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

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{{CMG}}; {{AE}}{{MIR}}
{{CMG}}; {{AE}}{{MIR}}
==Overview==
==Overview==
*'''First Sentences:'''
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.
:If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. Common complications of [disease name] include [complication 1], [complication 2], and [complication 3]. Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
:OR
:Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary.  However, the prognosis is generally regarded as poor/good/excellent.
*'''Examples:'''
:Example 1: If left untreated, 20% to 30% of patients with IgA nephropathy may progress to develop ESRD. Common complications of IgA nephropathy include pro-thrombotic states, such as stroke and myocardial infarction. Prognosis is generally good, and the 5-year mortality rate of patients with IgA nephropathy is approximately 5%.
*'''Additional Sentences:'''
:[Disease/malignancy] is associated with a 5 year survival rate of [#]%.
:The presence of metastasis is associated with a particularly poor prognosis among patients with [disease/malignancy]. The 5 year event free survival rate is less than [#]%.
:The [Subtype of disease or malignancy] is associated with the most favorable prognosis.
:The prognosis varies with the [characteristic] of tumor: [subtype of disease/malignancy] have the most favorable prognosis.
*'''Examples:'''
:Example 1: Rhabdomyosarcoma is associated with a 5 year survival rate of 72%.
:Example 2: The presence of metastasis is associated with a particularly poor prognosis among patients with rhabdomyosarcoma. The 5 year event free survival rate is less than 30%.
:Example 3: The embryonal subtype of rhabdomyosarcoma is associated with the most favorable prognosis.
:Example 4: The prognosis varies with the location of tumor: orbital and genitourinary tract rhabdomyosarcomas have the most favorable prognosis.
==Natural History==
==Natural History==
*Systemic lupus erythematosus is an autoimmune disease. There is no definite cause for the disease. SLE is a disease of waxing and waning, with a lot of flare up episodes. SLE usually develop in the second and third decade of life, although it can develop in 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:
*Developmental phase:
**Genetic mutations
**UV radiation exposure
**Smoking
*Preclinical phase:
**Mostly associated with auto-immune antibody production
***Autoantibodies common to other systemic autoimmune diseases
***proceeds with a more disease-specifi c clinically overt autoimmune phase
*Clinical phase:
**The phase due to damages of the auto-antibodies to the body tissues (mostly related to disease itself)
***Inflammation
***Involvement of first organs
***Flares
***Involvement of additional organs
***Early damages (e.g. alopecia, fixed erythema, cognitive dysfunction, valvular heart disease, avascular necrosis, tendon rupture, Jaccoud’s arthropathy, and osteoporosis)
*Comorbidity-complication phase:
**The phase of damages due to complications of longstanding disease, immunosuppressive therapy, and end organ damages (irreversible damages and complications)
***Infections
***Atherosclerosis
***Malignancies
==Complications==
*Using lists can be helpful for describing this section.
*You can use these template sentences;
**"Complications that can develop as a result of (disease name) are ___ (describe in list form)".
**"Complications that can develop as a result of the treatment of (disease name) are ___ (describe in list form).
**Next to each complication, provide a brief one sentence description detailing the complication.
*For an example of the complications section in a natural history, complications and prognosis page, click [[Pericarditis natural history#complications|here]].
*Pulmonary:
**Shrinking lung  ===== Pleuropulmonary manifestations =====
** pleuritis: considered one of the commons of thoracic manifestations 2
** acute lupus pneumonitis
** diaphragmatic dysfunction and shrinking lung syndrome
** cavitating pulmonary nodules
** pulmonary hypertension
** pulmonary vasculitis
** pulmonary embolism (often due to circulating anticardiolipin antibodies)
** pulmonary haemorrhage / alveolar haemorrhage (reflecting diffuse endothelial injury)
** chronic interstitial pneumonitis: may complicate 3-13% of patients
*** interlobular septal thickening 6
*** pulmonary fibrosis associated with this can occur but is rare 1
*** parenchymal bands
*** subpleural bands
*** bronchiectasis
*** bronchial wall thickening
*** pleural thickening
** bronchiolitis obliterans (with or without organising pneumonia)
** opportunistic pulmonary infection or drug toxicity from immunosuppressive therapy  ===== Cardiac complications =====
** pancarditis
*** Libman-Sacks endocarditis: sterile vegetations on the mitral and aortic valves (rare)


