Systemic lupus erythematosus natural history, complications and prognosis: Difference between revisions

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{{CMG}}; {{AE}}{{MIR}}
{{CMG}}; {{AE}}{{MIR}}
==Overview==
==Overview==
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]] and [[arthritis]]. [[Prognosis]] is generally poor, and the 10-year [[mortality rate]] of patients with systemic lupus erythematosus is approximately 40%. The disease course can be divided into 4 subcategories based on the course of the disease: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]], and [[arthritis]]. [[Prognosis]] is generally poor, and the 10-year [[mortality rate]] of patients with systemic lupus erythematosus is approximately 40%. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.
==Natural History==
==Natural History==
*Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE is a disease of waxing and waning, with possible flare up episodes. SLE usually develops in the second and third decade of life, although it can presents any age, and start with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which will eventually lead to death in most of the patients.
Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. Several flare-ups may happen in the course of the disease. SLE usually develops in the second and third decades of life, although it can present at any age. It usually starts with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which will eventually lead to death in most patients.
*The disease course can be divided into 4 subcategories based on the course of the disease:
 
The disease course can be divided into 4 subcategories based on the course of the disease:<ref name="pmid22028590">{{cite journal |vauthors=Iwata Y, Furuichi K, Kaneko S, Wada T |title=The role of cytokine in the lupus nephritis |journal=J. Biomed. Biotechnol. |volume=2011 |issue= |pages=594809 |year=2011 |pmid=22028590 |pmc=3199078 |doi=10.1155/2011/594809 |url=}}</ref><ref name="pmid18305268">{{cite journal |vauthors=Rahman A, Isenberg DA |title=Systemic lupus erythematosus |journal=N. Engl. J. Med. |volume=358 |issue=9 |pages=929–39 |year=2008 |pmid=18305268 |doi=10.1056/NEJMra071297 |url=}}</ref><ref name="pmid1893619">{{cite journal |vauthors=Deguchi Y, Kishimoto S |title=Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice |journal=Clin. Exp. Immunol. |volume=85 |issue=3 |pages=392–5 |year=1991 |pmid=1893619 |pmc=1535595 |doi= |url=}}</ref>
 
===== Developmental phase: =====
===== Developmental phase: =====
*[[Genetic mutations]]
*[[Genetic mutations]]
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===== Comorbidity-complication phase =====
===== Comorbidity-complication phase =====
The phase of damages due to complications of longstanding disease, [[immunosuppressive therapy]], and [[End organ damage|end organ damages]] (irreversible damages and complications)
The phase of damages due to complications of longstanding disease, [[immunosuppressive therapy]], and [[end organ damage]] (irreversible damages and complications)
*[[Infections]]
*[[Infections]]
*[[Atherosclerosis]]
*[[Atherosclerosis]]
*[[Malignancies]]
*[[Malignancies]]


=== Factors associated with flare up: ===
=== Factors associated with flare up: <ref name="pmid26330673">{{cite journal |vauthors=Crow MK, Olferiev M, Kirou KA |title=Identification of Candidate Predictors of Lupus Flare |journal=Trans. Am. Clin. Climatol. Assoc. |volume=126 |issue= |pages=184–96 |year=2015 |pmid=26330673 |pmc=4530671 |doi= |url=}}</ref><ref name="pmid1893619">{{cite journal |vauthors=Deguchi Y, Kishimoto S |title=Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice |journal=Clin. Exp. Immunol. |volume=85 |issue=3 |pages=392–5 |year=1991 |pmid=1893619 |pmc=1535595 |doi= |url=}}</ref><ref name="pmid3199078">{{cite journal |vauthors=Josić D, Hofmann W, Habermann R, Schulzke JD, Reutter W |title=Isolation of immunoglobulins and their use in immunoaffinity HPLC |journal=J. Clin. Chem. Clin. Biochem. |volume=26 |issue=9 |pages=559–68 |year=1988 |pmid=3199078 |doi= |url=}}</ref>  ===
* [[Stress]] (emotional etc.)
* [[Stress]] (emotional, etc.)
* [[Sunlight]]
* [[Sunlight]]
* [[Ultraviolet]] light
* [[Ultraviolet]] light
Line 44: Line 45:
* [[Medications]]
* [[Medications]]
* [[Immunization|Immunizations]]
* [[Immunization|Immunizations]]
* [[Lupus nephritis]]
* Presence of [[Neurological disease|neurologic]] complications
* Presence of [[vasculitis]]
* Elevated [[anti-dsDNA]]
* Low C3 level


