DISCUSSION
The present report describes an adult patient with DRESS syndrome and liver failure treated with OLT. Liver failure in the setting of DRESS syndrome is quite rare. Few case reports presented patients with DRESS syndrome and high level of hepatic injury (Table 1). In these studies, most of the patients were treated with corticosteroids8-29.
The management of DRESS syndrome is challenging. It is important to withdraw the suspected drug and the delay is associated with poorer outcomes.30-32 Supportive therapy in intensive care unit should be provided to stabilize the patient. Early administration of systemic corticosteroid therapy is generally recommended.33 Systemic corticosteroid helps to improve in both clinical symptoms and laboratory abnormalities within days.33 Most of the cases reports of DRESS syndrome with liver dysfunction showed success with corticosteroids treatment (Table 1).
Liver transplantation is an option when DRESS is associated with acute fulminant hepatic failure, but the prognosis remains poor (Table 2)34-39. Besnard et al.34 reported two pediatric Crohn’s disease patients undergoing liver transplantation after DRESS syndrome induced by sulfasalazine. During follow-up, one of them developed acute rejection and fatal aspergilosis. Song et al.37 reported living-donor liver transplantation in a 14-years old patient. Patient presented chronic rejection after 25-months follow-up. Amante et al.35 and Roales-Gómez et al. 38 reported adult patients treated with OLT, with no information concerning long-term follow-up. Mennickea et al.36 reported an adult patient treated with OLT, with mortality in postoperative period due to massive intrabominal blood loss.
Recent studies support the use of Molecular Adsorbents Recirculation System (MARS) as a rescue for patients with liver failure. Roales-Gómez et al. 38 described MARS use, although patient did not responde well, and patients was eventually submmited to OLT. Ng et al.23 reported a pediatric patient that underwent MARS in the intensive care unit, with satisfactory response.
The present study showed a patient with Sulfasalazine and Trimethoprim-sulfamethoxazole severe reaction. Sulfasalazine and Trimethoprim-sulfamethoxazole carries a significant risk of drug toxicity. Yusuf et al.24 reported the first case of DRESS syndrome in a child treated for toxoplasma retinochoroiditis. Rare cases of immunoallergic reactions to sulfasalazine, including DRESS syndromes, have been reported, such as the classic “3-week sulfasalazine syndrome” occurring 3 weeks after the first administration.8
CONCLUSION
DRESS is associated with acute liver failure is a lifethreatening condition. Liver transplantation is an option for the management of these patients, although the prognosis remains poor.