Steven-Johnsons Syndrome

We do not know why Joe had the reaction know as SJS it could of been triggered by a Drug or virus.

Although the first bad reaction was thought to be Drug related as he started a new drug it was probably that over the two years and his final reaction was viral triggered with his body fighting against other problems.

Most patients survive SJS but do not underestimate the effort or the medical care required.

SJS and lung failure due to on going issues after Joe had complete organ failure with serious damage to his lungs & kidneys was the start of what two years later became too much for him.

See our medical facts page and other links www.joeway.co.uk/info/stevens-johnson.aspx 

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April 2009 up dated news

NeLM news service

Drugs associated with Stevens-Johnson syndrome in children,Reference: Pediatrics 2009; 123: e297-e30, Source: Pediatrics, Date published: 09/02/2009 15:54

by: Jim Glare 

Analysis of two previous case-control studies confirms the association between anti-infective sulphonamides, phenobarbital, carbamazepine, and lamotrigine, and an increased risk of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children. The data also suggest a possible associate with paracetamol. 

The mucocutaneous reactions SJS and TEN are variants on a spectrum; they are rare (estimated incidence 1 to 2 cases per million population per year) but serious: mortality in TEN may be over 40%, and many survivors will have serious sequelae. By definition, skin detachment over <10% of the body surface area define SJS, whereas >30% defines TEN. Between 10% and 30% is termed SJS/TEN-overlap. In adults it is estimated that around 70% of cases are adverse drug reactions, although other non-medication factors may also be involved. While a number of drugs have been implicated in adult cases, there is limited information on those most likely to be associated with the condition in children. The authors of this study extracted data relating to children (age <15) from the two largest available case-control studies of SJS/TEN (SCAR, n=368, and EuroSCAR, n=383) to identify those drugs most likely to be implicated in childhood cases. 

They selected children from the pooled studies, and matched each case with up to three controls by age, gender, and country. Controls were selected from patients hospitalised for acute conditions not expected to be drug-induced (e.g. infection, trauma, etc.). Drug exposure was assessed for the 7 days (3 weeks for phenobarbital) preceding the probably day of disease onset. A possible spurious association with drugs given to treat the early symptoms was controlled for by repeating the analysis for suspect drugs with an onset day three days earlier. 

The pooled data provided 80 cases aged <15, and 216 controls; 26 were classified as having SJS, 34% TEN, and 40% SJS/TEN-overlap. Median duration of hospitalisation was 17 days, and six cases died. Cases were much more likely than controls to have taken drugs in the period before onset: only 7.5% of cases took no drug in the week before onset compared to 25% of controls, and mean numbers of drugs to which they were exposure were 2.4 for cases and 0.75 for controls. In a univariate analysis, anti-infective sulphonamides, phenobarbital, lamotrigine, and carbamazepine were strongly associated with SJS/TEN.  Statistically significant associations were also found for valproate (remaining statistically significant when patients taking other anticonvulsants were excluded), NSAID as a group, and paracetamol. The association for paracetamol remained statistically significant when the analysis was adjusted to minimise cause and effect confusion. 

The authors conclude that their data support the hypothesis that medication use is the major risk factor for SJS/TEN in children, and confirm an association between four previously suspected drugs / drug groups (anti-infective sulphonamides, phenobarbital, carbamazepine, and lamotrigine) and significantly increased risk of SJS/TEN in children. They also identified an increased risk associated with valproate, paracetamol, and NSAID as a group: this association could not be completely explained by bias due to their use as symptomatic treatment for a condition precipitated by one of the highly suspected drugs. There were insufficient data to confirm or refute associations with other drugs suggested of being causative: 30% of cases were not exposed to a highly suspected drug, and 7.5% were not exposed to any drug within the preceding week, suggesting a contribution from other non-drug risk factors. 

© National electronic Library for Medicines 2007 Guy's and St Thomas & UKMI