PMID- 28375199
OWN - NLM
STAT- MEDLINE
DCOM- 20180703
LR  - 20180703
IS  - 2184-8777 (Electronic)
IS  - 0303-464X (Linking)
VI  - 42
IP  - 3
DP  - 2017 Jul-Sep
TI  - Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset 
      systemic lupus erythematosus patients: a multicenter study.
PG  - 250-255
AB  - OBJECTIVE: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal 
      necrolysis (TEN) in a large population of childhood-onset systemic lupus 
      erythematosus (cSLE) patients. METHODS: Multicenter study including 852 cSLE 
      patients followed in Pediatric Rheumatology centers in Sao Paulo, Brazil. SJS was 
      defined as epidermal detachment below 10% of body surface area (BSA), overlap 
      SJS-TEN 10-30% and TEN greater than 30% of BSA. RESULTS: SJS and TEN was observed 
      in 5/852 (0.6%) cSLE female patients, three patients were classified as SJS and 
      two patients were classified as overlap SJS-TEN; TEN was not observed. The mean 
      duration of SJS and overlap SJS-TEN was 15 days (range 7-22) and antibiotics 
      induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was 
      observed in two cSLE patients, nephritis in two and neuropsychiatric involvement 
      and conjunctivitis were observed respectively in one patient. Hematological 
      involvement included lymphopenia in four, leucopenia in three and 
      thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30). 
      Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the 
      presence of anti-dsDNA autoantibody in two patients. One patient had lupus 
      anticoagulant and another one had anticardiolipin IgG. All patients were treated 
      with steroids and four needed additional treatment such as intravenous 
      immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and 
      intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE 
      patients. Two patients required intensive care and death was observed in one 
      patient. CONCLUSION: Our study identified SJS and overlap SJS-TEN as rare 
      manifestations of active cSLE associated with severe multisystemic disease, with 
      potentially lethal outcome.
FAU - Sakamoto, Ana Paula
AU  - Sakamoto AP
AD  - Universidade Federal de Sao Paulo (UNIFESP).
FAU - Silva, Clovis Artur
AU  - Silva CA
AD  - Faculdade de Medicina da Universidade de Sao Paulo (USP).
FAU - Saad-Magalhaes, Claudia
AU  - Saad-Magalhaes C
AD  - Sao Paulo State University (UNESP) - Faculdade de Medicina de Botucatu.
FAU - Alencar, Aline Nicacio
AU  - Alencar AN
AD  - Universidade Federal de Sao Paulo (UNIFESP).
FAU - Pereira, Rosa Maria Rodrigues
AU  - Pereira RMR
AD  - Faculdade de Medicina da Universidade de Sao Paulo (USP).
FAU - Kozu, Katia
AU  - Kozu K
AD  - Faculdade de Medicina da Universidade de Sao Paulo (USP).
FAU - Barbosa, Cassia Maria Passarelli Lupoli
AU  - Barbosa CMPL
AD  - Hospital Infantil Darcy Vargas.
FAU - Terreri, Maria Teresa
AU  - Terreri MT
AD  - Universidade Federal de Sao Paulo (UNIFESP).
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
TT  - Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset 
      systemic lupus erythematosus patients: a multicenter study.
PL  - Portugal
TA  - Acta Reumatol Port
JT  - Acta reumatologica portuguesa
JID - 0431702
SB  - IM
MH  - Adolescent
MH  - Age of Onset
MH  - Child
MH  - Child, Preschool
MH  - Cohort Studies
MH  - Female
MH  - Humans
MH  - Lupus Erythematosus, Systemic/*complications
MH  - Retrospective Studies
MH  - Stevens-Johnson Syndrome/*complications
EDAT- 2017/04/05 06:00
MHDA- 2018/07/04 06:00
CRDT- 2017/04/05 06:00
PHST- 2017/04/05 06:00 [pubmed]
PHST- 2018/07/04 06:00 [medline]
PHST- 2017/04/05 06:00 [entrez]
AID - AO160252 [pii]
PST - ppublish
SO  - Acta Reumatol Port. 2017 Jul-Sep;42(3):250-255.