Systemic lupus erythematosus: Difference between revisions

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Systemic lupus erythematosus (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening.

'''Systemic lupus erythematosus''' (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening.

SLE affects 20 – 100/100 000 in Europe. It more frequently affects women in the ages 16 – 55, being 12x more common in women. Affected men have a worse prognosis. SLE more frequently affects Black and Hispanic ethnicities than White. The 10-year survival rate is 90%.

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* HLA-DR2 or -DR3

* High oestrogenic state (oral contraceptives, endometriosis)

* High oestrogenic state (oral [[Contraceptive|contraceptives]], [[endometriosis]])

* Smoking

* [[Smoking]]

* Certain drugs

Drug-induced lupus erythematosus is an SLE-like syndrome caused by certain drugs. The most commonly identified drugs are procainamide, hydralazine, and <abbr>TNF</abbr>-α inhibitors. The first two have the highest risk but aren’t widely used anymore.

[[Drug-induced lupus erythematosus]] is an SLE-like syndrome caused by certain drugs. The most commonly identified drugs are procainamide, hydralazine, and <abbr>TNF</abbr>-α inhibitors. The first two have the highest risk but aren’t widely used anymore.

== Pathomechanism ==

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The most classical skin symptom in SLE is the malar rash (butterfly rash), which is an erythematous rash on both malar eminences which spares the nasolabial folds. Other skin symptoms include Raynaud phenomenon, photosensitivity, discoid rash, oral ulcers, and alopecia.

The most common musculoskeletal symptom is non-erosive symmetrical polyarthritis affecting distal joints.

The most common musculoskeletal symptom is non-erosive symmetrical [[polyarthritis]] affecting distal joints.

The renal manifestation of lupus is called lupus nephritis, which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome.

The renal manifestation of lupus is called [[lupus nephritis]], which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome.

CNS manifestations may be anything from headaches to psychosis and seizures. Cardiovascular manifestations may be myocarditis, valvular disease, and accelerated atherosclerosis. Pulmonary manifestations include interstitial lung disease, pneumonitis, and pulmonary hypertension. Any serous membrane may be inflamed.

CNS manifestations may be anything from headaches to [[psychosis]] and [[Seizure|seizures]]. Cardiovascular manifestations may be [[myocarditis]], valvular disease, and accelerated [[atherosclerosis]]. Pulmonary manifestations include [[interstitial lung disease]], [[pneumonitis]], and [[pulmonary hypertension]]. Any serous membrane may be inflamed.

Any haematological cell line may be affected, causing anaemia, leukopaenia, lymphocytopaenia, or thrombocytopaenia. <abbr>GI</abbr> manifestations include abdominal pain, peritonitis, ascites, pancreatitis, and hepatitis.

Any haematological cell line may be affected, causing [[anaemia]], [[leukopaenia]], [[lymphocytopaenia]], or [[thrombocytopaenia]]. <abbr>GI</abbr> manifestations include abdominal pain, [[peritonitis]], [[ascites]], [[Acute pancreatitis|pancreatitis]], and [[hepatitis]].

== Diagnosis and evaluation ==

SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an ANA titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive).

SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an [[ANA]] titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive).

Typical laboratory findings include:

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* Leukocytopaenia

* Thrombocytopaenia

* Autoimmune haemolysis

* Autoimmune [[haemolysis]]

* Elevated ESR (normal <abbr>CRP</abbr>)

* Elevated [[ESR]] (normal <abbr>[[C-reactive protein|CRP]]</abbr>)

* Decreased level of complement

* ANA titre > 1:80

* [[ANA]] titre > 1:80

* Presence of antiphospholipid antibodies

* Presence of [[Antiphospholipid antibody|antiphospholipid antibodies]]

** Anti-cardiolipin

** Lupus anticoagulant

* Presence of SLE-specific antibodies

** Anti-dsDNA

** [[Anti-dsDNA]]

** Anti-Sm

** [[Anti-Sm]]

== Treatment ==

For the best possible treatment of SLE, it’s important to determine the severity of the disease. There exist countless indexes for this purpose, but the most frequently used is the SLEDAI-2K index. The presence of renal or CNS manifestations necessitates more intensive treatment, and so detection of these complications is important.