==Complications==
==Complications==
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:<ref name="pmid23395811">{{cite journal |vauthors=Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA |title=Systemic lupus erythematosus: a review of the disease and treatment options |journal=Consult Pharm |volume=28 |issue=2 |pages=110–21 |year=2013 |pmid=23395811 |doi=10.4140/TCP.n.2013.110 |url=}}</ref><ref name="pmid24234325">{{cite journal |vauthors=Zubair A, Frieri M |title=Lupus nephritis: review of the literature |journal=Curr Allergy Asthma Rep |volume=13 |issue=6 |pages=580–6 |year=2013 |pmid=24234325 |doi=10.1007/s11882-013-0394-4 |url=}}</ref><ref name="pmid20477476">{{cite journal |vauthors=Torres A, Askari AD, Malemud CJ |title=Cardiovascular disease complications in systemic lupus erythematosus |journal=Biomark Med |volume=3 |issue=3 |pages=239–52 |year=2009 |pmid=20477476 |doi=10.2217/bmm.09.14 |url=}}</ref><ref name="pmid18852220">{{cite journal |vauthors=Cortes S, Chambers S, Jerónimo A, Isenberg D |title=Diabetes mellitus complicating systemic lupus erythematosus - analysis of the UCL lupus cohort and review of the literature |journal=Lupus |volume=17 |issue=11 |pages=977–80 |year=2008 |pmid=18852220 |doi=10.1177/0961203308091539 |url=}}</ref><ref name="pmid18703174">{{cite journal |vauthors=Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A |title=Infections as triggers and complications of systemic lupus erythematosus |journal=Autoimmun Rev |volume=8 |issue=1 |pages=24–8 |year=2008 |pmid=18703174 |doi=10.1016/j.autrev.2008.07.019 |url=}}</ref><ref name="pmid6127033">{{cite journal |vauthors=Zizic TM, Classen JN, Stevens MB |title=Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa |journal=Am. J. Med. |volume=73 |issue=4 |pages=525–31 |year=1982 |pmid=6127033 |doi= |url=}}</ref><ref name="pmid22879850">{{cite journal |vauthors=Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD |title=Manifestations of systemic lupus erythematosus |journal=Maedica (Buchar) |volume=6 |issue=4 |pages=330–6 |year=2011 |pmid=22879850 |pmc=3391953 |doi= |url=}}</ref><ref name="pmid18456233">{{cite journal |vauthors=Clowse ME, Jamison M, Myers E, James AH |title=A national study of the complications of lupus in pregnancy |journal=Am. J. Obstet. Gynecol. |volume=199 |issue=2 |pages=127.e1–6 |year=2008 |pmid=18456233 |pmc=2542836 |doi=10.1016/j.ajog.2008.03.012 |url=}}</ref><ref name="pmid25369438">{{cite journal |vauthors=Bhattacharyya S, Helfgott SM |title=Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis |journal=Semin Neurol |volume=34 |issue=4 |pages=425–36 |year=2014 |pmid=25369438 |doi=10.1055/s-0034-1390391 |url=}}</ref><ref name="pmid27329649">{{cite journal |vauthors=Alves SC, Fasano S, Isenberg DA |title=Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review |journal=Lupus |volume=25 |issue=14 |pages=1509–1519 |year=2016 |pmid=27329649 |doi=10.1177/0961203316655210 |url=}}</ref><ref name="pmid12960476">{{cite journal |vauthors=Kang I, Park SH |title=Infectious complications in SLE after immunosuppressive therapies |journal=Curr Opin Rheumatol |volume=15 |issue=5 |pages=528–34 |year=2003 |pmid=12960476 |doi= |url=}}</ref>
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:<ref name="pmid23395811">{{cite journal |vauthors=Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA |title=Systemic lupus erythematosus: a review of the disease and treatment options |journal=Consult Pharm |volume=28 |issue=2 |pages=110–21 |year=2013 |pmid=23395811 |doi=10.4140/TCP.n.2013.110 |url=}}</ref><ref name="pmid24234325">{{cite journal |vauthors=Zubair A, Frieri M |title=Lupus nephritis: review of the literature |journal=Curr Allergy Asthma Rep |volume=13 |issue=6 |pages=580–6 |year=2013 |pmid=24234325 |doi=10.1007/s11882-013-0394-4 |url=}}</ref><ref name="pmid20477476">{{cite journal |vauthors=Torres A, Askari AD, Malemud CJ |title=Cardiovascular disease complications in systemic lupus erythematosus |journal=Biomark Med |volume=3 |issue=3 |pages=239–52 |year=2009 |pmid=20477476 |doi=10.2217/bmm.09.14 |url=}}</ref><ref name="pmid18852220">{{cite journal |vauthors=Cortes S, Chambers S, Jerónimo A, Isenberg D |title=Diabetes mellitus complicating systemic lupus erythematosus - analysis of the UCL lupus cohort and review of the literature |journal=Lupus |volume=17 |issue=11 |pages=977–80 |year=2008 |pmid=18852220 |doi=10.1177/0961203308091539 |url=}}</ref><ref name="pmid18703174">{{cite journal |vauthors=Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A |title=Infections as triggers and complications of systemic lupus erythematosus |journal=Autoimmun Rev |volume=8 |issue=1 |pages=24–8 |year=2008 |pmid=18703174 |doi=10.1016/j.autrev.2008.07.019 |url=}}</ref><ref name="pmid6127033">{{cite journal |vauthors=Zizic TM, Classen JN, Stevens MB |title=Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa |journal=Am. J. Med. |volume=73 |issue=4 |pages=525–31 |year=1982 |pmid=6127033 |doi= |url=}}</ref><ref name="pmid22879850">{{cite journal |vauthors=Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD |title=Manifestations of systemic lupus erythematosus |journal=Maedica (Buchar) |volume=6 |issue=4 |pages=330–6 |year=2011 |pmid=22879850 |pmc=3391953 |doi= |url=}}</ref><ref name="pmid18456233">{{cite journal |vauthors=Clowse ME, Jamison M, Myers E, James AH |title=A national study of the complications of lupus in pregnancy |journal=Am. J. Obstet. Gynecol. |volume=199 |issue=2 |pages=127.e1–6 |year=2008 |pmid=18456233 |pmc=2542836 |doi=10.1016/j.ajog.2008.03.012 |url=}}</ref><ref name="pmid25369438">{{cite journal |vauthors=Bhattacharyya S, Helfgott SM |title=Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis |journal=Semin Neurol |volume=34 |issue=4 |pages=425–36 |year=2014 |pmid=25369438 |doi=10.1055/s-0034-1390391 |url=}}</ref><ref name="pmid27329649">{{cite journal |vauthors=Alves SC, Fasano S, Isenberg DA |title=Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review |journal=Lupus |volume=25 |issue=14 |pages=1509–1519 |year=2016 |pmid=27329649 |doi=10.1177/0961203316655210 |url=}}</ref><ref name="pmid12960476">{{cite journal |vauthors=Kang I, Park SH |title=Infectious complications in SLE after immunosuppressive therapies |journal=Curr Opin Rheumatol |volume=15 |issue=5 |pages=528–34 |year=2003 |pmid=12960476 |doi= |url=}}</ref>