==== Symmetric polyarthritis ====
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>
Seen in 75-90% of patients with varying degrees of severity, it represents the most common presenting complaint clinically, usually worse in the morning. Areas of involvement most commonly include the small joints of the hand, knees, wrists, and shoulders.


===== Plain radiograph =====
===== Developmental phase: =====
Plain radiographs demonstrate soft tissue swelling of the involved joints, periarticular osteoporosis, and normal joint spaces. Carpal instability may be seen in 15% of patients 2.
*[[Genetic mutations]]
*[[UV radiation]] exposure
*Smoking
===== Preclinical phase: =====
*Mostly associated with [[auto-immune]] [[antibody]] production
*[[Autoantibodies]] common to other systemic autoimmune diseases
*Proceeds with a more disease-specific clinically overt [[Autoimmune disease|autoimmune phase]]
===== Clinical phase: =====
*The phase due to damages of the [[autoantibodies]] to the body tissues (mostly related to disease itself)
*[[Inflammation]]
*Involvement of first organs
*Flares
*Involvement of additional organs
*Early damages (e.g. [[alopecia]], [[Erythema|fixed erythema]], [[Cognitive disorder|cognitive dysfunction]], [[valvular heart disease]], [[avascular necrosis]], [[Tendonitis|tendon rupture]], [[Jaccoud's arthropathy|Jaccoud’s arthropathy]], and [[osteoporosis]])
===== Comorbidity-complication phase =====
The phase of damages due to complications of longstanding disease, [[immunosuppressive therapy]], and [[end organ damage]] (irreversible damages and complications)
*[[Infections]]
*[[Atherosclerosis]]
*[[Malignancies]]


==== Deforming non-erosive arthropathy ====
=== 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>  ===
When articular abnormalities are present, approximately 5-40% will develop a deforming non-erosive arthropathy due to ligamentous laxity (not articular destruction) and muscle contracture. This is more common in those with long-standing disease. In the hands this can be classically seen on Norgaard views but absent on AP views and are termed as reducible deformities.
* [[Stress]] (emotional, etc.)
* [[Sunlight]]
* [[Ultraviolet]] light
* [[Infection]]
* [[Injuries]]
* [[Surgery]]
* [[Pregnancy]]
* Abrupt discontinuation of medications
* Treatment noncompliance
* [[Medications]]
* [[Immunization|Immunizations]]
* [[Lupus nephritis]]
* Presence of [[Neurological disease|neurologic]] complications
* Presence of [[vasculitis]]
* Elevated [[anti-dsDNA]]
* Low C3 level


Due to their frequently reducible nature, deformities are seldom disabling.
==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>
===== Hands and feet =====
Symmetric involvement of interphalangeal joints is most common, showing swan neck and boutonniere deformities, subluxation with ulnar deviation at MCP joints, subluxation of the 1st metacarpophalangeal joint, a widened forefoot, and hallux valgus.
 
Joint space narrowing is uncommon, and when present is likely due to disuse atrophy or pressure from an adjacent subluxed bone. Altered stresses across the joint may also cause a "hook erosion" at the metacarpal heads due to capsular stress, mimicking findings of rheumatoid arthritis. This is more often observed on the radial side.
 
===== Spine =====
Spinal manifestations are uncommon and nonspecific, but a higher incidence of spinal findings is seen in those with deforming arthropathy. Up to 10% may have atlantoaxial subluxation/dislocation.
 
==== Myositis ====
Clinically observed in 30-50% of patients, true myositis occurs in approximately only 4% of patients. Elevated serum levels of muscle enzymes may or may not be observed.
 
