Clinical Characteristics And Risk Of Relapse For Adult Onset Still's Disease

BackgroundAdult onset still's disease(AOSD)is a rare multisystem autoinflammatory disorder disease,typically manifesting as a high spiking fever,arthralgia or arthritis,skin rash,leukocytosis,and hyperferritinemia.To date,the pathogenesis of AOSD is not clearly understood.Typical eruption is evanescent,macular or maculopapular,which appears with fever fades away as fever subsides.In recent years,another atypical rash of AOSD that differs from the typical eruptions has been reported in the literature cases.The atypical rash is characterized by persistent squamous erythema,papules or plaques with severe itching,which is non-evanescent,and is called persistent pruritic eruptions(PPEs).The treatment of AOSD remains empirical,is mainly based on case studies and lacks of clinical trial evidence,including corticosteroids,non-steroidal anti-inflammatory drugs(NSAIDs)and disease-modifying anti-rheumatic drugs(DMARDs).The effectiveness of these treatment ranges from 20% to 60%.More and more evidence demonstrate that targeted therapy is effective in controlling AOSD,especially for patients who show refractory response to traditional treatment.Although biological agents,including tumor necrosis factor(TNF),interleukin(IL)-1,IL-6,and IL-18,have potential effects in refractory AOSD patients,corticosteroids are still widely used in treating AOSD,especially when systemic symptoms predominate.NSAIDs are no longer considered as a first-line treatment because of their poor risk-benefit ratio.It seems that many factors are considered to be related to the prognosis of AOSD,including fever(> 39.5?),thrombocytopenia,hyperferritinemia and arthritis.But most of them come from single-center,small-sample studies.Few studies have focused on the clinical characteristics and prognostic factors in AOSD.This study is a systematic retrospective study of AOSD patients in our hospital,focusing on the clinical features and prognosis analysis about eruptions and PPEs,providing help for the clinical diagnosis and treatment of AOSD in the future.ObjectiveInvestigating whether the patients with or without cutaneous manifestations and different types of eruptions of AOSD have different clinical manifestations and laboratory tests.To retrospectively analyze the risk of relapse for AOSD,and to provide more information and evidence for clinical diagnosis and treatment of AOSD.MethodsRetrospective data of patients diagnosed with AOSD in our institute during 2013-2019 were analyzed.The diagnoses were based on the Yamaguchi criteria for AOSD.The basic information including age,sex and date at diagnosis.Clinical manifestation including fever,arthralgia or arthritis,pharyngitis,myalgia,lymphadenopathy,splenomegaly or hepatomegaly,serositis,abdominal pain and rash.Laboratory examinations including blood routine examination,liver function,erythrocyte sedimentation rate,serum ferritin,autoantibody series,imaging examination and other special laboratory examinations.All long-term follow-up data were collected from medical records and phone calls.Continuous variables are summarized as mean ± standard deviation and compared using the Chi-squared(?2)test.Categorical variables are summarized as frequency(percent)and compared using either the Student's t-test or the Mann-Whitney U significant test.Regression analyses is performed to assess the factors associated with rash and relapse.ResultsIn total,166 patients with AOSD were included in this study.The mean age of the patients was 41.98±16.43 years,and 114 patients(68.7%)were female.For clinical manifestations,the proportion of female patients(p=0.003),the incidence of arthralgia or arthritis(p=0.025)and lymphadenopathy(p=0.035)in the group with cutaneous manifestations were significantly higher than that in the group without cutaneous manifestations.Conversely,the incidence of abdominal pain(p=0.013)in the group without cutaneous manifestations was significantly higher than that in the group with cutaneous manifestations.For laboratory examinations,the level of serum ferritin(p<0.001)and lactic dehydrogenase(p<0.001)in the group with cutaneous manifestations were significantly higher than that in the group without cutaneous manifestations,and the white blood count(p=0.002)in the group without cutaneous manifestations was significantly higher than that in the group with cutaneous manifestations.The proportion of female patients(p=0.016)in the group with PPEs were significantly higher than that in the group with evanescent rash.There was no statistic difference in other clinical manifestations or laboratory examinations between the group with evanescent rash and the group with PPEs.Fifty-six patients with AOSD were included in this follow-up.The mean age of the patients was 39.2 ± 14.2 years,and 37 patients were female.Twenty-three patients had experienced at least one relapse of AOSD.The factors associated with relapse were increased levels of lactic dehydrogenase(OR=1.003,p=0.044)and creatine kinase-myocardial band(OR=1.111,p=0.032).ConclusionsThe incidence of arthralgia or arthritis and lymphadenopathy was higher in AOSD patients with cutaneous manifestations.Compared with those without cutaneous manifestations,the level of serum ferritin and lactate dehydrogenase was higher,while the level of white blood count was lower in AOSD patients with cutaneous manifestations.The incidence of PPEs was higher in female.AOSD patients with elevated serum levels of LDH and CK-MB were more likely to have relapses.