<small>(Up arrows represent higher frequencies and down arrows represent lower frequencies)</small>
{| class="wikitable"
{| class="wikitable"
!Organ
! style="background: #4479BA; color: #FFFFFF; " |Organ
!Disease
! style="background: #4479BA; color: #FFFFFF; " |Disease
!Description
! style="background: #4479BA; color: #FFFFFF; " |Description
!Frequency
! style="background: #4479BA; color: #FFFFFF; " |Frequency
|-
|-
| rowspan="8" |Gastrointestinal system
| rowspan="8" |<small>Gastrointestinal</small>
|[[Dysphagia]]
| style="background: #DCDCDC; " align="center" |[[Dysphagia]]
|
|
* Usually due to an underlying [[esophageal motility disorder]]
* Usually due to an underlying [[esophageal motility disorder]]
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|↑↑↑
|↑↑↑
|-
|-
|[[Peptic ulcer disease]]
| style="background: #DCDCDC; " align="center" |[[Peptic ulcer disease]]
|
|
* Due to
* Due to:
** The disease itself
** The disease itself
** The effect of SLE treatment
** The adverse effect of SLE treatment
|↑
|↑
|-
|-
|[[Intestinal pseudo-obstruction|Intestinal pseudo-obstruction]]
| style="background: #DCDCDC; " align="center" |[[Intestinal pseudo-obstruction|Intestinal pseudo-obstruction]]
|
|
* May lead to [[Gastrointestinal obstruction|mechanical obstruction]] of the small or large bowel in the absence of an anatomic lesion
* May lead to [[Gastrointestinal obstruction|mechanical obstruction]] of the small or large bowel in the absence of an anatomic lesion
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|↓↓
|↓↓
|-
|-
|[[Protein-losing enteropathy|Protein-losing enteropathy]]
| style="background: #DCDCDC; " align="center" |[[Protein-losing enteropathy|Protein-losing enteropathy]]
|
|
* Occurs in patients with clinically severe SLE with multi-organ involvement
* Occurs in patients with clinically severe SLE with multi-organ involvement
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|↓↓
|↓↓
|-
|-
|[[Hepatitis]]
| style="background: #DCDCDC; " align="center" |[[Hepatitis]]
|
|
* May be due to:
* May be due to:
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|↑
|↑
|-
|-
|[[Acute pancreatitis|Acute pancreatitis]]
| style="background: #DCDCDC; " align="center" |[[Acute pancreatitis|Acute pancreatitis]]
|
|
* Occurs usually in the setting of active SLE
* Occurs usually in the setting of active SLE
|↓
|↓
|-
|-
|[[Mesenteric vascular occlusion|Mesenteric vasculitis]]
| style="background: #DCDCDC; " align="center" |[[Mesenteric vascular occlusion|Mesenteric vasculitis]]
|
|
* Mostly involve:
* Mostly involve:
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|↓↓
|↓↓
|-
|-
|[[Acute cholecystitis]]
| style="background: #DCDCDC; " align="center" |[[Acute cholecystitis]]
|
|
* Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]]
* Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]]
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|↓↓
|↓↓
|-
|-
| rowspan="6" |Pulmonary involvement
| rowspan="6" |<small>Pulmonary</small>
|[[Pleural disease|Pleural disease]]
| style="background: #DCDCDC; " align="center" |[[Pleural disease|Pleural disease]]
|
|
* May lead to:
* May lead to:
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|↑
|↑
|-
|-
|[[Pneumonitis|Acute pneumonitis]]
| style="background: #DCDCDC; " align="center" |[[Pneumonitis|Acute pneumonitis]]
|
|
* Fulminant form of diffuse lung injury
* Fulminant form of diffuse lung injury
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|↓↓
|↓↓
|-
|-
|[[Pulmonary hemorrhage]]
| style="background: #DCDCDC; " align="center" |[[Pulmonary hemorrhage]]
|
|
* Pulmonary alveolar hemorrhage:
* Pulmonary alveolar hemorrhage:
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|↓↓
|↓↓
|-
|-
|[[Pulmonary hypertension]]
| style="background: #DCDCDC; " align="center" |[[Pulmonary hypertension]]
|
|
* Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]]  
* Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]]  
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|↑
|↑
|-
|-
|[[Thromboembolic disease]]
| style="background: #DCDCDC; " align="center" |[[Thromboembolic disease]]
|
|
* Chronic [[inflammation]] that increase the risk of [[Thromboembolic disease|thromboembolic events]]
* Chronic [[inflammation]] that increase the risk of [[Thromboembolic disease|thromboembolic events]]
|↑
|↑
|-
|-
|Shrinking lung syndrome
| style="background: #DCDCDC; " align="center" |Shrinking lung syndrome
|
|
* Characterized by [[dyspnea]] and [[Dyspnea|shortness of breath]] (estimated prevalence approximately 0.5%)
* Characterized by [[dyspnea]] and [[Dyspnea|shortness of breath]] (estimated prevalence approximately 0.5%)
|↓↓
|↓↓
|-
|-
| rowspan="5" |Cardiac involvement
| rowspan="5" |<small>Cardiac</small>
|[[Cardiomegaly]]  
| style="background: #DCDCDC; " align="center" |[[Cardiomegaly]]  
|
|
* Due to:
* Due to:
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|↑↑
|↑↑
|-
|-
|[[Valvular disease]]
| style="background: #DCDCDC; " align="center" |[[Valvular disease]]
|
|
* Different [[Valvular Diseases|valvular]] complications include:
* Different [[Valvular Diseases|valvular]] complications include:
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** Valve thickening
** Valve thickening
** Valve regurgitation
** Valve regurgitation
* Mainly due to [[Immunoglobulin]] and [[complement]] deposition in valvular structure
* Mainly due to [[immunoglobulin]] and [[complement]] deposition in valvular structure
* More frequently affect [[mitral valve]]
* Affect the [[mitral valve]] more frequently
|↑↑
|↑↑
|-
|-
|[[Pericardial disease]]
| style="background: #DCDCDC; " align="center" |[[Pericardial disease]]
