DRESS syndrome natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{DRESS syndrome}} | {{DRESS syndrome}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} {{YD}} | ||
==Overview== | ==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== | ==Natural History== | ||
*DRESS syndrome is characterized by a prolonged latency period. | *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. | *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. | *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. | *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:<ref name="pmid23882307">{{cite journal| author=Choudhary S, McLeod M, Torchia D, Romanelli P| title=Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. | journal=J Clin Aesthet Dermatol | year= 2013 | volume= 6 | issue= 6 | pages= 31-7 | pmid=23882307 | doi= | pmc=PMC3718748 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23882307 }} </ref> | *The nature of the visceral involvement is thought to be associated with the identity of the triggering drug:<ref name="pmid23882307">{{cite journal| author=Choudhary S, McLeod M, Torchia D, Romanelli P| title=Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. | journal=J Clin Aesthet Dermatol | year= 2013 | volume= 6 | issue= 6 | pages= 31-7 | pmid=23882307 | doi= | pmc=PMC3718748 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23882307 }} </ref> | ||
| Line 13: | Line 15: | ||
:*Renal involvement has been associated with allopurinol | :*Renal involvement has been associated with allopurinol | ||
:*Pulmonary involvement has been associated with abacavir and minocycline | :*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== | ==Complications== | ||
| Line 19: | Line 21: | ||
*Complications of DRESS syndrome include the following: | *Complications of DRESS syndrome include the following: | ||
:*[[Hepatitis]] and hepatic necrosis | :*[[Hepatitis]] and hepatic necrosis | ||
:*[[ | :*[[Acute interstitial nephritis]] | ||
:*[[Chronic kidney disease]] | |||
:*[[Pneumonitis]] | :*[[Pneumonitis]] | ||
:*Eosinophilic pneumopathy | :*[[Pneumonitis|Eosinophilic pneumopathy]] | ||
:*[[Myocarditis|Eosinophilic myocarditis]] | |||
:*[[ | |||
:*[[Pleuritis]] | :*[[Pleuritis]] | ||
:*[[Pericarditis]] | :*[[Pericarditis]] | ||
:*[[ | :*[[Myositis]] | ||
:*[[Uveitis]] | |||
:*[[Pancreatitis]] | :*[[Pancreatitis]] | ||
:*[[Encephalitis]] | |||
:*[[Meningitis]] | |||
*Long term complications often include the development of autoimmune diseases, such as: | |||
:*[[Autoimmune hemolytic anemia]] | |||
:*[[Graves' disease]] | |||
:*[[Type 1 diabetes mellitus|Insulin-dependent diabetes mellitus]] | |||
==Prognosis== | ==Prognosis== | ||
*The prognosis of DRESS syndrome is generally good. | *The prognosis of DRESS syndrome is generally good. | ||
*The case-fatality rate of DRESS syndrome is approximately 10%. | *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. | *Factors associated with worse prognosis of DRESS syndrome have not yet been established. Early drug discontinuation has been suggested as a favorable prognostic factor<ref name="pmid20136879">{{cite journal| author=Santiago F, Gonçalo M, Vieira R, Coelho S, Figueiredo A| title=Epicutaneous patch testing in drug hypersensitivity syndrome (DRESS). | journal=Contact Dermatitis | year= 2010 | volume= 62 | issue= 1 | pages= 47-53 | pmid=20136879 | doi=10.1111/j.1600-0536.2009.01659.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20136879 }} </ref>, 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, <ref name="pmid20713773">{{cite journal| author=Chen YC, Chiu HC, Chu CY| title=Drug reaction with eosinophilia and systemic symptoms: a retrospective study of 60 cases. | journal=Arch Dermatol | year= 2010 | volume= 146 | issue= 12 | pages= 1373-9 | pmid=20713773 | doi=10.1001/archdermatol.2010.198 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20713773 }} </ref>, but patients who do not undergo slow corticosteroid tapering (over several weeks) are thought to be at an increased risk of relapse. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
[[Category:Hepatology]] | [[Category:Hepatology]] | ||
{{WS}} | |||
{{WH}} | |||
Latest revision as of 13:23, 20 July 2016
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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:[1]
- 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:
- Long term complications often include the development of autoimmune diseases, such as:
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[2], 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, [3], but patients who do not undergo slow corticosteroid tapering (over several weeks) are thought to be at an increased risk of relapse.
References
- ↑ Choudhary S, McLeod M, Torchia D, Romanelli P (2013). "Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome". J Clin Aesthet Dermatol. 6 (6): 31–7. PMC 3718748. PMID 23882307.
- ↑ Santiago F, Gonçalo M, Vieira R, Coelho S, Figueiredo A (2010). "Epicutaneous patch testing in drug hypersensitivity syndrome (DRESS)". Contact Dermatitis. 62 (1): 47–53. doi:10.1111/j.1600-0536.2009.01659.x. PMID 20136879.
- ↑ Chen YC, Chiu HC, Chu CY (2010). "Drug reaction with eosinophilia and systemic symptoms: a retrospective study of 60 cases". Arch Dermatol. 146 (12): 1373–9. doi:10.1001/archdermatol.2010.198. PMID 20713773.