Status of Plasmapheresis for the Treatment of Toxic Epidermal Necrolysis in Japan

Yamada, Hiromichi; Takamori, Kenji

doi:10.1111/j.1744-9987.2008.00609.x

Cited by 46 publications

(42 citation statements)

References 19 publications

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“…Reau et al noted that the cell death receptor CD95 (Fas) and its ligand (Fas-L) have been implicated in bile duct injury. 2 Similarly, elevated levels of Fas-L have been found in TEN patients, 16 and a case series showed resolution of the TEN mucosal damage once plasmapheresis was initiated, correlating with removal of Fas-L from the serum. 17 Its use in patients with isolated VBDS is limited.…”

Section: Discussionmentioning

confidence: 99%

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

White¹,

Appleman²

2014

Pediatrics

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Vanishing bile duct syndrome (VBDS) is a rare disorder characterized by loss of interlobular bile ducts and progressive worsening cholestasis. The acute presentation of this disease is typically associated with a drug hypersensitivity and Stevens-Johnson syndrome/toxic epidermal necrolysis (TEN). The mainstay of treatment has been ursodeoxycholic acid with mixed results from immunosuppressive regimens. Anti–tumor necrosis factor-α and plasmapheresis have been speculated to be of potential benefit. It is hoped that early identification and intervention in VBDS secondary to Stevens-Johnson syndrome/TEN with continued reporting will lead to better regimens and outcomes. Our case report details the first reported use of infliximab and plasmapheresis, in addition to steroids, in a patient with VBDS secondary to TEN, as well as a literature review that supports a mechanism for why these modalities could be effective treatments. Unfortunately, our patient died, and the use of these therapies had an unclear benefit on his liver and skin disease. We hope that additional work can be published to confirm or refute their utility in the treatment of these diseases.

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Section: Discussionmentioning

confidence: 99%

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

White¹,

Appleman²

2014

Pediatrics

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“…Plasmapheresis is valued for its rapid results, rare side effects, and efficiency in treating patients with TEN refractory to steroids or IVIg. 8 The results of our own experience 11 and the findings in other case reports, 12 case series, 13 and retrospective cohort studies 8 have consistently indicated clinical benefits with plasmapheresis, and additional research on this method has been recommended. 1,7 Regardless of the therapeutic option chosen, a comprehensive team approach that emphasizes supportive care is essential for any patient with TEN.…”

Section: Treatmentmentioning

confidence: 99%

“…Only a few investigators have examined this topic in detail. A large-scale (47 patients) study of plasmapheresis treatment and TEN by Yamada et al 8 revealed that even a low number of cycles (mean, 3.1) had good efficacy (80.9%), and mortality (23.4%) was lower than predicted in patients with the most severe and resistant form of SJS or TEN. A major difficulty limiting further study of this treatment method is the low incidence of TEN, a situation that results in few patients who could potentially be included in prospective studies.…”

Section: Painmentioning

confidence: 99%

“…Overall mortality rates are about 25% to 35%. 1 In a large retrospective study (47 patients, 11 deaths) of TEN by Yamada et al, 8 sepsis (64%) was the most common cause of death. Other causes included disseminated intravascular coagulation (27%) and liver failure (11%), but the percentages may vary.…”

Section: Pathophysiology and Clinical Manifestationsmentioning

confidence: 99%

“…The lack of improvement after 48 hours of treatment with corticosteroid, rapid progression of the disease, and the need for a fastacting treatment prompted the decision to immediately begin therapeutic plasma exchange (TPE), which has been used to treat patients with steroid-refractory TEN who required rapid improvement. 8,11 An initial 5-cycle course of TPE was performed (days 1, 2, 4, 5, and 6), and 30 g of IVIg was given after the last cycle. Empirical broad-spectrum antibiotic therapy (ceftriaxone) was started before the patient was admitted to the ICU, and regular electrolyte and fluid replacements were maintained throughout the ICU stay.…”

Section: Case Descriptionmentioning

confidence: 99%

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Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Seczyńska