==== Osteonecrosis ====
The most common location of osteonecrosis is the femoral head, but nearly any site may be affected. This is more commonly seen in younger patients and those with Raynaud phenomenon and other signs of vasculitis. This may also be seen as a complication of steroid therapy.


==== Spontaneous tendon weakening and rupture ====
<small>(Up arrows represent higher frequencies and down arrows represent lower frequencies)</small>
Typically observed about weight bearing joints as a complication of steroid therapy.
{| class="wikitable"
! style="background: #4479BA; color: #FFFFFF; " |Organ
! style="background: #4479BA; color: #FFFFFF; " |Disease
! style="background: #4479BA; color: #FFFFFF; " |Description
! style="background: #4479BA; color: #FFFFFF; " |Frequency
|-
| rowspan="8" |<small>Gastrointestinal</small>
| style="background: #DCDCDC; " align="center" |[[Dysphagia]]
|
* Usually due to an underlying [[esophageal motility disorder]]
* Concomitant [[gastroesophageal reflux disease]]
|↑↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Peptic ulcer disease]]
|
* Due to:
** The disease itself
** The adverse effect of SLE treatment
|↑
|-
| 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
* Obstructing the flow of intestinal contents
|↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Protein-losing enteropathy|Protein-losing enteropathy]]
|
* Occurs in patients with clinically severe SLE with multi-organ involvement
* Characterized by:
** [[Edema|Profound edema]]
** [[Hypoalbuminemia]]
* The absence of [[nephrotic]] range [[proteinuria]]
|↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Hepatitis]]
|
* May be due to:
** [[Drug-induced lupus erythematosus|Drug-induced damage]]
** [[Steatosis]]
** [[Viral hepatitis]] (concomitant with SLE)
** [[Thrombosis|Vascular thrombosis]]
** Overlap with [[autoimmune hepatitis]] due to SLE itself
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Acute pancreatitis|Acute pancreatitis]]
|
* Occurs usually in the setting of active SLE
|↓
|-
| style="background: #DCDCDC; " align="center" |[[Mesenteric vascular occlusion|Mesenteric vasculitis]]
|
* Mostly involve:
** Medium-sized branches of the [[Celiac artery|celiac artery]]
** [[Superior mesenteric artery|Superior mesenteric artery]] 
** [[Inferior mesenteric artery|Inferior mesenteric artery]]
* Features of [[mesenteric]] [[vasculitis]] include:
** [[Abdominal pain]]
** [[Gastrointestinal bleeding]]
** [[Intestinal ischemia]], [[infarction]], [[perforation]]
** [[Pancreatitis]]
** [[SBP|Primary spontaneous peritonitis]]:
*** An [[infection]] that develops in the [[peritoneum]] mainly due to lupus [[vasculitis]]
|↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Acute cholecystitis]]
|
* Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]]
* May progress to [[gangrene]], [[perforation]], and [[sepsis]]
|↓↓
|-
| rowspan="6" |<small>Pulmonary</small>
| style="background: #DCDCDC; " align="center" |[[Pleural disease|Pleural disease]]
|
* May lead to:
** [[Pleuritic chest pain]] with or without [[radiographic]] evidence of a [[pleural effusion]]
* [[Inflammation]] of the [[pleura]], the lining of the [[pleural cavity]], surrounding the [[Lung|lungs]]
* [[Pneumothorax]]: a collection of air within the [[pleural cavity]]
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Pneumonitis|Acute pneumonitis]]
|
* Fulminant form of diffuse lung injury
* Characterized by rapid onset with [[fever]], [[cough]], and [[Dyspnea|shortness of breath]]
|↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Pulmonary hemorrhage]]
|
* Pulmonary alveolar hemorrhage:
** Acutely ill patients with [[hemoptysis]], [[fever]], [[cough]], and [[hypoxemia]]
** Extensive blood loss
** Associated with high mortality rates of 70%–90%
|↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Pulmonary hypertension]]
|
* Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]]
* Characterized by [[Dypsnea|shortness of breath]], [[dizziness]], and [[faint]]
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Thromboembolic disease]]
|
* Chronic [[inflammation]] that increase the risk of [[Thromboembolic disease|thromboembolic events]]
|↑
|-
| style="background: #DCDCDC; " align="center" |Shrinking lung syndrome
|
* Characterized by [[dyspnea]] and [[Dyspnea|shortness of breath]] (estimated prevalence approximately 0.5%)
|↓↓
|-