Important lifestyle changes include appropriate sun protection, smoke cessation, exercise, and adherence to vaccination protocols. NSAIDs can provide symptomatic relief.

Important lifestyle changes include appropriate sun protection, smoke cessation, exercise, and adherence to vaccination protocols. [[NSAID|NSAIDs]] can provide symptomatic relief.

Pharmacological treatment can be considered in three different forms: induction of remission, maintenance of remission, and supportive treatment. Side effects of pharmacological therapy is unfortunately a major cause of SLE-related morbidity and mortality, mostly due to ~~glucocorticoids~~.

Pharmacological treatment can be considered in three different forms: induction of remission, maintenance of remission, and supportive treatment. Side effects of pharmacological therapy is unfortunately a major cause of SLE-related morbidity and mortality, mostly due to [[corticosteroids]].

For induction of remission, ~~glucocorticoids~~ are used. In cases of renal or CNS affection, other immunosuppressants like cyclophosphamide or mycophenolate mofetil may also be necessary.

For induction of remission, corticosteroids are used. In cases of renal or CNS affection, other immunosuppressants like [[cyclophosphamide]] or [[mycophenolate mofetil]] may also be necessary.

For maintenance of remission, hydroxychloroquine is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like azathioprine, methotrexate, and sometimes even long-term low-dose glucocorticoids, may be required. Belimumab and rituximab are the only biological therapies indicated for SLE.

For maintenance of remission, [[hydroxychloroquine]] is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like [[azathioprine]], [[methotrexate]], and sometimes even long-term low-dose glucocorticoids, may be required. [[Belimumab]] and [[rituximab]] are the only biological therapies indicated for SLE.

Supportive treatment includes medications like ACE inhibitors, ARBs, statins, antiepileptics, bisphosphonates, etc.

[[Category:Rheumatology]]