Nowak²,

Sega³

et al. 2013

Critical Care Nurse

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L yell disease, commonly referred to as toxic epidermal necrolysis (TEN), is a rare, lifethreatening condition probably related to drug hypersensitivity; the mortality rate is approximately 30%.1 The disease is characterized by an initial pruritic, erythematous rash followed by the appearance of bullae, which subsequently lead to ulceration and sloughing of the underlying epidermal base similar to physiological damages caused by burns. Moreover, TEN causes crusting, desquamation, and ulceration of the mucous membranes in areas such as the oral, ocular, and genital mucosa.2 Bleeding from internal mucous membranes, especially those in the gastrointestinal and respiratory tracts, can progress to hypovolemia and lead to death. TEN is often accompanied by ocular manifestations ranging from acute conjunctivitis to corneal abrasion and, in the most severe cases, corneal ulceration.3 These changes can lead to permanent loss of vision. Feature A patient with severe toxic epidermal necrolysis underwent 2 cycles of therapeutic plasma exchange and received specialized wound care for widespread skin damage of more than 80% of his body surface area. Extensive involvement of mucous membranes, including the conjunctivas and the oropharyngeal cavity, and damage of his genitourinary organs required meticulous wound care. Daily care of injuries of tissues affected only in the most severe cases of toxic epidermal necrolysis was provided by an experienced intensive care unit nursing team. A meticulous supportive therapy regimen was a major contributing factor to this patient's remission. Supportive Therapy(Critical Care Nurse.

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Evaluation of Plasmapheresis vs Immunoglobulin as First Treatment After Ineffective Systemic Corticosteroid Therapy for Patients With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

et al. 2023

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ImportanceStevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse reactions, and patients with SJS/TEN frequently require intensive care. However, there is limited evidence on the clinical outcomes of immunomodulating therapy, including plasmapheresis and intravenous immunoglobulin (IVIG) in patients with SJS/TEN.ObjectiveTo compare clinical outcomes of patients with SJS/TEN who were treated with plasmapheresis first vs IVIG first after ineffective systemic corticosteroid therapy.Design, Setting, and ParticipantsThis retrospective cohort study used data from a national administrative claims database in Japan that included more than 1200 hospitals and was conducted from July 2010 to March 2019. Inpatients with SJS/TEN who received plasmapheresis and/or IVIG therapy after initiation of at least 1000 mg/d of methylprednisolone equivalent systemic corticosteroid therapy within 3 days of hospitalization were included. Data were analyzed from October 2020 to May 2021.ExposuresPatients who received IVIG or plasmapheresis therapy within 5 days after initiation of systemic corticosteroid therapy were included in the IVIG- and plasmapheresis-first groups, respectively.Main Outcomes and MeasuresIn-hospital mortality, length of hospital stay, and medical costs.ResultsOf 1215 patients with SJS/TEN who had received at least 1000 mg/d of methylprednisolone equivalent within 3 days of hospitalization, 53 and 213 patients (mean [SD] age, 56.7 [20.2] years; 152 [57.1%] women) were included in the plasmapheresis- and IVIG-first groups, respectively. Propensity-score overlap weighting showed no significant difference in inpatient mortality rates between the plasmapheresis- and IVIG-first groups (18.3% vs 19.5%; odds ratio, 0.93; 95% CI, 0.38–2.23; P = .86). Compared with the IVIG-first group, the plasmapheresis-first group had a longer hospital stay (45.3 vs 32.8 days; difference, 12.5 days; 95% CI, 0.4–24.5 d; P = .04) and higher medical costs (US $34 262 vs $23 054; difference, US $11 207; 95% CI, $2789–$19 626; P = .009).Conclusions and RelevanceThis nationwide retrospective cohort study found no significant benefit to administering plasmapheresis therapy first instead of IVIG first after ineffective systemic corticosteroid treatment in patients with SJS/TEN. However, medical costs and length of hospital stay were greater for the plasmapheresis-first group.

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Status of Plasmapheresis for the Treatment of Toxic Epidermal Necrolysis in Japan

Cited by 46 publications

References 19 publications

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Evaluation of Plasmapheresis vs Immunoglobulin as First Treatment After Ineffective Systemic Corticosteroid Therapy for Patients With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

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