Adult-onset Still's disease natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course. Initial presentation of AOSD may be between 16 to 35 years of age. Symptoms usually evolve over weeks to months. Life-threatening complications known to be associated with AOSD include, macrophage activating syndrome (MAS), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), diffuse alveolar | If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course. Initial presentation of AOSD may be between 16 to 35 years of age. [[Symptoms]] usually evolve over weeks to months. Life-threatening complications known to be associated with AOSD include, [[Hemophagocytic syndrome|macrophage activating syndrome]] ([[Hemophagocytic syndrome|MAS]]), [[disseminated intravascular coagulation]] ([[Disseminated intravascular coagulation|DIC]]), [[thrombotic thrombocytopenic purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]]), [[Diffuse alveolar damage|diffuse alveolar hemorrhage]], [[pulmonary arterial hypertension]] ([[Pulmonary hypertension|PAH]]), [[pericardial tamponade]] and [[myocarditis]]. The [[prognosis]] of adult-onset Still's disease depends upon the clinical course of the disease. The chronic articular form of the disease is associated with worse [[prognosis]]. Other indicators of poor [[prognosis]] of AOSD include, presence of [[polyarthritis]], interestitial pneumonia, [[pleuritis]], joint erosions, Still's rash (salmon-colored [[maculopapular rash]]) and development of secondary complications. | ||
==Natural History== | ==Natural History== | ||
* If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course. | * If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course. | ||
* Initial presentation of AOSD may be between 16 to 35 years of age. | * Initial presentation of AOSD may be between 16 to 35 years of age. | ||
* Symptoms usually evolve over weeks to months. | * [[Symptoms]] usually evolve over weeks to months. | ||
* AOSD exhibits a variable clinical course: | * AOSD exhibits a variable clinical course: | ||
** 20% with long-term remission | ** 20% with long-term remission | ||
** 30% remit-relapse | ** 30% remit-relapse | ||
** 50% chronic arthritis | ** 50% chronic arthritis | ||
* May progress to develop macrophage activating syndrome (MAS), also known as hemophagocytic syndrome. | * May progress to develop [[Hemophagocytic syndrome|macrophage activating syndrome]] (MAS), also known as [[hemophagocytic syndrome]]. | ||
==Complications== | ==Complications== | ||
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=== Life threatening complications === | === Life threatening complications === | ||
Adult-onset Still's disease may lead to the development of the following life-threatening complications:<ref name="pmid24435354">{{cite journal |vauthors=Efthimiou P, Kadavath S, Mehta B |title=Life-threatening complications of adult-onset Still's disease |journal=Clin. Rheumatol. |volume=33 |issue=3 |pages=305–14 |date=March 2014 |pmid=24435354 |doi=10.1007/s10067-014-2487-4 |url=}}</ref><ref name="pmid2351497">{{cite journal |vauthors=Sachs RN, Talvard O, Lanfranchi J |title=Myocarditis in adult Still's disease |journal=Int. J. Cardiol. |volume=27 |issue=3 |pages=377–80 |date=June 1990 |pmid=2351497 |doi= |url=}}</ref> | Adult-onset Still's disease may lead to the development of the following life-threatening complications:<ref name="pmid24435354">{{cite journal |vauthors=Efthimiou P, Kadavath S, Mehta B |title=Life-threatening complications of adult-onset Still's disease |journal=Clin. Rheumatol. |volume=33 |issue=3 |pages=305–14 |date=March 2014 |pmid=24435354 |doi=10.1007/s10067-014-2487-4 |url=}}</ref><ref name="pmid2351497">{{cite journal |vauthors=Sachs RN, Talvard O, Lanfranchi J |title=Myocarditis in adult Still's disease |journal=Int. J. Cardiol. |volume=27 |issue=3 |pages=377–80 |date=June 1990 |pmid=2351497 |doi= |url=}}</ref> | ||
* Macrophage activation syndrome (MAS) | * [[Macrophage activation syndrome]] ([[Macrophage-activation syndrome|MAS]]) | ||