|
|
* [[Acute pericarditis]]
* [[Acute pericarditis]]
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|↓
|↓
|-
|-
|[[Myocarditis]]
| style="background: #DCDCDC; " align="center" |[[Myocarditis]]
|
|
* Characterized by:
* Characterized by:
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** [[Heart failure]]
** [[Heart failure]]
** [[Sudden death]]
** [[Sudden death]]
* [[Myonecrosis]] may happen as a consequence of [[autoimmune]] reaction
* [[Myonecrosis]] may happen as a consequence of [[autoimmune]] reaction
|↓
|↓
|-
|-
|[[Coronary heart disease|Coronary artery disease]]
| style="background: #DCDCDC; " align="center" |[[Coronary heart disease|Coronary artery disease]]
|
|
* Mainly as a result of increased risk of [[Atheroma|atheromatous plaques]] due to autoimmune status of SLE
* Mainly as a result of increased risk of [[Atheroma|atheromatous plaques]] due to autoimmune status of SLE
|↑↑
|↑↑
|-
|-
| rowspan="5" |Neurological involvement
| rowspan="5" |<small>Neurological</small>
|[[Cognitive-shifting|Cognitive dysfunction]]
| style="background: #DCDCDC; " align="center" |[[Cognitive-shifting|Cognitive dysfunction]]
|
|
* May be temporarily affected by multiple, transient [[metabolic]] and systemic processes
* May be temporarily affected by multiple, transient [[metabolic]] and systemic processes
|↑
|↑
|-
|-
|[[Stroke]]
| style="background: #DCDCDC; " align="center" |[[Stroke]]
|
|
* Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]]
* Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]]
|↓
|↓
|-
|-
|[[Seizure|Seizures]]
| style="background: #DCDCDC; " align="center" |[[Seizure|Seizures]]
|
|
* May happen secondary to [[increased intracranial pressure]]:
* May happen secondary to [[increased intracranial pressure]]:
** [[Hypercoagulability]] state (due to inflammation)
** [[Hypercoagulability]] state (due to [[inflammation]])
** [[Thrombosis]] within the [[Cerebral venous sinus thrombosis|cerebral venous]]
** [[Thrombosis]] within the [[Cerebral venous sinus thrombosis|cerebral venous]]
|↑
|↑
|-
|-
|[[Psychosis]]
| style="background: #DCDCDC; " align="center" |[[Psychosis]]
|
|
* Can indirectly influence [[Cognition|cognitive performance]]
* Can indirectly influence [[Cognition|cognitive performance]]
Line 225: Line 233:
|↑↑
|↑↑
|-
|-
|[[Neuropathies]]
| style="background: #DCDCDC; " align="center" |[[Neuropathies]]
|
|
* [[Peripheral neuropathy]]
* [[Peripheral neuropathy]]
Line 233: Line 241:
|↑↑
|↑↑
|-
|-
| rowspan="4" |Musculoskeletal involvement
| rowspan="4" |<small>Musculoskeletal</small>
|[[Arthritis]]
| style="background: #DCDCDC; " align="center" |[[Arthritis]]
|
|
*Mostly symmetrical and non-erosive arthritis
*Mostly symmetrical and non-erosive [[arthritis]]
*[[Arthralgias]]
*[[Arthralgias]]
*Effusions
*Effusions
*Decreased [[range of motion]] of both small and large joints
*Decreased [[range of motion]] of both small and large joints
*Morning stiffness
*Morning [[stiffness]]
|↑↑↑↑
|↑↑↑↑
|-
|-
|[[Osteonecrosis]] ([[Avascular necrosis]])
| style="background: #DCDCDC; " align="center" |[[Osteonecrosis]] ([[Avascular necrosis]])
|
|
* Most common in the [[Femoral|femoral head]]
* Most common in the [[Femoral|femoral head]]
* Can involve [[Humerus|humeral head]], [[Tibial|tibial plateau]], and scaphoid navicular 
* Can involve [[Humerus|humeral head]], [[Tibial|tibial plateau]], and scaphoid navicular
* Usually [[bilateral]]
* Usually [[bilateral]]
* Often [[asymptomatic]]
* Often [[asymptomatic]]
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|↓
|↓
|-
|-
|[[Subcutaneous tissue|Subcutaneous nodules]]
| style="background: #DCDCDC; " align="center" |[[Subcutaneous tissue|Subcutaneous nodules]]
|
|
* Associated with active disease and flare ups
* Associated with active disease and flare ups
|↑
|↑
|-
|-
|[[Osteoporosis]]  
| style="background: #DCDCDC; " align="center" |[[Osteoporosis]]  
|
|
*Mostly due to [[glucocorticoid]] usage
*Mostly due to [[glucocorticoid]] usage
*Loss of height
*Loss of height
*Sudden back pain
*Sudden [[back pain]]
|↑
|↑
|-
|-
| rowspan="5" |Skin disorder
| rowspan="5" |<small>Skin</small>
|[[Cutaneous lupus erythematosus]]
| style="background: #DCDCDC; " align="center" |[[Cutaneous lupus erythematosus]]
|
|
*[[Erythema]] in a [[malar]] distribution over the cheeks and nose (but sparing the [[nasolabial folds]]), which appears after sun exposure
*[[Erythema]] in a [[malar]] distribution over the cheeks and nose (but sparing the [[nasolabial folds]]), which appears after sun exposure
|↑
|↑
|-
|-
|[[Photosensitivity]]
| style="background: #DCDCDC; " align="center" |[[Photosensitivity]]
|
|
* Common theme for skin [[lesions]] associated with SLE
* Common theme for skin [[lesions]] associated with SLE
|↑↑↑
|↑↑↑
|-
|-
|[[Alopecia|Non-scarring alopecia]]
| style="background: #DCDCDC; " align="center" |[[Alopecia|Non-scarring alopecia]]
|
|
* May occur at some point during the course of their disease
* May occur at some point during the course of their disease
|↑
|↑
|-
|-
|Oral and nasal ulcers
| style="background: #DCDCDC; " align="center" |Oral and nasal ulcers
|
|
* Usually painless
* Usually painless
|↑↑
|↑↑
|-
|-
|Discoid lesions
| style="background: #DCDCDC; " align="center" |Discoid lesions
|
|
* More [[inflammatory]]
* More [[inflammatory]]
Line 291: Line 299:
|↑
|↑
|-
|-
| rowspan="2" |Very rare disorders
| rowspan="2" |<small>Very rare disorders</small>
|[[Malignancy]]
| style="background: #DCDCDC; " align="center" |[[Malignancy]]
|
|
* [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]]
* [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]]
Line 301: Line 309:
|↓↓↓
|↓↓↓
|-
|-
|[[Diabetes mellitus]] 
| style="background: #DCDCDC; " align="center" |[[Diabetes mellitus]]  
|
|
* Increase predisposition to:
* Increase predisposition to:
** [[Lupus nephritis]]
** [[Lupus nephritis]]
** [[Peripheral neuropathy]]
** [[Peripheral neuropathy]]
** [[Retinal disease]]
** [[Retinal disease]]
|↓
|↓
|}
|}


