Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: Results from a multicenter, international and retrospective study.
OBJECTIVE
Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients.


METHODS
Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients.


RESULTS
165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002).


CONCLUSIONS
Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.
Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. Electronic address: elena.bartolonibocci@unipg.it.
Elena
E
Bartoloni
Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
Miguel A
MA
Gonzalez-Gay
Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy.
Carlo
C
Scirè
Rheumatology Department, Hospital Universitario de la Princesa, IIS-IP, Madrid, Spain.
Santos
S
Castaneda
Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
Roberto
R
Gerli
Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Francisco Javier
FJ
Lopez-Longo
Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Julia
J
Martinez-Barrio
UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy.
Marcello
M
Govoni
UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy.
Federica
F
Furini
Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
Trinitario
T
Pina
Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy.
Florenzo
F
Iannone
Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy.
Margherita
M
Giannini
Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain.
Laura
L
Nuño
Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, Udine, Italy.
Luca
L
Quartuccio
Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain.
Norberto
N
Ortego-Centeno
Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
Alessia
A
Alunno
Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany.
Christopher
C
Specker
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy.
Carlomaurizio
C
Montecucco
ACURA Rheumatology Center, Bad Kreuznach, Germany.
Konstantinos
K
Triantafyllias
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy.
Silvia
S
Balduzzi
Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain.
Walter Alberto
WA
Sifuentes-Giraldo
Rheumatology Unit, Santa Chiara Hospital, Trento, Italy.
Giuseppe
G
Paolazzi
Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy.
Elena
E
Bravi
Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany.
Andreas
A
Schwarting
Division of Rheumatology, Mauriziano Hospital, Turin, Italy.
Raffaele
R
Pellerito
Division of Rheumatology, Mauriziano Hospital, Turin, Italy.
Alessandra
A
Russo
Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy.
Carlo
C
Selmi
Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center New Orleans, New Orleans, LA, USA.
Lesley-Ann
LA
Saketkoo
Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy.
Enrico
E
Fusaro
Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy.
Simone
S
Parisi
Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera ASMN, Reggio Emilia, Italy.
Nicolò
N
Pipitone
Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy.
Franco
F
Franceschini
Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy.
Ilaria
I
Cavazzana
Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Rossella
R
Neri
Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Simone
S
Barsotti
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy.
Veronica
V
Codullo
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy.
Lorenzo
L
Cavagna
D001323:Autoantibodies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008025:Ligases / Q000037:antagonists & inhibitors*; D008297:Male; D008875:Middle Aged; D011928:Raynaud Disease / Q000378:metabolism*; D012189:Retrospective Studies; D013577:Syndrome
D001323:Autoantibodies; D008025:Ligases
Anti-synthetase syndrome; Interstitial lung disease; Myositis; Prognosis; Raynaud's phenomenon; mechanic's hand