| rowspan="5" |<small>Cardiac</small>
| style="background: #DCDCDC; " align="center" |[[Cardiomegaly]]
|
* Due to:
** [[Myocarditis]]
** [[Libman-Sacks endocarditis]]
** [[Subacute bacterial endocarditis]]
** [[Uremia]]
** [[Pulmonary arterial hypertension]] with [[right-sided heart failure]]
** [[Corticosteroid]]-related [[cardiomyopathy]]
|↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Valvular disease]]
|
* Different [[Valvular Diseases|valvular]] complications include:
** [[Libman-Sacks endocarditis|Libman-Sacks vegetations]]
** Valve thickening
** Valve regurgitation
* Mainly due to [[immunoglobulin]] and [[complement]] deposition in valvular structure
* Affect the [[mitral valve]] more frequently
|↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Pericardial disease]]
|
* [[Acute pericarditis]]
* [[Pericardial effusion]]
|↓
|-
| style="background: #DCDCDC; " align="center" |[[Myocarditis]]
|
* Characterized by:
** [[Chest pain]]
** [[ST segment elevation]]
** Elevated biomarkers of [[myonecrosis]]
** [[Heart failure]]
** [[Sudden death]]
* [[Myonecrosis]] may happen as a consequence of [[autoimmune]] reaction
|↓
|-
| 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
|↑↑
|-
| rowspan="5" |<small>Neurological</small>
| style="background: #DCDCDC; " align="center" |[[Cognitive-shifting|Cognitive dysfunction]]
|
* May be temporarily affected by multiple, transient [[metabolic]] and systemic processes
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Stroke]]
|
* Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]]
|↓
|-
| style="background: #DCDCDC; " align="center" |[[Seizure|Seizures]]
|
* May happen secondary to [[increased intracranial pressure]]:
** [[Hypercoagulability]] state (due to [[inflammation]])
** [[Thrombosis]] within the [[Cerebral venous sinus thrombosis|cerebral venous]]
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Psychosis]]
|
* Can indirectly influence [[Cognition|cognitive performance]]
* Mostly accompanied by [[Hallucination|hallucinations]] in SLE
|↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Neuropathies]]
|
* [[Peripheral neuropathy]]
* Mostly related to disease activity
* [[Central nervous system]] involvement association
* A predilection for asymmetric and [[lower extremities]] involvement, especially [[peroneal]] and [[Sural nerve|sural nerves]]
|↑↑
|-
| rowspan="4" |<small>Musculoskeletal</small>
| style="background: #DCDCDC; " align="center" |[[Arthritis]]
|
*Mostly symmetrical and non-erosive [[arthritis]]
*[[Arthralgias]]
*Effusions
*Decreased [[range of motion]] of both small and large joints
*Morning [[stiffness]]
|↑↑↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Osteonecrosis]] ([[Avascular necrosis]])
|
* Most common in the [[Femoral|femoral head]]
* Can involve [[Humerus|humeral head]], [[Tibial|tibial plateau]], and scaphoid navicular
* Usually [[bilateral]]
* Often [[asymptomatic]]
* [[Glucocorticoids|Glucocorticoids treatment]] is associated with the greatest risk of developing the disease
|↓
|-
| style="background: #DCDCDC; " align="center" |[[Subcutaneous tissue|Subcutaneous nodules]]
|
* Associated with active disease and flare ups
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Osteoporosis]]
|
*Mostly due to [[glucocorticoid]] usage
*Loss of height
*Sudden [[back pain]]
|↑
|-
| rowspan="5" |<small>Skin</small>
| 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
|↑
|-
| style="background: #DCDCDC; " align="center" |[[Photosensitivity]]
|
* Common theme for skin [[lesions]] associated with SLE
|↑↑↑
|-
| style="background: #DCDCDC; " align="center" |[[Alopecia|Non-scarring alopecia]]
|
* May occur at some point during the course of their disease
|↑
|-
| style="background: #DCDCDC; " align="center" |Oral and nasal ulcers
|
* Usually painless
|↑↑
|-
| style="background: #DCDCDC; " align="center" |Discoid lesions
|
* More [[inflammatory]]
* Have a tendency to [[scar]]
|↑
|-
| rowspan="2" |<small>Very rare disorders</small>
| style="background: #DCDCDC; " align="center" |[[Malignancy]]
|
* [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]]
* [[Lung cancer]]
* [[Liver mass|Liver cancer]]
* [[Vaginal cancer|Vulvar/vaginal cancer]]
* [[Thyroid cancer]]
|↓↓↓
|-
| style="background: #DCDCDC; " align="center" |[[Diabetes mellitus]]
|
* Increase predisposition to:
** [[Lupus nephritis]]
** [[Peripheral neuropathy]]
** [[Retinal disease]]
|↓
|}


