Systemic lupus erythematosus natural history, complications and prognosis: Difference between revisions
Line 58: | Line 58: | ||
* Usually due to an underlying [[esophageal motility disorder]] | * Usually due to an underlying [[esophageal motility disorder]] | ||
* Concomitant [[gastroesophageal reflux disease]] | * Concomitant [[gastroesophageal reflux disease]] | ||
| | |↑↑↑ | ||
|- | |- | ||
|[[Peptic ulcer disease]] | |[[Peptic ulcer disease]] | ||
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** [[Hypoalbuminemia]] | ** [[Hypoalbuminemia]] | ||
* The absence of [[nephrotic]] range [[proteinuria]] | * The absence of [[nephrotic]] range [[proteinuria]] | ||
| | |↓↓ | ||
|- | |- | ||
|[[Hepatitis]] | |[[Hepatitis]] | ||
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** [[SBP|Primary spontaneous peritonitis]]: | ** [[SBP|Primary spontaneous peritonitis]]: | ||
*** An [[infection]] that develops in the [[peritoneum]] mainly due to lupus [[vasculitis]] | *** An [[infection]] that develops in the [[peritoneum]] mainly due to lupus [[vasculitis]] | ||
| | |↓↓ | ||
|- | |- | ||
|[[Acute cholecystitis]] | |[[Acute cholecystitis]] | ||
Line 116: | Line 116: | ||
* Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]] | * Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]] | ||
* May progress to [[gangrene]], [[perforation]], and [[sepsis]] | * May progress to [[gangrene]], [[perforation]], and [[sepsis]] | ||
| | |↓↓ | ||
|- | |- | ||
| rowspan="6" |Pulmonary involvement | | rowspan="6" |Pulmonary involvement | ||
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* [[Inflammation]] of the [[pleura]], the lining of the [[pleural cavity]], surrounding the [[Lung|lungs]] | * [[Inflammation]] of the [[pleura]], the lining of the [[pleural cavity]], surrounding the [[Lung|lungs]] | ||
* [[Pneumothorax]]: a collection of air within the [[pleural cavity]] | * [[Pneumothorax]]: a collection of air within the [[pleural cavity]] | ||
| | |↑ | ||
|- | |- | ||
|[[Pneumonitis|Acute pneumonitis]] | |[[Pneumonitis|Acute pneumonitis]] | ||
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| | | | ||
* 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 | |Shrinking lung syndrome | ||
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** [[Pulmonary arterial hypertension]] with [[right-sided heart failure]] | ** [[Pulmonary arterial hypertension]] with [[right-sided heart failure]] | ||
** [[Corticosteroid]]-related [[cardiomyopathy]] | ** [[Corticosteroid]]-related [[cardiomyopathy]] | ||
| | |↑↑ | ||
|- | |- | ||
|[[Valvular disease]] | |[[Valvular disease]] | ||
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* [[Acute pericarditis]] | * [[Acute pericarditis]] | ||
* [[Pericardial effusion]] | * [[Pericardial effusion]] | ||
| | |↓ | ||
|- | |- | ||
|[[Myocarditis]] | |[[Myocarditis]] | ||
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** [[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]] | |[[Coronary heart disease|Coronary artery disease]] | ||
Line 205: | Line 205: | ||
| | | | ||
* May be temporarily affected by multiple, transient [[metabolic]] and systemic processes | * 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]] | * Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]] | ||
| | |↓ | ||
|- | |- | ||
|[[Seizure|Seizures]] | |[[Seizure|Seizures]] | ||
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** [[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]] | |[[Psychosis]] | ||
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* [[Central nervous system]] involvement association | * [[Central nervous system]] involvement association | ||
* A predilection for asymmetric and [[lower extremities]] involvement, especially [[peroneal]] and [[Sural nerve|sural nerves]] | * A predilection for asymmetric and [[lower extremities]] involvement, especially [[peroneal]] and [[Sural nerve|sural nerves]] | ||
| | |↑↑ | ||
|- | |- | ||
| rowspan="4" |Musculoskeletal involvement | | rowspan="4" |Musculoskeletal involvement | ||
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*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]]) | ||
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* Often [[asymptomatic]] | * Often [[asymptomatic]] | ||
* [[Glucocorticoids|Glucocorticoids treatment]] is associated with the greatest risk of developing the disease | * [[Glucocorticoids|Glucocorticoids treatment]] is associated with the greatest risk of developing the disease | ||
| | |↓ | ||
|- | |- | ||
|[[Subcutaneous tissue|Subcutaneous nodules]] | |[[Subcutaneous tissue|Subcutaneous nodules]] | ||
| | | | ||
* Associated with active disease | * Associated with active disease and flare ups | ||
