * Chi-square test.

Disclosure: F. Franceschini, None; E. Bartoloni-Bocci, None; S. Castañeda, None; L. Nuno, None; C. A. Scirè, None; F. J. López-Longo, None; J. Martínez-Barrio, None; I. Cavazzana, None; P. Airò, None; J. Bachiller Corral, None; A. Sifuentes Giraldo, None; R. Neri, None; S. Barsotti, None; R. Caporali, UCB, Roche, 8,AbbVie, Pfizer, MSD, 5; C. Montecucco, None; M. Govoni, None; R. La Corte, None; F. Furini, None; F. Iannone, None; M. Giannini, None; E. Fusaro, None; S. Parisi, None; G. Paolazzi, None; G. Barausse, None; R. Pellerito, None; A. Russo, None; L. A. Saketkoo, None; N. Ortego-Centeno, None; L. Quartuccio, None; C. Specker, None; A. Schwarting, None; K. Triantafyllias, None; C. Selmi, None; F. Salaffi, None; M. A. Cimmino, None; A. Iuliano, None; F. Conti, None; G. Baiocchi, None; E. Bravi, None; A. Ghirardello, None; T. Pina, None; M. A. Gonzalez-Gay, None; L. Cavagna, None.

To cite this abstract in AMA style:

Franceschini F, Bartoloni-Bocci E, Castañeda S, Nuno L, Scirè CA, López-Longo FJ, Martínez-Barrio J, Cavazzana I, Airò P, Bachiller Corral J, Sifuentes Giraldo A, Neri R, Barsotti S, Caporali R, Montecucco C, Govoni M, La Corte R, Furini F, Iannone F, Giannini M, Fusaro E, Parisi S, Paolazzi G, Barausse G, Pellerito R, Russo A, Saketkoo LA, Ortego-Centeno N, Quartuccio L, Specker C, Schwarting A, Triantafyllias K, Selmi C, Salaffi F, Cimmino MA, Iuliano A, Conti F, Baiocchi G, Bravi E, Ghirardello A, Pina T, Gonzalez-Gay MA, Cavagna L. Clinical Follow-up Predictors of Disease Pattern Change in Anti Jo-1 Positive Antisynthetase Syndrome: Results from a Multicenter, International and Retrospective Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/clinical-follow-up-predictors-of-disease-pattern-change-in-anti-jo-1-positive-antisynthetase-syndrome-results-from-a-multicenter-international-and-retrospective-study/. Accessed .

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