Systemic lupus erythematosus natural history, complications and prognosis: Difference between revisions
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Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE involves many flare ups. SLE usually develops in the second and third decade of life, although it can present 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 patients. | Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE involves many flare ups. SLE usually develops in the second and third decade of life, although it can present 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 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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*[[Malignancies]] | *[[Malignancies]] | ||
=== Factors associated with flare up: | === Factors associated with flare up: <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> === | ||
* [[Stress]] (emotional, etc.) | * [[Stress]] (emotional, etc.) | ||
* [[Sunlight]] | * [[Sunlight]] | ||
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==Complications== | ==Complications== | ||
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are: | Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are: | ||
(Up arrows represent higher frequencies and down arrows represent lower frequencies) | (Up arrows represent higher frequencies and down arrows represent lower frequencies) | ||
{| class="wikitable" | {| class="wikitable" | ||
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==Prognosis== | ==Prognosis== | ||
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 more than 60% mortality rate during the mid-20th century. SLE is associated with a 10 year mortality of more than 50% among patients 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 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 more than 60% mortality rate during the mid-20th century. SLE is associated with a 10 year mortality of more than 50% among patients 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: | === Poor prognostic factors for SLE survival: === | ||
* Presence of [[nephritis]] (especially diffuse proliferative glomerulonephritis) | * Presence of [[nephritis]] (especially diffuse proliferative glomerulonephritis) | ||
* [[Hypertension]] | * [[Hypertension]] | ||
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** Increase in mortality rate | ** Increase in mortality rate | ||
=== SLE in men compared to women: | === SLE in men compared to women: === | ||
* Less [[photosensitivity]] | * Less [[photosensitivity]] | ||
* More [[serositis]] | * More [[serositis]] | ||
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* 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: === | ||
* Lower incidence of: | * Lower incidence of: | ||
** [[Malar rash]] | ** [[Malar rash]] | ||
Revision as of 20:56, 31 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 involves many flare ups. SLE usually develops in the second and third decade of life, although it can present 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 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: [1]
- Stress (emotional, etc.)
- Sunlight
- Ultraviolet light
- Infection
- Injuries
- Surgery
- Pregnancy
- Abrupt discontinuation of medications
- Treatment noncompliance
- Medications
- Immunizations
- Lupus nephritis
- Presence of neurologic complications
- Presence of vasculitis
- Elevated anti-dsDNA
- Low C3 level
Complications
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:
(Up arrows represent higher frequencies and down arrows represent lower frequencies)
| Organ | Disease | Description | Frequency |
|---|---|---|---|
| Gastrointestinal | Dysphagia |
|
↑↑↑ |
| Peptic ulcer disease |
|
↑ | |
| Intestinal pseudo-obstruction |
|
↓↓ | |
| Protein-losing enteropathy |
|
↓↓ | |
| Hepatitis |
|
↑ | |
| Acute pancreatitis |
|
↓ | |
| Mesenteric vasculitis |
|
↓↓ | |
| Acute cholecystitis |
|
↓↓ | |
| Pulmonary | Pleural disease |
|
↑ |
| Acute pneumonitis |
|
↓↓ | |
| Pulmonary hemorrhage |
|
↓↓ | |
| Pulmonary hypertension |
|
↑ | |
| Thromboembolic disease |
|
↑ | |
| Shrinking lung syndrome |
|
↓↓ | |
| Cardiac | Cardiomegaly | ↑↑ | |
| Valvular disease |
|
↑↑ | |
| Pericardial disease | ↓ | ||
| Myocarditis |
|
↓ | |
| Coronary artery disease |
|
↑↑ | |
| Neurological | Cognitive dysfunction |
|
↑ |
| Stroke |
|
↓ | |
| Seizures |
|
↑ | |
| Psychosis |
|
↑↑ | |
| Neuropathies |
|
↑↑ | |
| Musculoskeletal | Arthritis |
|
↑↑↑↑ |
| Osteonecrosis (Avascular necrosis) |
|
↓ | |
| Subcutaneous nodules |
|
↑ | |
| Osteoporosis |
|
↑ | |
| Skin | 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 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 more than 60% mortality rate during the mid-20th century. SLE is associated with a 10 year mortality of more than 50% among patients 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: [2]
- 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
References
- ↑ 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.
- ↑ 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.