==Prognosis==
==Prognosis==
The prognosis of systemic lupus erythematosus is ranging from a [[benign]] illness to an extremely rapid progressive disease that can lead to a [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus eryhtematosus will result in a very high [[mortality rate]], with a report of more than 60% mortality rate during the mid-20th century. SLE is associated with a 10 year mortality of more than 50% among patient with [[nephritis]]. The presence of [[nephritis]] is associated with a particularly poor prognosis among patients with SLE. The increase in [[survival rate]] of patients and better prognosis may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvement in SLE diagnosis have led to better [[prognosis]], the [[mortality rate]] among SLE patients is still 5 times more than normal population.<ref name="pmid24851681">{{cite journal |vauthors=Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L |title=[Clinical features and prognosis of patients with lupus nephritis] |language=Chinese |journal=Zhonghua Yi Xue Za Zhi |volume=94 |issue=13 |pages=973–6 |year=2014 |pmid=24851681 |doi= |url=}}</ref><ref name="pmid27307448">{{cite journal |vauthors=Ugarte A, Ruiz-Irastorza G |title=SLE: the changing prognosis |journal=Lupus |volume=25 |issue=12 |pages=1285–7 |year=2016 |pmid=27307448 |doi=10.1177/0961203316652948 |url=}}</ref><ref name="pmid20453401">{{cite journal |vauthors=Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y |title=The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide |journal=Intern. Med. |volume=49 |issue=9 |pages=823–8 |year=2010 |pmid=20453401 |doi= |url=}}</ref><ref name="pmid26434992">{{cite journal |vauthors=Sabio JM |title=[Systemic lupus erythematosus today] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=146 |issue=4 |pages=160–2 |year=2016 |pmid=26434992 |doi=10.1016/j.medcli.2015.08.001 |url=}}</ref>
The prognosis of systemic lupus erythematosus ranges from a [[benign]] illness to an extremely rapid progressive disease that can lead to a [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus eryhtematosus will result in a very high [[mortality rate]], with a report of higher than a 60% mortality rate during the mid-20th century. The presence of [[nephritis]] is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with [[nephritis]]. The increase in [[survival rate]] of patients and better prognosis may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better [[prognosis]], the [[mortality rate]] among SLE patients is still 5 times higher than the normal population.<ref name="pmid24851681">{{cite journal |vauthors=Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L |title=[Clinical features and prognosis of patients with lupus nephritis] |language=Chinese |journal=Zhonghua Yi Xue Za Zhi |volume=94 |issue=13 |pages=973–6 |year=2014 |pmid=24851681 |doi= |url=}}</ref><ref name="pmid27307448">{{cite journal |vauthors=Ugarte A, Ruiz-Irastorza G |title=SLE: the changing prognosis |journal=Lupus |volume=25 |issue=12 |pages=1285–7 |year=2016 |pmid=27307448 |doi=10.1177/0961203316652948 |url=}}</ref><ref name="pmid20453401">{{cite journal |vauthors=Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y |title=The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide |journal=Intern. Med. |volume=49 |issue=9 |pages=823–8 |year=2010 |pmid=20453401 |doi= |url=}}</ref><ref name="pmid26434992">{{cite journal |vauthors=Sabio JM |title=[Systemic lupus erythematosus today] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=146 |issue=4 |pages=160–2 |year=2016 |pmid=26434992 |doi=10.1016/j.medcli.2015.08.001 |url=}}</ref>