* Disseminated intravascular coagulation (DIC) | * [[Disseminated intravascular coagulation]] ([[Disseminated intravascular coagulation|DIC]]) | ||
* Thrombotic thrombocytopenic purpura (TTP) | * [[Thrombotic thrombocytopenic purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]]) | ||
* Diffuse alveolar | * [[Diffuse alveolar damage|Diffuse alveolar hemorrhage]] | ||
* Pulmonary arterial hypertension (PAH) | * [[Pulmonary arterial hypertension]] ([[Pulmonary hypertension|PAH]]) | ||
* Pericardial tamponade | * [[Pericardial tamponade]] | ||
* Myocarditis | * [[Myocarditis]] | ||
==Prognosis== | ==Prognosis== | ||
The prognosis of adult-onset Still's disease depends upon the clinical course of the disease:<ref name="pmid11100625">{{cite journal |vauthors=Cush JJ |title=Adult-onset Still's disease |journal=Bull Rheum Dis |volume=49 |issue=6 |pages=1–4 |date=2000 |pmid=11100625 |doi= |url=}}</ref><ref name="pmid3659248">{{cite journal |vauthors=Wouters JM, van de Putte LB |title=Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases |journal=Q. J. Med. |volume=61 |issue=235 |pages=1055–65 |date=November 1986 |pmid=3659248 |doi= |url=}}</ref> | The [[prognosis]] of adult-onset Still's disease depends upon the clinical course of the disease:<ref name="pmid11100625">{{cite journal |vauthors=Cush JJ |title=Adult-onset Still's disease |journal=Bull Rheum Dis |volume=49 |issue=6 |pages=1–4 |date=2000 |pmid=11100625 |doi= |url=}}</ref><ref name="pmid3659248">{{cite journal |vauthors=Wouters JM, van de Putte LB |title=Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases |journal=Q. J. Med. |volume=61 |issue=235 |pages=1055–65 |date=November 1986 |pmid=3659248 |doi= |url=}}</ref> | ||
=== Self-limited course === | === Self-limited course === | ||
* The self-limited form of AOSD is characterized by systemic symptoms for example, fever, rash, serositis, and organomegaly. | * The self-limited form of AOSD is characterized by systemic symptoms for example, [[fever]], [[rash]], [[serositis]], and [[organomegaly]]. | ||
* Most patients experience one episode and remit within 1 year of the initial presentation. | * Most patients experience one episode and remit within 1 year of the initial presentation. | ||
=== Intermittent systemic course === | === Intermittent systemic course === | ||
* Patients experience pre-dominantly systemic symptoms with or without joint symptoms. | * Patients experience pre-dominantly [[systemic]] [[symptoms]] with or without [[joint]] [[symptoms]]. | ||
* Patients typically follow a relapsing-remitting course with intermittent flares. | * Patients typically follow a relapsing-remitting course with intermittent flares. | ||
* Most episodes milder than the initial presentation. | * Most episodes milder than the initial presentation. | ||
=== Chronic articular form === | === Chronic articular form === | ||
* Patients have pre-dominant articular symptoms and joint destruction. | * Patients have pre-dominant [[articular]] [[symptoms]] and [[joint]] destruction. | ||
* The articular form is associated with a worse prognosis than the systemic form. | * The articular form is associated with a worse [[prognosis]] than the [[systemic]] form. | ||
* Severe joint destruction may warrant joint replacement surgery in severely ill patients. | * Severe [[joint]] destruction may warrant joint replacement surgery in severely ill patients. | ||
=== Indicators of poor prognosis === | === Indicators of poor prognosis === | ||
* The following findings may be associated with a poor prognosis in patients suffering from AOSD: | * The following findings may be associated with a poor [[prognosis]] in patients suffering from AOSD: | ||