==== Soft tissue calcification ====
Linear or nodular calcification in the subcutaneous and deep soft tissues may be seen, especially in the lower extremities.  Associations with diuretic therapy and vitamin D supplementation has been documented.
==== Insufficiency fracture ====
Those with SLE are at increased risk for insufficiency fracture, possibly due to disuse demineralization or osteopenia secondary to steroid therapy, or both.
==== Osteomyelitis and septic arthritis ====
Lupus patients are at increased risk of bacterial and mycotic infections, in large part due to steroid administration and renal disease. Osteomyelitis and septic arthritis involvement are less common than infection elsewhere.
*
==Prognosis==
==Prognosis==
The prognosis of systemic lupus erythematosis is ranging from a benign illness to an extremely rapid progressive disease that can lead to a fulminant organ failure and death poor/good with treatment. Without treatment, (disease name) will result in ___. SLE is associated with a 1/5/10 year mortality of __ among patient with ______ (for example high grade lesions). The presence of nephritis is associated with a particularly poor prognosis among patients with SLE.
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>
* SLE in men compared to women:
** Less photosensitivity
** More serositis
** Older age at diagnosis
** Higher 1 year mortality compared to women.
* SLE in the elderly (>65) compared to middle age prevalency:
** Lower incidence of:
*** Malar rash
*** Photosensitivity
*** Purpura
*** Alopecia
*** Raynaud’s phenomenon
*** Renal system involvement
*** Central nervous system involvement
** Greater prevalence of:
** Serositis
** Pulmonary involvement
** Sicca symptoms
** Musculoskeletal manifestations
 
*For an example of a prognosis section within a natural history, complications and prognosis page, click [[Pericarditis natural history#prognosis|here]].
OGNOSIS — Systemic lupus erythematosus (SLE) can run a varied clinical course, ranging from a relatively benign illness to a rapidly progressive disease with fulminant organ failure and death. The five-year survival rate in SLE has dramatically increased since the mid-20th century, from approximately 40 percent in the 1950s to greater than 90 percent since 1980s [121-125]. The improvement in patient survival is probably due to multiple factors including increased disease recognition with more sensitive diagnostic tests [126], earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications [127]. Despite these improvements, patients with SLE still have mortality rates ranging from two to five times higher than that of the general population [128].
 