=== Poor prognostic factors for SLE survival: ===<ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref>
=== Poor prognostic factors for SLE survival:<ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref> ===
* Presence of [[nephritis]] (especially diffuse proliferative glomerulonephritis)
* Presence of [[nephritis]] (especially diffuse proliferative [[glomerulonephritis]])
* [[Hypertension]]
* [[Hypertension]]
* Male sex
* Male sex
Line 320: Line 328:
* Older age at presentation
* Older age at presentation
* Low socioeconomic status
* Low socioeconomic status
* Black race: Higher rate of nephritis
* Black race: Higher rate of [[nephritis]]
* Presence of [[antiphospholipid antibodies]]
* Presence of [[antiphospholipid antibodies]]
* High overall disease activity
* High overall disease activity


=== Prognosis markers: ===<ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref>
=== Prognosis markers: <ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref>===
 
===== Serum anti ds-DNA titres correlated with: =====
===== Serum anti ds-DNA titres correlated with: =====
** [[Lupus nephritis]]
* [[Lupus nephritis]]
** Progression to [[end-stage renal disease]]
* Progression to [[end-stage renal disease]]
** Increased disease severity
* Increased disease severity
** Damage or poor survival
* Damage or poor survival


===== Antiphospholipid antibodies correlated with: =====
===== Antiphospholipid antibodies correlated with: =====
** Features of the [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]]   
* Features of the [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]]   
** CNS involvement  
* CNS involvement  
** Severe [[lupus nephritis]]  
* Severe [[lupus nephritis]]  
** Increase in mortality rate
* Increase in mortality rate


