Systemic lupus erythematosus natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
*'''First Sentences:''' | |||
: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== | |||
*The natural history of disease details how the disease progresses without treatment. | |||
*Here are a few template sentences you can use: "The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. The symptoms of (disease name) typically develop ___ years after exposure to ___. Without treatment, the patient will develop symptoms of ___, which will/ may eventually lead to ___. | |||
==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]]. | |||
==Prognosis== | |||
The prognosis of systemic lupus erythematosis is poor/good with treatment. Without treatment, (disease name) will result in ___. (Disease name) is associated with a 1/5/10 year mortality of __ among patient with ______ (for example high grade lesions). The presence of ___ is associated with a particularly poor prognosis among patients with (disease name). | |||
*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 | |||
●Lower incidence of malar rash, photosensitivity, purpura, alopecia, Raynaud phenomenon, renal, central nervous system, and hematologic involvement | |||
●Lower prevalence of anti-La, anti-Sm, and anti-RNP antibodies and of hypocomplementemia | |||
●Greater prevalence of sicca symptoms, serositis, pulmonary involvement, and musculoskeletal manifestations | |||
●Greater prevalence of rheumatoid factor | |||
==References== | ==References== | ||
[[Reflist|2]] | [[Reflist|2]] | ||
Revision as of 12:53, 28 June 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
- First Sentences:
- 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
- The natural history of disease details how the disease progresses without treatment.
- Here are a few template sentences you can use: "The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. The symptoms of (disease name) typically develop ___ years after exposure to ___. Without treatment, the patient will develop symptoms of ___, which will/ may eventually lead to ___.
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 here.
Prognosis
The prognosis of systemic lupus erythematosis is poor/good with treatment. Without treatment, (disease name) will result in ___. (Disease name) is associated with a 1/5/10 year mortality of __ among patient with ______ (for example high grade lesions). The presence of ___ is associated with a particularly poor prognosis among patients with (disease name).
- For an example of a prognosis section within a natural history, complications and prognosis page, click 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
●Lower incidence of malar rash, photosensitivity, purpura, alopecia, Raynaud phenomenon, renal, central nervous system, and hematologic involvement
●Lower prevalence of anti-La, anti-Sm, and anti-RNP antibodies and of hypocomplementemia
●Greater prevalence of sicca symptoms, serositis, pulmonary involvement, and musculoskeletal manifestations
●Greater prevalence of rheumatoid factor