Churg?Strauss syndrome (CSS) in a patient receiving pranlukast

Abstract

Pranlukast is a cysteinyl leukotriene receptor I antagonist (LTRAs) approved for treatment of asthma in Japan since 1995. Compared to other LTRAs, such as zafilukast and montelukast, only few cases with Churg-Strauss syndrome (CSS) have been reported in association with treatment with pranlukast. We describe a 17-year-old Japanese male patient who developed CSS with a 13 month history of mild asthma receiving pranlukast for 11 months without systemic and/or inhaled corticosteroid administration prior to development of CSS. From the aspect of temporal relationship between treatment with pranlukast and development of CSS, a direct induction of CSS by pranlukast is suggested in our case.

Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

“…It has been reported that childhood CSS is rare, and the features of the disease in children have not been fully understood (Ikemoto et al 2001;Kobayashi et al 2003;Boyer et al 2006;Zwerina et al 2008;Kawakami and Soma 2009;Mutsaers et al 2009;Domircin et al 2010;Salerno et al 2010). Regarding serum ANCA positivity, relatively low frequency in children with CSS, about 25%, were reported to be positive for serum ANCA (Zwerina et al 2008), which is different from that of adults with CSS.…”

“…It has been postulated that the occurrence of CSS in some patients treated with leukotriene modifiers may be related to a residual underlying vasculitic syndrome (Boyer et al 2006;Salerno et al 2010). However, some case reports have suggested clear causal association between the use of leukotriene modifiers and CSS (Kobayashi et al 2003;Kanda et al 2010). In our patient, CSS occurred following significant hypereosinophilia, which developed after a 6-month course of pranlukast treatment, which is similar to a previous report by Kobayashi et al (2003).…”

“…Since massive use of corticosteroids increases the risk of intestinal perforation (Venditti et al 2011), we suggest that an aggressive immunosuppressive regimen including Tac is beneficial for children with refractory CSS like our case, although further studies are needed. Some previous reports have suggested a potentially causal relationship between treatment with leukotriene modifiers and the onset of CSS (Kobayashi et al 2003;Boyer et al 2006;Kanda et al 2010;Salerno et al 2010,). It has been postulated that the occurrence of CSS in some patients treated with leukotriene modifiers may be related to a residual underlying vasculitic syndrome (Boyer et al 2006;Salerno et al 2010).…”

“…However, some case reports have suggested clear causal association between the use of leukotriene modifiers and CSS (Kobayashi et al 2003;Kanda et al 2010). In our patient, CSS occurred following significant hypereosinophilia, which developed after a 6-month course of pranlukast treatment, which is similar to a previous report by Kobayashi et al (2003). From the clinical course of our patient, marked eosinophilia emerged 5 months after the start of pranlukast, as described in the case presentation.…”

“…Since CSS is an uncommon disease entity in children, the treatment of pediatric patients with CSS, especially in severe cases, remains challenging (Ikemoto et al 2001;Boyer et al 2006;Zwerina et al 2008;Domircin et al 2010). Regarding the etiology of CSS, some case reports have suggested an association between the onset of CSS and the use of leukotriene modifiers for the treatment of pre-existing asthma (Kobayashi et al 2003;Boyer et al 2006;Salerno et al 2010), although this remains speculative (Harrold 2007). We experienced an informative case of severe CSS with a relatively short-term history of asthma treated with a leukotriene modifier, pranlukast.…”