** Polyarthritis | ** [[Polyarthritis]] | ||
** Interestitial pneumonia | ** Interestitial pneumonia | ||
** Pleuritis | ** [[Pleuritis]] | ||
** Joint erosions | ** [[Joint]] erosions | ||
** Still's rash | ** Still's rash | ||
** Development of secondary complications, for example: | ** Development of secondary complications, for example: | ||
*** Macrophage activation syndrome (MAS) | *** [[Macrophage-activation syndrome|Macrophage activation syndrome]] ([[Macrophage-activation syndrome|MAS]]) | ||
*** Disseminated intravascular coagulopathy | *** [[Disseminated intravascular coagulation|Disseminated intravascular coagulopathy]] ([[Disseminated intravascular coagulation|DIC]]) | ||
*** Thrombotic thrombocytopenic purpura | *** [[Thrombotic thrombocytopenic purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]]) | ||
*** Diffuse alveolar hemorrhage | *** [[Diffuse alveolar damage|Diffuse alveolar hemorrhage]] | ||
*** [[Pulmonary hypertension|Pulmonary arterial hypertension]] | |||
==References== | ==References== | ||
Revision as of 13:23, 23 April 2018
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Adult-onset Still's disease |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]
Overview
If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course. Initial presentation of AOSD may be between 16 to 35 years of age. Symptoms usually evolve over weeks to months. Life-threatening complications known to be associated with AOSD include, macrophage activating syndrome (MAS), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), diffuse alveolar hemorrhage, pulmonary arterial hypertension (PAH), pericardial tamponade and myocarditis. The prognosis of adult-onset Still's disease depends upon the clinical course of the disease. The chronic articular form of the disease is associated with worse prognosis. Other indicators of poor prognosis of AOSD include, presence of polyarthritis, interestitial pneumonia, pleuritis, joint erosions, Still's rash (salmon-colored maculopapular rash) and development of secondary complications.
Natural History
- If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course.
- Initial presentation of AOSD may be between 16 to 35 years of age.
- Symptoms usually evolve over weeks to months.
- AOSD exhibits a variable clinical course:
- 20% with long-term remission
- 30% remit-relapse
- 50% chronic arthritis
- May progress to develop macrophage activating syndrome (MAS), also known as hemophagocytic syndrome.
Complications
Life threatening complications
Adult-onset Still's disease may lead to the development of the following life-threatening complications:[1][2]
- Macrophage activation syndrome (MAS)
- Disseminated intravascular coagulation (DIC)
- Thrombotic thrombocytopenic purpura (TTP)
- Diffuse alveolar hemorrhage
- Pulmonary arterial hypertension (PAH)
- Pericardial tamponade
- Myocarditis
Prognosis
The prognosis of adult-onset Still's disease depends upon the clinical course of the disease:[3][4]
Self-limited course
- The self-limited form of AOSD is characterized by systemic symptoms for example, fever, rash, serositis, and organomegaly.
- Most patients experience one episode and remit within 1 year of the initial presentation.
Intermittent systemic course
- Patients experience pre-dominantly systemic symptoms with or without joint symptoms.
- Patients typically follow a relapsing-remitting course with intermittent flares.
- Most episodes milder than the initial presentation.
Chronic articular form
- Patients have pre-dominant articular symptoms and joint destruction.
- The articular form is associated with a worse prognosis than the systemic form.
- Severe joint destruction may warrant joint replacement surgery in severely ill patients.
Indicators of poor prognosis
- The following findings may be associated with a poor prognosis in patients suffering from AOSD:
- Polyarthritis
- Interestitial pneumonia
- Pleuritis
- Joint erosions
- Still's rash
- Development of secondary complications, for example:
References
- ↑ Efthimiou P, Kadavath S, Mehta B (March 2014). "Life-threatening complications of adult-onset Still's disease". Clin. Rheumatol. 33 (3): 305–14. doi:10.1007/s10067-014-2487-4. PMID 24435354.
- ↑ Sachs RN, Talvard O, Lanfranchi J (June 1990). "Myocarditis in adult Still's disease". Int. J. Cardiol. 27 (3): 377–80. PMID 2351497.
- ↑ Cush JJ (2000). "Adult-onset Still's disease". Bull Rheum Dis. 49 (6): 1–4. PMID 11100625.
- ↑ Wouters JM, van de Putte LB (November 1986). "Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases". Q. J. Med. 61 (235): 1055–65. PMID 3659248.