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 | 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. | ||
==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]]. 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. | ||
*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: | ||
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*Mostly associated with [[auto-immune]] [[antibody]] production | *Mostly associated with [[auto-immune]] [[antibody]] production | ||
*[[Autoantibodies]] common to other systemic autoimmune diseases | *[[Autoantibodies]] common to other systemic autoimmune diseases | ||
*Proceeds with a more disease-specific clinically overt autoimmune phase | *Proceeds with a more disease-specific clinically overt [[Autoimmune disease|autoimmune phase]] | ||
===== Clinical phase: ===== | ===== Clinical phase: ===== | ||
*The phase due to damages of the | *The phase due to damages of the [[autoantibodies]] to the body tissues (mostly related to disease itself) | ||
*[[Inflammation]] | *[[Inflammation]] | ||
*Involvement of first organs | *Involvement of first organs | ||
*Flares | *Flares | ||
*Involvement of additional organs | *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) | *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 ===== | ===== 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) | |||
*Infections | *[[Infections]] | ||
*[[Atherosclerosis]] | *[[Atherosclerosis]] | ||
*[[Malignancies]] | *[[Malignancies]] | ||
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* [[Ultraviolet]] light | * [[Ultraviolet]] light | ||
* [[Infection]] | * [[Infection]] | ||
* Injuries | * [[Injuries]] | ||
* [[Surgery]] | * [[Surgery]] | ||
* [[Pregnancy]] | * [[Pregnancy]] | ||
* Abrupt discontinuation of medications | * Abrupt discontinuation of medications | ||
* Treatment noncompliance | * Treatment noncompliance | ||
* Medications | * [[Medications]] | ||
* Immunizations | * [[Immunization|Immunizations]] | ||
==Complications== | ==Complications== | ||
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|[[Dysphagia]] | |[[Dysphagia]] | ||
| | | | ||
* | * Usually due to an underlying [[esophageal motility disorder]] | ||
| | * Concomitant [[gastroesophageal reflux disease]] | ||
|↑↑ | |||
|- | |- | ||
|[[Peptic ulcer disease]] | |[[Peptic ulcer disease]] | ||
| | | | ||
* | * Due to | ||
| | ** The disease itself | ||
** The effect of SLE treatment | |||
|↑ | |||
|- | |- | ||
|[[Intestinal pseudo-obstruction|Intestinal pseudo-obstruction]] | |[[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 | ||
|↓↓ | |||
|- | |- | ||
|[[Protein-losing enteropathy|Protein-losing enteropathy]] | |[[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]] | |||
|↓ | |||
|- | |- | ||
|[[Hepatitis]] | |[[Hepatitis]] | ||
| | | | ||
* May be due to | * 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 | |||
|↑ | |||
|- | |- | ||
|[[Acute pancreatitis|Acute pancreatitis]] | |[[Acute pancreatitis|Acute pancreatitis]] | ||
| | | | ||
* | * Occurs usually in the setting of active SLE | ||
| | |↓ | ||
|- | |- | ||
|[[Mesenteric vascular occlusion|Mesenteric vasculitis]] | |[[Mesenteric vascular occlusion|Mesenteric vasculitis]] | ||
| | | | ||
* | * Mostly involve: | ||
* [[SBP|Primary spontaneous peritonitis]]: | ** 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]] | |||
| | | | ||
|- | |- | ||
|[[Acute cholecystitis]] | |[[Acute cholecystitis]] | ||
| | | | ||
* Due to periarterial [[fibrosis]] and acute vasculitis | * Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]] | ||
* | * May progress to [[gangrene]], [[perforation]], and [[sepsis]] | ||
| | | | ||
|- | |- | ||
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|[[Pleural disease|Pleural disease]] | |[[Pleural disease|Pleural disease]] | ||
| | | | ||
* | * May lead to: | ||
* [[Pneumothorax]]: a collection of air within the pleural cavity | ** [[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]] | |||
| | | | ||
|- | |- | ||
|[[Pneumonitis|Acute pneumonitis]] | |[[Pneumonitis|Acute pneumonitis]] | ||
| | | | ||
* | * Fulminant form of diffuse lung injury | ||
| | * Characterized by rapid onset with [[fever]], [[cough]], and [[Dyspnea|shortness of breath]] | ||
|↓↓ | |||
|- | |- | ||
|[[Pulmonary hemorrhage]] | |[[Pulmonary hemorrhage]] | ||
| | | | ||
* Pulmonary alveolar hemorrhage: | * Pulmonary alveolar hemorrhage: | ||
