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: Yazan Daaboul, M.D.

Overview

DRESS syndrome is characterized by a prolonged latency period (2-8 weeks following the administration of triggering drug). If left untreated, DRESS syndrome self-resolves following the discontinuation of the triggering drug in the majority of cases, but clinical manifestations may persist up to 3 months and the long-term sequelae have not yet been identified. Complications of DRESS syndrome include visceral organ involvement and long-term autoimmune diseases. The prognosis of DRESS syndrome is generally good, and the case-fatality rate is approximately 10%. Factors associated with worse prognosis have not yet been established.

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 that involves the face, upper trunk, and upper extremities, 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

• If left untreated, DRESS syndrome self-resolves following the discontinuation of triggering drug in the majority of cases, but clinical manifestations may persist up to 3 months and the long-term sequelae have not yet been identified.

Complications

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

• Hepatitis and hepatic necrosis
• Acute interstitial nephritis
• Chronic kidney disease
• Pneumonitis
• Eosinophilic pneumopathy
• Eosinophilic myocarditis
• Pleuritis
• Pericarditis
• Myositis
• Uveitis
• Pancreatitis
• Encephalitis
• Meningitis

• 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%. Mortality is most commonly due to fulminant hepatic failure.
• Factors associated with worse prognosis of DRESS syndrome have not yet been established. Early drug discontinuation has been suggested as a favorable prognostic factor, whereas extent of affected body surface area is thought to be a poor prognostic factor.
• Administration of corticosteroid therapy has not been demonstrated to be associated with reduced risk of death, , but patients who do not undergo slow corticosteroid tapering (over several weeks) are thought to be at an increased risk of relapse.