A Retrospective Study Of 104 Patients With Pemphigus

Part ? A Review of 59 Pemphigus Vulgaris Cases Treated with Pulsed Methylprednisolone and CyclophosphamideObjective:We retrospectively investigated a total of 59 patients with pemphigus vulgaris.The parameters including age at onset,sex,diagnostic methods,hospitalizations,side-effects with different treatments,the therapeutic features and duration of follow up were evaluated.The aim of the current study was to assess the epidemiological,clinical and therapeutic features,adverse events,prognosis of patients with pemphigus vulgaris,and to determine the efficacy and safety of pulsed methylprednisolone and cyclophosphamide(PPC)therapy.Methods:The data for the study were retrieved from the medical records of 59 patients with pemphigus vulgaris who had been hospitalized in the Shandong Provincial Hospital for Skin Diseases during the period 2008?2013.Epidemiological,clinical and therapeutic features were re-evaluated and statistically analysed.Of the 59 enrolled patients,30 cases were treated with conventional systemic corticosteroids and cyclophosphamide(PC group),while 29 cases had received our modified pulsed methylprednisolone and cyclophosphamide therapy(PPC group)if uncontrolled with conventional corticosteroids at dose above 150?200mg/day for 3?5d.The pulsed intravenous methylprednisolone was given respectively 750mg,500mg,250mg prednisolone equivalent on three consecutive days.In addition,all patients received 2?3mg/(kg d)i.v.cyclophosphamide in combination with the above-mentioned regimen.The mean initial dose,maximum control dose,total dose before reducing,hospitalized total dose of prednisolone,time to initial control and hospitalized time were recorded in each group.The data were analyzed using SPSS version 19.0 software.Results:1.Demographics:Of these 59 patients,36(61.02%)were males and 23(38.98%)were females.The male-to-female ratio was 1.57/1.A statistically significant difference in the incidence rate of pemphigus vulgaris was observed between males and females(P<0.05).However,all age groups exhibited no statistical differences in incidence rate of pemphigus vulgaris between males and females.The mean age at the onset of the disease was 46.90�11.77 years(range,21?75years),most occurred in those aged 31-50 years.The duration time of the disease ranged 0.5?72 months,and the mean was 9.08 months.At the same time,we found that 50 cases of patients(84.75%)presented the mucocutaneous type in lesion distribution,and 9 cases(15.25%)had only mucosal lesions.2.Laboratory tsets:The levels of autoantibodies against Dsg-1 and Dsg3 in patient sera were measured by specific ELISA,the positive rates of Dsg-1 and Dsg3 are 88.89%and 69.44%respectively.All 59 cases showed DIF positive and 4 cases IIF 1:10(-).The initial antibody titer ranged from negative to 1:1280 in PC group,and negative to 1:2560 in PPC group.IIF demonstrated no significant statistic difference between the two groups(P>0.05).In addition mean IIF values decreased significantly between the pre and after therapy in both groups(P<0.05).3.Therapy and efficacy:We observed statistical differences in the maximum control dose,total dose before reducing,hospitalized total dose of steroid and hospitalized time between the both groups in the moderate subgroup(P<0.05),but not in initial dose and time to initial control(P>0.05).Moderate patients of PPC group usually needed higher doses of steroid.However,in severe group,statistical differences were observed in the maximum control dose,total dose before reducing,hospitalized total dose of steroid and time to initial control(P<0.05),but not in hospitalized time(P>0.05).Severe patients of PPC group also needed higher doses of steroid,while needing shorter time to control disease.At discharge,the rates of clinical remission were 20.00%and 48.28%in PC and PPC group,demonstrating significant statistic difference(P<0.05).At 2-year follow up,the rates of complete remission on therapy were 17.86 and 28.57%,complete remission off therapy 14.29%and 17.86%,recurrence 17.86%and 10.71%,mortality 10.71%and 0 in PC and PPC groups,respectively.There was no statistical differences in the above four observation items between the two groups(P>0.05).4.PPC combined IVIG therapy:Statistical difference was observed in the hospitalized total dose of steroid(P<0.05),but not in maximum control dose,total dose before reducing,time to initial control and hospitalized time(P>0.05)between the PPC group and PPC combined IVIG group.5.Adverse effects:Adverse events were recorded in 40.00%of PC group cases and in 72.41%of PPC group cases.There was greatly statistical difference between the two groups(P<0.05).Conclusions:1.The pemphigus vulgaris is more frequent in the middle-aged persons,especially between the third to fifth decade of life.We found that the incidence of pemphigus vulgaris in males was 1.57-fold higher than females.2.The clinical diagnosis of pemphigus vulgaris can be confirmed by histopathologic and immunofluorescence findings,and enzymelinked immunosorbent assay.The titer of IIF is parallel to the severnty of the disease,but it can not be used as