** Acutely ill patients with [[hemoptysis]], [[fever]], [[cough]], and [[hypoxemia]] | |||
** Acutely ill patients with [[hemoptysis]], [[fever]], [[cough]], and [[hypoxemia]] | ** Extensive blood loss | ||
** | |||
** Associated with high mortality rates of 70%–90% | ** Associated with high mortality rates of 70%–90% | ||
| | |↓↓ | ||
|- | |- | ||
|[[Pulmonary hypertension]] | |[[Pulmonary hypertension]] | ||
| | | | ||
* Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]] | * Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]] | ||
| | * Characterized by [[Dypsnea|shortness of breath]], [[dizziness]], and [[faint]] | ||
|↑ | |||
|- | |- | ||
|[[Thromboembolic disease]] | |[[Thromboembolic disease]] | ||
| | | | ||
* Chronic [[inflammation]] | * Chronic [[inflammation]] that increase the risk of [[Thromboembolic disease|thromboembolic events]] | ||
| | | | ||
|- | |- | ||
|Shrinking lung syndrome | |Shrinking lung syndrome | ||
| | | | ||
* | * Characterized by [[dyspnea]] and [[Dyspnea|shortness of breath]] (estimated prevalence approximately 0.5%) | ||
| | |↓↓ | ||
|- | |- | ||
| rowspan="5" |Cardiac involvement | | rowspan="5" |Cardiac involvement | ||
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** [[Subacute bacterial endocarditis]] | ** [[Subacute bacterial endocarditis]] | ||
** [[Uremia]] | ** [[Uremia]] | ||
** [[Pulmonary arterial hypertension]] with right-sided heart failure | ** [[Pulmonary arterial hypertension]] with [[right-sided heart failure]] | ||
** [[Corticosteroid]]-related [[cardiomyopathy]] | ** [[Corticosteroid]]-related [[cardiomyopathy]] | ||
| | | | ||
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|[[Valvular disease]] | |[[Valvular disease]] | ||
| | | | ||
* Different [[Valvular Diseases|valvular]] complications include: | |||
* Different valvular complications include Libman-Sacks vegetations | ** [[Libman-Sacks endocarditis|Libman-Sacks vegetations]] | ||
* More frequently affect mitral valve | ** Valve thickening | ||
| | ** Valve regurgitation | ||
* Mainly due to [[Immunoglobulin]] and [[complement]] deposition in valvular structure | |||
* More frequently affect [[mitral valve]] | |||
|↑↑ | |||
|- | |- | ||
|[[Pericardial disease]] | |[[Pericardial disease]] | ||
| | | | ||
* Acute pericarditis | * [[Acute pericarditis]] | ||
* Pericardial effusion | * [[Pericardial effusion]] | ||
| | | | ||
|- | |- | ||
|[[Myocarditis]] | |[[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 | |||
| | | | ||
|- | |- | ||
|[[Coronary heart disease|Coronary artery disease]] | |[[Coronary heart disease|Coronary artery disease]] | ||
| | | | ||
* | * Mainly as a result of increased risk of [[Atheroma|atheromatous plaques]] due to autoimmune status of SLE | ||
|↑↑ | |↑↑ | ||
|- | |- | ||
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|[[Cognitive-shifting|Cognitive dysfunction]] | |[[Cognitive-shifting|Cognitive dysfunction]] | ||
| | | | ||
* | * May be temporarily affected by multiple, transient [[metabolic]] and systemic processes | ||
| | | | ||
|- | |- | ||
|[[Stroke]] | |[[Stroke]] | ||
| | | | ||
* | * Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]] | ||
| | | | ||
|- | |- | ||
|[[Seizure|Seizures]] | |[[Seizure|Seizures]] | ||
| | | | ||
* | * May happen secondary to [[increased intracranial pressure]]: | ||
** [[Hypercoagulability]] state (due to inflammation) | |||
** [[Thrombosis]] within the [[Cerebral venous sinus thrombosis|cerebral venous]] | |||
| | | | ||
|- | |- | ||
|[[Psychosis]] | |[[Psychosis]] | ||
| | | | ||
* | * Can indirectly influence [[Cognition|cognitive performance]] | ||
| | * Mostly accompanied by [[Hallucination|hallucinations]] in SLE | ||
|↑↑ | |||
|- | |- | ||
|[[Neuropathies]] | |[[Neuropathies]] | ||
| | | | ||
* Peripheral neuropathy | * [[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]] | |||
| | | | ||
|- | |- | ||
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|[[Arthritis]] | |[[Arthritis]] | ||
| | | | ||
*Mostly symmetrical and non-erosive | *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]]) | |[[Osteonecrosis]] ([[Avascular necrosis]]) | ||
| | | | ||
* Most common in the femoral head | * Most common in the [[Femoral|femoral head]] | ||
* Can involve humeral head, tibial plateau, and scaphoid navicular | * Can involve [[Humerus|humeral head]], [[Tibial|tibial plateau]], and scaphoid navicular | ||
