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.