@@ Line 1: / Line 1: @@
-Systemic lupus erythematosus (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening.
+'''Systemic lupus erythematosus''' (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening.
 SLE affects 20 – 100/100 000 in Europe. It more frequently affects women in the ages 16 – 55, being 12x more common in women. Affected men have a worse prognosis. SLE more frequently affects Black and Hispanic ethnicities than White. The 10-year survival rate is 90%.
@@ Line 9: / Line 9: @@
 * HLA-DR2 or -DR3
-* High oestrogenic state (oral contraceptives, endometriosis)
+* High oestrogenic state (oral [[Contraceptive|contraceptives]], [[endometriosis]])
-* Smoking
+* [[Smoking]]
 * Certain drugs
-Drug-induced lupus erythematosus is an SLE-like syndrome caused by certain drugs. The most commonly identified drugs are procainamide, hydralazine, and <abbr>TNF</abbr>-α inhibitors. The first two have the highest risk but aren’t widely used anymore.
+[[Drug-induced lupus erythematosus]] is an SLE-like syndrome caused by certain drugs. The most commonly identified drugs are procainamide, hydralazine, and <abbr>TNF</abbr>-α inhibitors. The first two have the highest risk but aren’t widely used anymore.
 == Pathomechanism ==
@@ Line 40: / Line 40: @@
 The most classical skin symptom in SLE is the malar rash (butterfly rash), which is an erythematous rash on both malar eminences which spares the nasolabial folds. Other skin symptoms include Raynaud phenomenon, photosensitivity, discoid rash, oral ulcers, and alopecia.
-The most common musculoskeletal symptom is non-erosive symmetrical polyarthritis affecting distal joints.
+The most common musculoskeletal symptom is non-erosive symmetrical [[polyarthritis]] affecting distal joints.
-The renal manifestation of lupus is called lupus nephritis, which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome.
+The renal manifestation of lupus is called [[lupus nephritis]], which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome.
-CNS manifestations may be anything from headaches to psychosis and seizures. Cardiovascular manifestations may be myocarditis, valvular disease, and accelerated atherosclerosis. Pulmonary manifestations include interstitial lung disease, pneumonitis, and pulmonary hypertension. Any serous membrane may be inflamed.
+CNS manifestations may be anything from headaches to [[psychosis]] and [[Seizure|seizures]]. Cardiovascular manifestations may be [[myocarditis]], valvular disease, and accelerated [[atherosclerosis]]. Pulmonary manifestations include [[interstitial lung disease]], [[pneumonitis]], and [[pulmonary hypertension]]. Any serous membrane may be inflamed.
-Any haematological cell line may be affected, causing anaemia, leukopaenia, lymphocytopaenia, or thrombocytopaenia. <abbr>GI</abbr> manifestations include abdominal pain, peritonitis, ascites, pancreatitis, and hepatitis.
+Any haematological cell line may be affected, causing [[anaemia]], [[leukopaenia]], [[lymphocytopaenia]], or [[thrombocytopaenia]]. <abbr>GI</abbr> manifestations include abdominal pain, [[peritonitis]], [[ascites]], [[Acute pancreatitis|pancreatitis]], and [[hepatitis]].
 == Diagnosis and evaluation ==
-SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an ANA titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive).
+SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an [[ANA]] titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive).
 Typical laboratory findings include:
@@ Line 55: / Line 55: @@
 * Leukocytopaenia
 * Thrombocytopaenia
-* Autoimmune haemolysis
+* Autoimmune [[haemolysis]]
-* Elevated ESR (normal <abbr>CRP</abbr>)
+* Elevated [[ESR]] (normal <abbr>[[C-reactive protein|CRP]]</abbr>)
 * Decreased level of complement
-* ANA titre > 1:80
+* [[ANA]] titre > 1:80
-* Presence of antiphospholipid antibodies
+* Presence of [[Antiphospholipid antibody|antiphospholipid antibodies]]
 ** Anti-cardiolipin
 ** Lupus anticoagulant
 * Presence of SLE-specific antibodies
-** Anti-dsDNA
+** [[Anti-dsDNA]]
-** Anti-Sm
+** [[Anti-Sm]]
 == Treatment ==
 For the best possible treatment of SLE, it’s important to determine the severity of the disease. There exist countless indexes for this purpose, but the most frequently used is the SLEDAI-2K index. The presence of renal or CNS manifestations necessitates more intensive treatment, and so detection of these complications is important.
-Important lifestyle changes include appropriate sun protection, smoke cessation, exercise, and adherence to vaccination protocols. NSAIDs can provide symptomatic relief.
+Important lifestyle changes include appropriate sun protection, smoke cessation, exercise, and adherence to vaccination protocols. [[NSAID|NSAIDs]] can provide symptomatic relief.
-Pharmacological treatment can be considered in three different forms: induction of remission, maintenance of remission, and supportive treatment. Side effects of pharmacological therapy is unfortunately a major cause of SLE-related morbidity and mortality, mostly due to glucocorticoids.
+Pharmacological treatment can be considered in three different forms: induction of remission, maintenance of remission, and supportive treatment. Side effects of pharmacological therapy is unfortunately a major cause of SLE-related morbidity and mortality, mostly due to [[corticosteroids]].
-For induction of remission, glucocorticoids are used. In cases of renal or CNS affection, other immunosuppressants like cyclophosphamide or mycophenolate mofetil may also be necessary.
+For induction of remission, corticosteroids are used. In cases of renal or CNS affection, other immunosuppressants like [[cyclophosphamide]] or [[mycophenolate mofetil]] may also be necessary.
-For maintenance of remission, hydroxychloroquine is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like azathioprine, methotrexate, and sometimes even long-term low-dose glucocorticoids, may be required. Belimumab and rituximab are the only biological therapies indicated for SLE.
+For maintenance of remission, [[hydroxychloroquine]] is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like [[azathioprine]], [[methotrexate]], and sometimes even long-term low-dose glucocorticoids, may be required. [[Belimumab]] and [[rituximab]] are the only biological therapies indicated for SLE.
-Supportive treatment includes medications like ACE inhibitors, ARBs, statins, antiepileptics, bisphosphonates, etc.
+Supportive treatment includes medications like [[ACE inhibitor|ACE inhibitors]], [[ARB|ARBs]], [[Statin|statins]], [[Antiepileptic drug|antiepileptics]], [[Bisphosphonate|bisphosphonates]], etc.
 [[Category:Rheumatology]]