=== SLE in men compared to women: ===
=== SLE in men compared to women: <ref name="pmid19784840">{{cite journal |vauthors=de Carvalho JF, do Nascimento AP, Testagrossa LA, Barros RT, Bonfá E |title=Male gender results in more severe lupus nephritis |journal=Rheumatol. Int. |volume=30 |issue=10 |pages=1311–5 |year=2010 |pmid=19784840 |doi=10.1007/s00296-009-1151-9 |url=}}</ref>===
* Less [[photosensitivity]]
* Less [[photosensitivity]]
* More [[serositis]]
* More [[serositis]]
Line 344: Line 351:
* Higher 1 year mortality compared to women
* Higher 1 year mortality compared to women


=== SLE in the elderly (>65) compared to middle age prevalency: ===
=== SLE in the elderly (>65) compared to middle age prevalency: <ref name="pmid24297642">{{cite journal |vauthors=Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M, Tao J, Zhang Y, Tan K, Li J, Chen Z, Ding X, Qian X, Da Z, Wang M, Sun L |title=Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus |journal=Lupus |volume=23 |issue=3 |pages=327–34 |year=2014 |pmid=24297642 |doi=10.1177/0961203313513508 |url=}}</ref> ===
* Lower incidence of:
* Lower incidence of:
** [[Malar rash]]
** [[Malar rash]]
Line 360: Line 367:


==References==
==References==
[[Reflist|2]]
{{Reflist|2}}

Latest revision as of 11:58, 17 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

Common complications of systemic lupus erythematosus include dermatitis, nephritis, and arthritis. Prognosis is generally poor, and the 10-year mortality rate of patients with systemic lupus erythematosus is approximately 40%. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

Natural History

Systemic lupus erythematosus (SLE) is an autoimmune disease. Several flare-ups may happen in the course of the disease. SLE usually develops in the second and third decades of life, although it can present at any age. It usually starts with mild symptoms such as fatigue, fever, and skin rashes. Without treatment, the patient will develop symptoms of end organ damage, which will eventually lead to death in most patients.

The disease course can be divided into 4 subcategories based on the course of the disease:[1][2][3]

Developmental phase:
Preclinical phase:
Clinical phase:
Comorbidity-complication phase

The phase of damages due to complications of longstanding disease, immunosuppressive therapy, and end organ damage (irreversible damages and complications)

Factors associated with flare up: [4][3][5]

Complications

Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:[6][7][8][9][10][11][12][13][14][15][16]

(Up arrows represent higher frequencies and down arrows represent lower frequencies)

Organ Disease Description Frequency
Gastrointestinal Dysphagia ↑↑↑
Peptic ulcer disease
  • Due to:
    • The disease itself
    • The adverse effect of SLE treatment
 ↑
Intestinal pseudo-obstruction
  • May lead to mechanical obstruction of the small or large bowel in the absence of an anatomic lesion
  • Obstructing the flow of intestinal contents
 ↓↓
Protein-losing enteropathy ↓↓
Hepatitis ↑
Acute pancreatitis
  • Occurs usually in the setting of active SLE
 ↓
Mesenteric vasculitis ↓↓
Acute cholecystitis ↓↓
Pulmonary Pleural disease ↑
Acute pneumonitis ↓↓
Pulmonary hemorrhage
  • Pulmonary alveolar hemorrhage:
↓↓
Pulmonary hypertension ↑
Thromboembolic disease ↑
Shrinking lung syndrome ↓↓
Cardiac Cardiomegaly ↑↑
Valvular disease ↑↑
Pericardial disease ↓
Myocarditis ↓
Coronary artery disease ↑↑
Neurological Cognitive dysfunction
  • May be temporarily affected by multiple, transient metabolic and systemic processes
 ↑
Stroke ↓
Seizures ↑
Psychosis ↑↑
Neuropathies ↑↑
Musculoskeletal Arthritis ↑↑↑↑
Osteonecrosis (Avascular necrosis) ↓
Subcutaneous nodules
  • Associated with active disease and flare ups
 ↑
Osteoporosis ↑
Skin Cutaneous lupus erythematosus ↑
Photosensitivity
  • Common theme for skin lesions associated with SLE
 ↑↑↑
Non-scarring alopecia
  • May occur at some point during the course of their disease
 ↑
Oral and nasal ulcers
  • Usually painless
 ↑↑
Discoid lesions ↑
Very rare disorders Malignancy ↓↓↓
Diabetes mellitus ↓

Prognosis

The prognosis of systemic lupus erythematosus ranges from a benign illness to an extremely rapid progressive disease that can lead to a fulminant organ failure and death. Without treatment, systemic lupus eryhtematosus will result in a very high mortality rate, with a report of higher than a 60% mortality rate during the mid-20th century. The presence of nephritis is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with nephritis. The increase in survival rate of patients and better prognosis may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better prognosis, the mortality rate among SLE patients is still 5 times higher than the normal population.[17][18][19][20]

Poor prognostic factors for SLE survival:[21]

Prognosis markers: [21]

Serum anti ds-DNA titres correlated with:
Antiphospholipid antibodies correlated with:

SLE in men compared to women: [22]

SLE in the elderly (>65) compared to middle age prevalency: [23]