the only index to judge the severity of the disease.3.For moderate and severe pemphigus vulgaris patients,the timely use of this modified pulsed methylprednisolone therapy can shorten the time to initial control and obtain obvious short-term effect.In terms of long-term efficacy,this pulse therapy has not shown its advantages over traditional regimen.Pulsed therapy,usually needing higher doses of steroid,can be used as a treatment option for moderate and severe patients with pemphigus vulgaris who have failed to respond to conventional therapy.4.IVIG is a safe and effective treatment for pemphigus vulgaris,which can control the severity of refractory pemphigus vulgaris without increasing the total amount of steroid.But because of its high cost,IVIG can not be used as a routine therapy in China.5.The incidence of adverse events was associated with the dose of corticosteroids.Patients in PPC group presented higher frequencies of side-effects.As a result,during the use of corticosteroids,it is necessary to monitor closely to prevent the occurrence of adverse eventsPart ? A Review of 45 cases of Pemphigus FoliaceusObjective:We conducted a retrospective analysis of 45 patients diagnosed with pemphigus foliaceus(PF)between 2008 and 2013.The patient characteristics,clinical manifestations,treatment regimens,adverse reactions and therapeutic effects were studied.We compared PV and PF patients regarding corticosteroids dose,curative effect and prognosis.Theobjective of this study was to further deepen the understanding of PV and PF,and provide more reference for the diagnosis and treatment of pemphigus.Methods:Searching Electronic Medical Record System of Shandong Provincial Hospital for Skin Diseases with "Pemphigus" as the key word.The datas for the study were retrieved from the medical records of 45 patients with PF who had been hospitalized during the period 2008-2013.The general condition,clinical manifestation,treatment regimen,adverse reaction and therapeutic effect were recorded.Selecting 59 patients with PV in the first part of this study,the dosage,efficacy and prognosis of the two groups were compared and analyzed.The datas were analyzed using SPSS version 19.0 software.Results:1.Demographics:Of these 45 patients,21(46.67%)were males and 24(53.33%)were females.The male-to-female ratio was 1/1.14.There was no statistically significant difference in the incidence rate of PF between males and females(P>0.05).The youngest onset age was 5 years old,the oldest was 77 years old,and the average age of onset was 47.53�15.46 years,most occurred in those aged 41-60 years.The duration time of the disease ranged 0.5-36 months,and the mean was 11.94 months.There were 5 cases with mucosal lesions,accounting for 11.11%.2.Comparison of treatment between PF and PV patients:We observed statistical differences in the initial dose,maximum control dose,time to initial control and hospitalized time between the both groups(P<0.05),but not in hospitalized total dose of steroid(P>0.05).The initial and maximum dose of steroid of PF were less than those of PV,and the time needed to control the disease was shorter than that of PV,hospitalization days were also few.3.Adjuvant drugs:6 patients with mild PF were treated with oral tripterygium wilfordii,tetracycline and nicotinamide,whose disease was controlled within 7-10 days.The remaining 39 cases of PF were divided into corticosteroids group and corticosteroids combined with CTX group.We observed statistical differences in the hospitalized total dose of steroid between the both groups(P<0.05),but not in initial dose,maximum control dose and time to initial control(P>0.05).Corticosteroids combined with CTX group needed fewer dose of steroid.4.Comparison of curative effect between PF and PV patients:At discharge,the rate of clinical remission were respectively 64.44%and 33.90%in PF and PV group,demonstrating significant statistic difference(P<0.05).The clinical remission rate of PF group was higher than that of PV group.At 2-year follow up,There was no statistical differences in the rate of remissions,recurrence and mortalitybetween the two groups(P>0.05).Conclusions:1.The pemphigus foliaceus is more frequent in the middle-aged persons,especially between the fourth to sixth decade of life.We found a similar incidence between men and women.2.The type of pemphigus is one of the factors that affect steroid dosage and therapeutic effect.The initial dosage and maximum control amount of steroid required by PF patients to control the disease were smaller than those of PV.PF patients have quicker effect and shorter hospital stay than that of PV patients.3.For some mild PF patients,Tripterygium wilfordii,tetracycline and nicotinamide triple therapy are available.CTX takes effect slowly,it can not shorten the time to control the disease,but having steroid-sparing action to decrease the total usage dose of corticosteroids.4.The prognosis of both types of pemphigus has improved considerably since the introduction of corticosteroids.Our study suggests that PF and PV may share the same clinical course.There is no difference in the rate of remission,recurrence and mortality.