After plasmapheresis for SJS/TEN be

After plasmapheresis for SJS/TEN became eligible for coverage
by health insurance in 2006, the available options of treatment
modalities have been changing in TEN. Therefore, we separated the
cases by the date of each 7 years before and after this change
(2000e2006 and 2007e2013) and compared the treatment modalities
used and the mortality rates in these 2 periods. From 2000
to 2006, 22 cases of SJS and 17 cases of TEN were evaluated. From
2007 to 2013, 30 cases of SJS and 18 cases of TEN were evaluated.
Although steroid pulse therapy and the combination of IVIG therapy
(

Results (Indonesian) 1:

After plasmapheresis for SJS/TEN became eligible for coverageby health insurance in 2006, the available options of treatmentmodalities have been changing in TEN. Therefore, we separated thecases by the date of each 7 years before and after this change(2000e2006 and 2007e2013) and compared the treatment modalitiesused and the mortality rates in these 2 periods. From 2000to 2006, 22 cases of SJS and 17 cases of TEN were evaluated. From2007 to 2013, 30 cases of SJS and 18 cases of TEN were evaluated.Although steroid pulse therapy and the combination of IVIG therapy(<2 g/kg) with corticosteroid therapy were the mainstreamuntil 2006, the frequency of cases treated with the combination ofplasmapheresis and corticosteroid therapy increased remarkablyafter 2007 (shown in Fig. 4).The mortality rates showed a remarkable decrease after 2007,compared with 2000e2006, from 4.5% to 0.0% in SJS and from23.5% to 5.6% in TEN, although the average SCORTEN scores weresomewhat elevated after 2007 (2.18 in 2000e2006 and 2.50 in2007e2013). We compared the predicted mortality rate of TENcases with the actual rate. Only a little difference was shown in2000e2006; the predicted rate was 23.9% (4.1 cases) and the actualrate was 23.5% (4 cases). However, it showed a relatively large gapin 2007e2013; the predicted rate was 26.5% (4.8 cases) and theactual rate was 5.6% (1 case). Furthermore, when comparing theaverage SCORTEN score of the non-deceased cases between the 2periods, it showed a relatively large increase from 1.69 to 2.47.DiscussionSJS and TEN are rare but life-threatening disorders. The mortalityrates for these conditions were recently reported to be 34% at1 year for SJS/TEN in Europe18 and 3% and 19% for SJS and TEN,respectively, in Japan.19 Recent studies have revealed new detailsabout the apoptotic pathways of keratinocytes and immunologicalchanges that are related to adverse drug reactions in these diseases.8,20e23 In addition to the direct cytotoxicity by the cytotoxic Tcells (CTLs), several soluble factors such as tumor necrosis factor-a,nitric oxide, soluble Fas ligand (sFasL), granulysin, annexin A1 arenow considered to mediate keratinocyte apoptosis. Abe et al. reportedthat peripheral blood mononuclear cells (PBMCs) from SJS/TEN patients secrete sFasL on stimulation with the causal drug. Inaddition, they demonstrated that patients sera induce apoptosis incultured keratinocytes, indicating that sFasL produced by PMBCsmay contribute to the pathogenesis of SJS/TEN.21 Chung et al.clarified that granulysin produced by CTLs or natural killer cellsconcentrations in the blister fluids of SJS/TEN skin lesions were twoto four orders of magnitude higher than perforin, granzyme B orsFasL concentrations, and depleting granulysin reduced the cytotoxicityof the keratinocytes. Furthermore, they showed that injectionof granulysin into mouse skin resulted in featuresmimicking SJS-TEN.22 Recently Saito et al. revealed the contributionof annexin A1 in keratinocyte necroptosis of SJS/TEN. Depletion ofannexin A1 by a specific antibody diminished supernatant cytotoxicity.SJS/TEN keratinocytes expressed abundant formyl peptidereceptor 1, the receptor for annexin A1, whereas control keratinocytesdid not. They also showed that inhibition of necroptosiscompletely prevented SJS/TEN-like responses in a mouse model ofSJS/TEN.23There is no established therapy for SJS/TEN, although manytreatment modalities including corticosteroid, plasmapheresis, andIVIG have been used. The challenge remains that it is difficult toassess the efficacies of treatments for such serious and rare disordersin a large clinical randomized controlled trial (RCT).In this study, we presented the current clinical characteristicsand treatments of SJS and TEN in 87 patients treated in our 2hospitals to evaluate the usefulness of these treatmentsretrospectively.The ages of patients with SJS and TEN were widely distributedfrom young to older. The major causative drugs were antibiotics,anticonvulsants, NSAIDs, and cold medicines. The predominance ofthese drugs in causing the diseases seems to have been unchangedsince Aihara et al. analyzed 269 cases of SJS and 287 cases of TENthat were reported from 1981 to 1997 in Japan.24 However, in ourstudy, anticonvulsants were more frequently the causative drugsthan has been previously reported in SJS. This might be related tothe fact that in recent years, anticonvulsants have been used notonly for convulsions but also for other diseases, such as neurogenicpain and bipolar disorder.In addition to the severe skin symptoms, many organ involvementswere observed. The organs most commonly involvedwere liver and kidneys. However, while less common than hepatitisand renal dysfunction, respiratory and gastro-intestinal disorderswere severe conditions often resulting in fatality. In addition tomulti-organ involvement, another major problem in the clinicalcourse was secondary infections, especially sepsis.As for treatments, systemic corticosteroid therapy was mainlyused both in SJS and TEN in Japan.25 The use of corticosteroids isbased on the idea that corticosteroids effectively suppress anexcessive immune response. While their use is still controversial,18,26 recent studies have suggested them to be a valid treatment