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2015 ; 94
(32
): e1144
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome:
Results From an International Retrospective Multicenter Study
#MMPMID26266346
Cavagna L
; Nuño L
; Scirè CA
; Govoni M
; Longo FJL
; Franceschini F
; Neri R
; Castañeda S
; Giraldo WAS
; Caporali R
; Iannone F
; Fusaro E
; Paolazzi G
; Pellerito R
; Schwarting A
; Saketkoo LA
; Ortego-Centeno N
; Quartuccio L
; Bartoloni E
; Specker C
; Murcia TP
; La Corte R
; Furini F
; Foschi V
; Corral JB
; Airò P
; Cavazzana I
; Martínez-Barrio J
; Hinojosa M
; Giannini M
; Barsotti S
; Menke J
; Triantafyllias K
; Vitetta R
; Russo A
; Bajocchi G
; Bravi E
; Barausse G
; Bortolotti R
; Selmi C
; Parisi S
; Montecucco C
; González-Gay MA
Medicine (Baltimore)
2015[Aug]; 94
(32
): e1144
PMID26266346
show ga
Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD),
an autoimmune disease clinically characterized by the occurrence of arthritis,
myositis, and interstitial lung disease (ILD). These manifestations usually
co-occur (for practical purpose complete forms) in the same patient, but cases
with only 1 or 2 of these findings (for practical purpose incomplete forms) have
been described. In incomplete forms, the ex novo occurrence of further
manifestations is possible, although with frequencies and timing not still
defined. The aim of this international, multicenter, retrospective study was to
characterize the clinical time course of anti Jo-1 positive ASSD in a large
cohort of patients. Included patients should be anti Jo-1 positive and with at
least 1 feature between arthritis, myositis, and ILD. We evaluated the
differences between complete and incomplete forms, timing of clinical picture
appearance and analyzed factors predicting the appearance of further
manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males
and 167 females) with a median follow-up of 80 months. At the onset, complete
ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently
only 1 of the classic triad findings (110 cases), in particular, isolated
arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28
cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5
patients had an isolated arthritis, only 5 an isolated myositis, and 15 an
isolated ILD. During the follow-up, 108 patients with incomplete forms developed
further manifestations. Single main feature onset was the main risk factor for
the ex novo appearance of further manifestation. ILD was the prevalent ex novo
manifestation (74 cases). In conclusion, ASSD is a condition that should be
carefully considered in all patients presenting with arthritis, myositis, and
ILD, even when isolated. The ex novo appearance of further manifestations in
patients with incomplete forms is common, thus indicating the need for an
adequate clinical and instrumental follow-up. Furthermore, the study clearly
suggested that in ASSD multidisciplinary approach involving Rheumatology,
Neurology, Pneumology, and Internal Medicine specialists is mandatory.