Clinical Review Of Kimura's Disease

Background:Kimura's disease(KD)is a chronic inflammatory disease which is usually manifested as a long-term and easy to repeat subcutaneous soft tissue mass.And the cause is not yet clear.The incidence of KD is low.During the recent 20 years,the related cases have been reported nearly a thousand cases.It is easy to occur in the Asian population.Due to its lacking of specific clinical manifestations,it is pretty easy to be misdiagnosed.At present,the treatment methods commonly used in Kimura's disease include surgical resection,glucocorticoid therapy,chemotherapy,local radiotherapy.But all of them can't avoid the recurrence.And the researches about combination therapy are rarely.So the optimal choice of treatment is still undetermined.Objective:Through the analysis of the relevant literature,summed up the clinical characteristics,diagnosis and treatment points of kimura's disease.And compare the prognosis of the different treatment programs.Thereby deepening the clinician understanding of KD for reducing the rate of misdiagnosis and missed diagnosis and seeking the optimal treatment for reducing the recurrence rate of KD.Methods:Retrieved from January 2000 to March 2017 in the public published KD cases literature at home and abroad.According to the inclusion criteria and excluding the standard screening related cases.The basic data,clinical manifestations,related complications,laboratory tests,imaging examination,pathological examination and follow-up of the selected cases were summarized.Results:Of the screened 297 patients,including 244 males and 53 females.THe male to female ratio of 4.6: 1.Age of onset ranges 6months-75 years and the mean age is 34.2 years.Clinical duration ranges1 week to 45 years.285 cases are Asians.Among the 297 patients,85.8%(254/297)were painless head and neck subcutaneous masses,followed by inguinal,upper limb and supraclavicular mass.3.4%(10/297)complicated with nephrotic syndrome.In the laboratory examination,77.9%(74/95)of patients had elevated serum Ig E level.86.7%(196/226)peripheral blood eosinophils increased and 11.7%(16/137)of urine proteins were positive.After the treatment which arrives partial remission(PR)or complete remission(CR),65.5%(19/29)serum Ig E levels decreased to normal range,27.6%(8/29)decreased by more than50% but not normal;90.2%(37/41)peripheral blood eosinophils dropped to normal levels.The recurrence rate of glucocorticoid treatment group was higher than that of surgery alone,local radiotherapy and combination therapy.There was no significant difference in the recurrence rate between radiotherapy and radiotherapy alone,but their rates are significant lower than that of glucocorticoid combined with immunosuppressive therapy.The recurrence rate of Surgical treatment was no significant difference with that of glucocorticoid combined with immunosuppressive therapy.The rates of surgery combined with glucocorticoid therapy and surgery combined with both glucocorticoid of local radiotherapy treatment were significantly lower than that of the single surgical treatment.Conclusions:1.Due to KD's rarity and lacking of specific clinical symptoms,KD is prone to be misdiagnosed.So for head and neck painless subcutaneous tumor patients,should be alert to the possibility of Kimura's disease.And KD's diagnosis is based on its pathological examination,combined with its clinical features and laboratory tests can be diagnosed.2.Peripheral blood eosinophils and serum Ig E levels can be considered as an index for KD's diagnostic process,efficacy evaluation and follow-up process.3.Kimura disease patients in the treatment and follow-up process should be concerned about the presence of nephrotic syndrome.Achieving early diagnosis and treatment by concerning the relevant clinical manifestations and taking the related tests should be an objective.4.On the basis of the preferred surgical treatment,the glucocorticoid treatment can reduce the postoperative recurrences.Patients who can not or refused to surgical treatment,the recommended treatment is the local radiotherapy.Both of glucocorticoid treatment alone and glucocorticoid combined with immunosuppressive therapy are not recommended.