DRESS syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Natural History

• DRESS syndrome is characterized by a prolonged latency period.
• Clinical manifestations of DRESS syndrome are usually delayed. Earliest manifestations may appear 2-8 weeks following the administration of triggering drug.
• Initially, patients usually develop non-specific signs and symptoms, namely fever and rash, making the early diagnosis of DRESS syndrome difficult upon patient presentation.
• Additional clinical manifestations follow, and patients may subsequently develop lymphadenopathy, visceral disease (typically liver involvement), and worsening of the skin eruption.
• The nature of the visceral involvement is thought to be associated with the identity of the triggering drug:

• Hepatic and GI involvement has been associated with abacavir
• Renal involvement has been associated with allopurinol
• Pulmonary involvement has been associated with abacavir and minocycline

• In the majority of cases, DRESS syndrome resolves following the discontinuation of triggering drug, but clinical manifestations may persist up to 3 months.

Complications

• Organ involvement is considered the most important complication of DRESS syndrome.
• Complications of DRESS syndrome include the following:

• Hepatitis and hepatic necrosis
• Interstitial nephritis
• Chronic kidney disease
• Pneumonitis
• Eosinophilic pneumopathy
• Carditis
• Encephalopathy
• Pleuritis
• Pericarditis
• Myopathy
• Pancreatitis
• Thyroiditis

• Long term complications often include the development of autoimmune diseases, such as:
• Autoimmune hemolytic anemia
• Graves' disease
• Insulin-dependent diabetes mellitus

Prognosis

• The prognosis of DRESS syndrome is generally good.
• The case-fatality rate of DRESS syndrome is approximately 10%.
• Factors associated with worse prognosis of DRESS syndrome have not yet been established. Early drug discontinuation has been suggested as a favorable prognostic factor.
• Patients who do not undergo slow tapering (over several weeks) are thought to be at an increased risk of relapse.