| | |↑ | ||
|- | |- | ||
|[[Osteoporosis]] | |[[Osteoporosis]] | ||
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*Loss of height | *Loss of height | ||
*Sudden back pain | *Sudden back pain | ||
| | |↑ | ||
|- | |- | ||
| rowspan="5" |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]] | |[[Photosensitivity]] | ||
| | | | ||
* Common theme for skin lesions associated with SLE | * Common theme for skin [[lesions]] associated with SLE | ||
| | |↑↑↑ | ||
|- | |- | ||
|[[Alopecia|Non-scarring alopecia]] | |[[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 | |Oral and nasal ulcers | ||
| | | | ||
* Usually painless | * Usually painless | ||
| | |↑↑ | ||
|- | |- | ||
|Discoid lesions | |Discoid lesions | ||
| | | | ||
* More inflammatory | * More [[inflammatory]] | ||
* Have a tendency to scar | * Have a tendency to [[scar]] | ||
| | |↑ | ||
|- | |- | ||
| rowspan="2" |Very rare disorders | | rowspan="2" |Very rare disorders | ||
|[[Malignancy]] | |[[Malignancy]] | ||
| | | | ||
* [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]] | * [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]] | ||
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* [[Vaginal cancer|Vulvar/vaginal cancer]] | * [[Vaginal cancer|Vulvar/vaginal cancer]] | ||
* [[Thyroid cancer]] | * [[Thyroid cancer]] | ||
| | |↓↓↓ | ||
|- | |- | ||
|[[Diabetes mellitus]] | |[[Diabetes mellitus]] | ||
| | | | ||
* Increase predisposition to: | |||
** [[Lupus nephritis]] | |||
** [[Peripheral neuropathy]] | |||
** [[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 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. | 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. | ||
=== Poor prognostic factors for SLE survival: === | |||
* Presence of nephritis (especially diffuse proliferative glomerulonephritis) | * Presence of [[nephritis]] (especially diffuse proliferative glomerulonephritis) | ||
* Hypertension | * [[Hypertension]] | ||
* Male sex | * Male sex | ||
* Young age | * Young age | ||
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* 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: === | |||
** Lupus nephritis | ===== Serum anti ds-DNA titres correlated with: ===== | ||
** Progression to end-stage renal disease | ** [[Lupus nephritis]] | ||
** Progression to [[end-stage renal disease]] | |||
** Increased disease severity | ** Increased disease severity | ||
** Damage or poor survival | ** Damage or poor survival | ||
** Features of the antiphospholipid syndrome (APS) | ===== Antiphospholipid antibodies correlated with: ===== | ||
** CNS involvement | ** Features of the [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]] | ||
** Severe lupus nephritis | ** CNS involvement | ||
** Severe [[lupus nephritis]] | |||
** Increase in mortality rate | ** Increase in mortality rate | ||
=== SLE in men compared to women: === | |||
* Less photosensitivity | * Less [[photosensitivity]] | ||
* More serositis | * More [[serositis]] | ||
* Older age at diagnosis | * Older age at diagnosis | ||
* Higher 1 year mortality compared to women | * Higher 1 year mortality compared to women | ||
=== SLE in the elderly (>65) compared to middle age prevalency: === | |||
* Lower incidence of: | * Lower incidence of: | ||
** Malar rash | ** [[Malar rash]] | ||
** Photosensitivity | ** [[Photosensitivity]] | ||
** Purpura | ** [[Purpura]] | ||
** Alopecia | ** [[Alopecia]] | ||
** Raynaud’s phenomenon | ** [[Raynaud’s phenomenon]] | ||
** | ** [[Nephritis]] | ||
** Central nervous system involvement | ** [[Central nervous system]] involvement | ||
* Greater prevalence of: | * Greater prevalence of: | ||
** Serositis | ** [[Serositis]] | ||
** Pulmonary involvement | ** [[Pulmonary]] involvement | ||
** Sicca | ** [[Sicca syndrome]] | ||
** Musculoskeletal manifestations | ** [[Musculoskeletal]] manifestations | ||
==References== | ==References== | ||
[[Reflist|2]] | [[Reflist|2]] | ||
<references /> | <references /> |
Revision as of 05:24, 26 July 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 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 nasal ulcers |
|
↑↑ | |
Discoid lesions |
|
↑ | |
Very rare disorders | Malignancy | ↓↓↓ | |
Diabetes mellitus |
|
↓ |
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:
- Lupus nephritis
- Progression to end-stage renal disease
- Increased disease severity
- Damage or poor survival
- Features of the antiphospholipid syndrome (APS)
- CNS involvement
- Severe lupus nephritis
- 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:
- Greater prevalence of:
- Serositis
- Pulmonary involvement
- Sicca syndrome
- Musculoskeletal manifestations