Prognostic factors — Poor prognostic factors for survival in SLE include [122,123,129-137]:
 
●Renal disease (especially diffuse proliferative glomerulonephritis)
 
●Hypertension
 
●Male sex
 
●Young age
 
●Older age at presentation
 
●Low socioeconomic status
 
●Black race, which may primarily reflect low socioeconomic status
 
●Presence of antiphospholipid antibodies
 
●Antiphospholipid antibody syndrome
 
●High overall disease activity
 
Causes of death — The major causes of death in the first few years of illness are active disease (eg, central nervous system [CNS] and renal disease) or infection due to immunosuppression, while causes of late death include complications of SLE (eg, endstage renal disease), treatment complications, and cardiovascular disease [127,130,138-143]. The frequency of the different causes of death is best illustrated by a 2014 meta-analysis of 12 studies which included 27,123 patients with SLE (4993 observed deaths) [144]. This analysis reported an overall threefold increased risk of death in patients with SLE (standardized mortality rate [SMR] 2.98, 95% CI 2.32-3.83) when compared with the general population. The risks of death due to cardiovascular disease, infection, and renal disease were significantly increased. Mortality due to malignancy was not found to be increased, while patients with renal disease were found to have the highest mortality risk (SMR 7.90, 95% CI 5.5-11.0).
 
 
African Americans and Mexican Hispanics in the United States have a poorer renal prognosis than Caucasians, a finding not entirely independent of socioeconomic status [24]. African Americans are more likely to have anti-Sm, anti-RNP, discoid skin lesions, proteinuria, psychosis, and serositis [24-26]. African Americans and Latin Americans with lupus nephritis are also less likely to respond to cyclophosphamide treatment than Caucasians [27].
 
●The clinical status is poorer in those with less education [24,28]; this effect may reflect poor compliance [29]. Clinical status is also poorer in those with lower socioeconomic status and with inadequate access to medical care [30].
 
●The extent and degree of activity of SLE varies in different countries and in different ethnic groups [30-32].
 
●Men with lupus tend to have higher frequencies of renal disease, skin manifestations, cytopenias, serositis, neurologic involvement, thrombosis, cardiovascular disease, hypertension, and vasculitis than women [33]. By contrast, Raynaud phenomenon, photosensitivity, and mucosal ulceration are less frequent manifestations in men than women. Most, but not all studies suggest that men have a higher one-year mortality rate [33-38].
 
●SLE in children tends to be symptomatically more severe than in adults, with a high incidence of malar rashes, nephritis, pericarditis, hepatosplenomegaly, and hematologic abnormalities [22,34].
 
Lupus tends to be milder in older adults, who often have a presentation more similar to that of drug-induced lupus. Clinical features of lupus in older patients include the following [34,39-41]:


●A lower ratio of affected women to men than for younger patients
=== 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]])
* [[Hypertension]]
* Male sex
* Young age
* Older age at presentation
* Low socioeconomic status
* Black race: Higher rate of [[nephritis]]
* Presence of [[antiphospholipid antibodies]]
* High overall disease activity


●Lower incidence of malar rash, photosensitivity, purpura, alopecia, Raynaud phenomenon, renal, central nervous system, and hematologic involvement
=== 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: =====
* [[Lupus nephritis]]
* Progression to [[end-stage renal disease]]
* Increased disease severity
* Damage or poor survival


●Lower prevalence of anti-La, anti-Sm, and anti-RNP antibodies and of hypocomplementemia
===== Antiphospholipid antibodies correlated with: =====
* Features of the [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]] 
* CNS involvement
* Severe [[lupus nephritis]]
* Increase in mortality rate


●Greater prevalence of sicca symptoms, serositis, pulmonary involvement, and musculoskeletal manifestations
=== 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]]
* More [[serositis]]
* Older age at diagnosis
* Higher 1 year mortality compared to women


●Greater prevalence of rheumatoid factor
=== 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:
** [[Malar rash]]
** [[Photosensitivity]]
** [[Purpura]]
** [[Alopecia]]
** [[Raynaud’s phenomenon]]
** [[Nephritis]]
** [[Central nervous system]] involvement
* Greater prevalence of:
** [[Serositis]]
** [[Pulmonary]] involvement
** [[Sicca syndrome]]
** [[Musculoskeletal]] manifestations


==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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