* Usually bilateral | * Usually [[bilateral]] | ||
* Glucocorticoids treatment is associated with the greatest risk of developing the disease | * Often [[asymptomatic]] | ||
* [[Glucocorticoids|Glucocorticoids treatment]] is associated with the greatest risk of developing the disease | |||
| | | | ||
|- | |- | ||
|Subcutaneous nodules | |[[Subcutaneous tissue|Subcutaneous nodules]] | ||
| | | | ||
* | * Associated with active disease | ||
| | | | ||
|- | |- | ||
|Osteoporosis | |[[Osteoporosis]] | ||
| | | | ||
*Mostly due to [[glucocorticoid]] usage | *Mostly due to [[glucocorticoid]] usage | ||
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| | | | ||
|- | |- | ||
|Skin disorder | | rowspan="5" |Skin disorder | ||
|[[Cutaneous lupus erythematosus]] | |[[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]] | |||
| | | | ||
* Common theme for skin lesions associated with SLE | |||
|↑↑ | |||
| | |||
|- | |- | ||
|[[Alopecia|Non-scarring alopecia]] | |||
| | | | ||
* May occur at some point during the course of their disease | |||
| | | | ||
|- | |- | ||
|oral and/or nasal ulcers | |||
| | | | ||
* Usually painless | |||
| | | | ||
|- | |- | ||
|discoid lesions | |||
| | | | ||
* More inflammatory | |||
* Have a tendency to scar | |||
| | | | ||
|- | |- | ||
|Very rare disorders | | rowspan="3" |Very rare disorders | ||
|Malignancy | |Malignancy | ||
| | | | ||
| | | | ||
|- | |- | ||
|Diabetes mellitus | |Diabetes mellitus | ||
| | | | ||
| | | | ||
|- | |- | ||
|Premature gonadal failure | |Premature gonadal failure | ||
| | | |
Revision as of 04:07, 26 July 2017
Systemic lupus erythematosus Microchapters |
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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 course can be divided into 4 subcategories based on the course of the disease: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.
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.
- 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-specific clinically overt 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, 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)
Factors associated with flare up:
- Stress (emotional etc.)
- Sunlight
- Ultraviolet light
- Infection
- Injuries
- Surgery
- Pregnancy
- Abrupt discontinuation of medications
- Treatment noncompliance
- Medications
- Immunizations
Complications
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:
Organ | Disease | Description | Frequency |
---|---|---|---|
Gastrointestinal system | Dysphagia |
|
↑↑ |
Peptic ulcer disease |
|
↑ | |
Intestinal pseudo-obstruction |
|
↓↓ | |
Protein-losing enteropathy |
|
↓ | |
Hepatitis |
|
↑ | |
Acute pancreatitis |
|
↓ | |
Mesenteric vasculitis |
|
||
Acute cholecystitis |
|
||
Pulmonary involvement | Pleural disease |
|
|
Acute pneumonitis |
|
↓↓ | |
Pulmonary hemorrhage |
|
↓↓ | |
Pulmonary hypertension |
|
↑ | |
Thromboembolic disease |
|
||
Shrinking lung syndrome |
|
↓↓ | |
Cardiac involvement | Cardiomegaly | ||
Valvular disease |
|
↑↑ | |
Pericardial disease | |||
Myocarditis |
|
||
Coronary artery disease |
|
↑↑ | |
Neurological involvement | Cognitive dysfunction |
|
|
Stroke |
|
||
Seizures |
|
||
Psychosis |
|
↑↑ | |
Neuropathies |
|
||
Musculoskeletal involvement | Arthritis |
|
↑↑ |
Osteonecrosis (Avascular necrosis) |
|
||
Subcutaneous nodules |
|
||
Osteoporosis |
|
||
Skin disorder | Cutaneous lupus erythematosus |
|
|
Photosensitivity |
|
↑↑ | |
Non-scarring alopecia |
|
||
oral and/or nasal ulcers |
|
||
discoid lesions |
|
||
Very rare disorders | Malignancy | ||
Diabetes mellitus | |||
Premature gonadal failure |
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 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.
Poor prognostic factors for SLE survival:
- 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
Prognosis markers:
- Serum anti ds-DNA titres correlated with:
- Lupus nephritis
- Progression to end-stage renal disease
- Increased disease severity
- Damage or poor survival
- Antiphospholipid antibodies correlated with:
- Features of the antiphospholipid syndrome (APS) (arterial/ venous thrombosis, fetal loss, thrombocytopenia)
- CNS involvement (especially cerebrovascular disease)
- Severe lupus nephritis
- Damage accrual
- Increase in mortality rate
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