References

  1. ↑ Iwata Y, Furuichi K, Kaneko S, Wada T (2011). "The role of cytokine in the lupus nephritis". J. Biomed. Biotechnol. 2011: 594809. doi:10.1155/2011/594809. PMC 3199078. PMID 22028590.
  2. ↑ Rahman A, Isenberg DA (2008). "Systemic lupus erythematosus". N. Engl. J. Med. 358 (9): 929–39. doi:10.1056/NEJMra071297. PMID 18305268.
  3. ↑ 3.0 3.1 Deguchi Y, Kishimoto S (1991). "Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice". Clin. Exp. Immunol. 85 (3): 392–5. PMC 1535595. PMID 1893619.
  4. ↑ Crow MK, Olferiev M, Kirou KA (2015). "Identification of Candidate Predictors of Lupus Flare". Trans. Am. Clin. Climatol. Assoc. 126: 184–96. PMC 4530671. PMID 26330673.
  5. ↑ Josić D, Hofmann W, Habermann R, Schulzke JD, Reutter W (1988). "Isolation of immunoglobulins and their use in immunoaffinity HPLC". J. Clin. Chem. Clin. Biochem. 26 (9): 559–68. PMID 3199078.
  6. ↑ Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA (2013). "Systemic lupus erythematosus: a review of the disease and treatment options". Consult Pharm. 28 (2): 110–21. doi:10.4140/TCP.n.2013.110. PMID 23395811.
  7. ↑ Zubair A, Frieri M (2013). "Lupus nephritis: review of the literature". Curr Allergy Asthma Rep. 13 (6): 580–6. doi:10.1007/s11882-013-0394-4. PMID 24234325.
  8. ↑ Torres A, Askari AD, Malemud CJ (2009). "Cardiovascular disease complications in systemic lupus erythematosus". Biomark Med. 3 (3): 239–52. doi:10.2217/bmm.09.14. PMID 20477476.
  9. ↑ Cortes S, Chambers S, Jerónimo A, Isenberg D (2008). "Diabetes mellitus complicating systemic lupus erythematosus - analysis of the UCL lupus cohort and review of the literature". Lupus. 17 (11): 977–80. doi:10.1177/0961203308091539. PMID 18852220.
  10. ↑ Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A (2008). "Infections as triggers and complications of systemic lupus erythematosus". Autoimmun Rev. 8 (1): 24–8. doi:10.1016/j.autrev.2008.07.019. PMID 18703174.
  11. ↑ Zizic TM, Classen JN, Stevens MB (1982). "Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa". Am. J. Med. 73 (4): 525–31. PMID 6127033.
  12. ↑ Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD (2011). "Manifestations of systemic lupus erythematosus". Maedica (Buchar). 6 (4): 330–6. PMC 3391953. PMID 22879850.
  13. ↑ Clowse ME, Jamison M, Myers E, James AH (2008). "A national study of the complications of lupus in pregnancy". Am. J. Obstet. Gynecol. 199 (2): 127.e1–6. doi:10.1016/j.ajog.2008.03.012. PMC 2542836. PMID 18456233.
  14. ↑ Bhattacharyya S, Helfgott SM (2014). "Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis". Semin Neurol. 34 (4): 425–36. doi:10.1055/s-0034-1390391. PMID 25369438.
  15. ↑ Alves SC, Fasano S, Isenberg DA (2016). "Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review". Lupus. 25 (14): 1509–1519. doi:10.1177/0961203316655210. PMID 27329649.
  16. ↑ Kang I, Park SH (2003). "Infectious complications in SLE after immunosuppressive therapies". Curr Opin Rheumatol. 15 (5): 528–34. PMID 12960476.
  17. ↑ Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L (2014). "[Clinical features and prognosis of patients with lupus nephritis]". Zhonghua Yi Xue Za Zhi (in Chinese). 94 (13): 973–6. PMID 24851681.
  18. ↑ Ugarte A, Ruiz-Irastorza G (2016). "SLE: the changing prognosis". Lupus. 25 (12): 1285–7. doi:10.1177/0961203316652948. PMID 27307448.
  19. ↑ Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y (2010). "The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide". Intern. Med. 49 (9): 823–8. PMID 20453401.
  20. ↑ Sabio JM (2016). "[Systemic lupus erythematosus today]". Med Clin (Barc) (in Spanish; Castilian). 146 (4): 160–2. doi:10.1016/j.medcli.2015.08.001. PMID 26434992.
  21. ↑ 21.0 21.1 Lisnevskaia L, Murphy G, Isenberg D (2014). "Systemic lupus erythematosus". Lancet. 384 (9957): 1878–88. doi:10.1016/S0140-6736(14)60128-8. PMID 24881804.
  22. ↑ de Carvalho JF, do Nascimento AP, Testagrossa LA, Barros RT, Bonfá E (2010). "Male gender results in more severe lupus nephritis". Rheumatol. Int. 30 (10): 1311–5. doi:10.1007/s00296-009-1151-9. PMID 19784840.
  23. ↑ Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M, Tao J, Zhang Y, Tan K, Li J, Chen Z, Ding X, Qian X, Da Z, Wang M, Sun L (2014). "Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus". Lupus. 23 (3): 327–34. doi:10.1177/0961203313513508. PMID 24297642.
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