| Background:Antisynthetase syndrome(ASS)is an autoimmune disease characterized by the occurrence of antibodies directed against different aminoacyl-t RNA synthetase,one of the most common ARS antibodies is anti Jo-1 antibody.Interstitial lung disease(ILD),myositis and arthritis represent the classic clinical triad of the disease,whereas features such as Raynaud's phenomenon(RP),fever,and mechanic's hands(MHs)are less frequently reported.Some anti Jo-1 antibody positive patients have relatively few typical triads at the onset,often presenting only one or two classic triads and often presenting further triads during follow-up.For this reason,we set up this single-center retrospective study including anti Jo-1 antibody positive ASS patients to assess the disease course and outcomes of these patients.Objective: To evaluate the clinical manifestations,long-term clinical outcome and analyzed factors predicting the appearance of further manifestations in incomplete anti-synthetase syndrome(ASS)patients,in order to early detection and diagnosis of ASS.Methods: We collected 125 anti Jo-1 antibody positive patients(31 males and 94females)with a median follow-up of 36(IQR 14-60)months in a single center.Clinical characteristics and laboratory parameters were retrospectively collected and analyzed in anti Jo-1 antibody positive ASS patients.Results: 1.At the onset,complete ASS were 25(20%)and incomplete 100(80%).Patients with incomplete ASS had frequently only 1 of the classic triad findings(67cases,53.6%),in particular,isolated arthritis in 35 cases,isolated myositis in 19 cases,and isolated interstitial lung disease(ILD)in 13 cases.At the end of follow-up,complete ASS were 59(47.6%),incomplete 66(52.4%).Only 6 patients had an isolated arthritis,only 2 an isolated myositis,and 4 an isolated ILD.2.In the 100 incomplete ASS patients,34 patients(34.0%)developed complete ASS,among which patients with isolated arthritis or ILD had a higher rate of progressing to complete ASS than patients with isolated myositis.Patients developed new accompanying features during follow up,the odds ratio for the occurrence of complete ASS was 2.9 with respect to patients without new accompanying features.3.During the follow-up,in the 100 incomplete ASS patients,67 patients(67.0%)with incomplete forms developed further manifestations.Single main feature onset was the main risk factor for the ex novo appearance of further manifestation(OR=10.3,95% CI(3.7,28.8,P<0.001).ILD was the prevalent ex novo manifestation(116 cases,92.8%).And the new manifestations was observed in 37 out of 67 patients(55.2%)with new accompanying findings during follow-up.In patients with at least one new accompanying feature,especially the fever,the odds ratio for the occurrence of new triad manifestations was 4.0 with respect to patients not developing ex-novo accompanying findings(95% CI 1.2-12.8,P=0.021).We didn't find the laboratory data potentially associated with a pattern progression.4.Then,estimated survival rates in our cohort of patients with anti-Jo-1 antibody positive ASS were95.1%,93.0%,91.7%and 64.6% at 1,2,5 and 10 years.5.Furthermore,the most common pattern was NSIP(59 cases,50.9%),followed by NSIP/OP(20 cases,17.2%)and OP(19 cases,16.4%)in anti Jo-1 antibody positive ASS patients with ILD.The survival rate in our cohort of patients with ILD were 94.7%,92.5%,91.3% and 62.6%at1,2,5 and 10 years.Conclusion: 53% anti-Jo-1 antibody-positive ASS patients had isolated arthritis,myositis,or ILD at the onset.And 67% of patients with incomplete ASS had clinical progression at a median time of 25 months,the percentage of complete ASS increasedfrom 20.0% to 47.6% during follow up.Patients with different manifestations have different possibility of disease progression.Interstitial lung disease is the most common manifestation during follow-up,the percentage increased from 51.2% to92.8%,and the most common imaging pattern was NSIP.Anti Jo-1 antibody positive ASS patients have a moderate prognosis. |
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