| BackgroundAdult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease with intermittent high fever,rash,arthritis/arthralgia,hepatosplenomegaly,and lymph node enlargement,which are the primary clinical manifestations.The incidence of AOSD is low,and there are few related studies.The etiology and pathogenesis of AOSD still need to be completely clarified.The clinical manifestations of AOSD are diverse,and the lack of specific laboratory indicators often leads to delayed diagnosis or misdiagnosis.The typical rash is widely recognized as one of the leading diagnostic indicators of AOSD,characterized by transient erythema and wheal-like rash,without obvious pruritus,often accompanied by fever,and the rash subsides after the fever is relieved.In recent years,studies have also found various atypical rashes associated with AOSD,mainly manifested as persistent pruritic rashes,which do not subside with fever,can concomitant with typical rashes,and can also be the only skin manifestation of AOSD.AOSD has a variety of conditions and courses,which is highly heterogeneous.Some patients achieve long-term remission after treatment,while others have recurrent attacks,and in severe cases,they can develop life-threatening complications,such as hemophagocytic syndrome(HPS).Early diagnosis and timely treatment can improve the clinical prognosis.ObjectiveThe clinical data of AOSD patients were statistically analyzed to summarise the clinical features of the disease;we deeply expolred the correlation between the rash of AOSD and other clinical features,as well as the relevant prognostic factors,which provide a clinical reference for early diagnosis,early intervention and prognosis of the disease.MethodPatients,hospitalized at Qilu Hospital of Shandong University and diagnosed with AOSD during January 2016 and November 2022,were included.All patients met the Yamaguchi diagnostic criteria.General data,clinical manifestations,laboratory indices,imaging examinations,and pathological outcomes of the patients were collected and statistically analyzed.All patients were followed up to determine disease outcomes.Patients were divided into the group with rash and the group without rash according to the presence or absence of rash,and the group with rash was further divided into the group with typical rash and the group with atypical rash to analyze the clinical characteristics and prognosis of patients in different groups;patients were divided into two groups according to whether they developed hemophagocytic syndrome for comparative analysis between groups;patients were divided into the remission group and the relapse group according to the presence or absence of relapse for comparative analysis between groups.The data were analyzed using SPSS25.0 statistical software.The measurement data were first tested for normality and described using the mean±standard deviation if they conformed to a normal distribution,and the differences between comparative groups were compared using the independent samples t-test;the skewed distribution data were described using the median and interquartile spacing,and the differences between comparative groups were compared using the Mann-Whitney U-test.Count data were described by frequency(percentage),and differences between groups were compared dichotomously using the chi-square test or Fisher’s exact test;factors associated with the development of the hemophagocytic syndrome and relapse in AOSD were analyzed by binary logistic regression,with P<0.05 indicating a statistically significant difference.Objective1.68 cases of AOSD were included in this study,with a median age of onset of 33.5(26.00,46.75)years,11(16.2%)males and 57(83.8%)females,with a male-to-female ratio of 1:5.The main clinical manifestations of the 68 patients were fever(100%),rash(86.8%),arthritis/arthralgia(79.4%),sore throat(67.6%)and lymph node enlargement(58.8%).Other clinical manifestations were myalgia(48.5%),splenomegaly(45.6%),pericardial effusion(23.5%),pleural effusion(16.2%),weight loss(17.6%)and hepatomegaly(10.3%).2.Abnormal laboratory indicators in 68 patients with AOSD included elevated peripheral blood leukocytes(88.2%),neutrophil ratio>80%(80.9%),increased erythrocyte sedimentation rate(100%),elevated C-reactive protein(94.1%),elevated serum ferritin(98.5%),elevated lactate dehydrogenase(98.5%),elevated alanine aminotransferase(54.4%),elevated aspartate aminotransferase(52.9%),anemia(39.7%)and thrombocytosis(23.5%).3.A histopathological skin examination was performed on 22 of the 59 patients with a rash.Eight cases were taken at the site of a typical rash of transient origin,and 14 were taken at the site of a persistent atypical rash.Eight cases of the typical rash showed an approximately normal epidermis with dilated and congested superficial dermal vessels and a slight perivascular infiltration of neutrophils and lymphocytes.Fourteen cases of the atypical rash showed a superficial epidermis.In 14 cases of atypical rash,the histopathology showed scattered dyskeratotic cells,some arranged in a "bead-like" pattern,and lymphocytic and neutrophilic infiltration around the blood vessels in the superficial dermis,of which 11 cases also showed a small number of eosinophilic infiltrates.4.The proportion of female patients,the incidence of arthritis/arthralgia,and peripheral blood lactate dehydrogenase levels were statistically significantly higher in the group with rash than in the group without rash(P<0.05).5.The mean number of days in hospital,C-reactive protein,D-dimer,eosinophil count,aspartate aminotransferase,lactate dehydrogenase,and ferritin levels were all statistically higher in the atypical rash group than in the typical rash group(P<0.05).6.The hemophagocytic syndrome occurred in 6(8.8%)patients with AOSD,of which 1(1.5%)died.Patients who developed haemophagocytic syndrome had higher lactate dehydrogenase levels than those who did not develop the haemophagocytic syndrome,and the difference was statistically significant(P=0.038).Binary logistic regression analysis showed that elevated peripheral blood lactate dehydrogenase(OR=1.004,P=0.014)and elevated C-reactive protein(OR=1.013,P=0.022)were risk factors for AOSD complicating hemophagocytic syndrome.7.Patients were followed up through inpatient information,outpatient visit information,and telephone callbacks.18 patients were lost,and follow-up information was obtained from 49 patients,of which 34 patients achieved complete remission,13 patients relapsed,and 2 patients had partial remission.Excluding the 2 cases in partial remission,the remaining 47 patients were divided into a complete remission group and a relapse group,and the clinical characteristics of the 2 groups were compared.The results showed that the proportion of atypical rash was significantly higher in the relapse group of AOSD patients(92.3%)than in the complete remission group(55.9%),and the difference was statistically significant(p=0.036).The platelet count in patients in the relapse group(250.23±100.31)was lower than that in the complete remission group(344.71 ± 143.54),with a statistically significant difference(P=0.035).Binary logistic regression showed that atypical rash(OR=14.714,P=0.032)was a risk factor for AOSD recurrence,and elevated PLT(OR=0.991,P=0.042)was a protective factor for AOSD relapse.Conclusion1.AOSD is usually observed in young women and has various clinical manifestations,mainly presents with fever,rash,arthritis/arthralgia,sore throat,and lymph node enlargement.2.The atypical rash of AOSD has unique histopathological features,often manifesting as"bead-like" arrangement of dyskeratotic cells in superficial epidermis,and early recognition of the atypical rash is of great significance.3.The incidence of arthritis/arthralgia and serum lactate dehydrogenase levels are higher in patients with AOSD with the rash.4.C-reactive protein,ferritin,serum lactate dehydrogenase,eosinophil count,aspartate aminotransferase,and D-dimer levels were higher in patients with AOSD with atypical rash than in those with typical rash.5.The hemophagocytic syndrome complicates 8.8%of patients with AOSD.Elevated peripheral blood lactate dehydrogenase and C-reactive protein levels may be risk factors for AOSD complicating hemophagocytic syndrome.6.Atypical rash and reduced platelet levels may be risk factors for the relapse of AOSD. |
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