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Systemic lupus erythematosus (SLE) one of several diseases known as [[the great imitator]]<ref>[http://healthcare.utah.edu/healthtools/articles/lupus.html Lupus: The Great Imitator]</ref> because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.
Systemic lupus erythematosus (SLE) one of several diseases known as [[the great imitator]]<ref>[http://healthcare.utah.edu/healthtools/articles/lupus.html Lupus: The Great Imitator]</ref> because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.


The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] are suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
A positive history of familial lupus, skin rashes (especially photo-sensitive skin rashes), arthritis, and fatigue may be suggestive of systemic lupus erythematosus. The most common symptoms of SLE include .


==History==
==History==
Obtaining the history is the most important aspect of making a diagnosis of systemic lupus erythematosus. It provides insight into disease course and severity, precipitating risk factors and associated comorbid conditions. Complete history will help determine the correct complication diagnosis, and helps in determining the prognosis as well. Specific histories about the symptoms (duration, onset, progression), associated symptoms, and drug usage have to be obtained. Specific areas of focus when obtaining the history, are outlined below:
* Onset, duration and progression of symptoms
* History of weight changes (gain or loss)
* Anorexia
* Arthritis, arthralgias, or muscle pain
* Morning stiffness
* Skin rashes and their association to flare ups, especially after exposure to sunlight
* Medications and their association to flare ups
* Infections especially mononucleosis
* Sores in the mouth, nose, or other mucous membranes
* Symptoms of other organ failure
** [[Renal failure]]: Recent extremity edema and weight gain
** Cardiac involvement: Tachycardia, dyspnea, chest pains
* History of having a pet
* Hair loss or thinning
* Job history
* Co-morbid conditions include:
** Other rheumatologic diseases
** Hypertension
** [[diabetes]], [[immunodeficiency]]
* Seizures, convulsions, or other nervous system symptoms
* Family history of rheumatologic diseases


== Common Symptoms ==
== Common Symptoms ==
Line 25: Line 47:


===== Organ system based signs and symptoms<ref name="pmid14530779">{{cite journal |vauthors=Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR |title=Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients |journal=Medicine (Baltimore) |volume=82 |issue=5 |pages=299–308 |year=2003 |pmid=14530779 |doi=10.1097/01.md.0000091181.93122.55 |url=}}</ref><ref name="pmid2319520">{{cite journal |vauthors=Finol HJ, Montagnani S, Márquez A, Montes de Oca I, Müller B |title=Ultrastructural pathology of skeletal muscle in systemic lupus erythematosus |journal=J. Rheumatol. |volume=17 |issue=2 |pages=210–9 |year=1990 |pmid=2319520 |doi= |url=}}</ref> =====
===== Organ system based signs and symptoms<ref name="pmid14530779">{{cite journal |vauthors=Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR |title=Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients |journal=Medicine (Baltimore) |volume=82 |issue=5 |pages=299–308 |year=2003 |pmid=14530779 |doi=10.1097/01.md.0000091181.93122.55 |url=}}</ref><ref name="pmid2319520">{{cite journal |vauthors=Finol HJ, Montagnani S, Márquez A, Montes de Oca I, Müller B |title=Ultrastructural pathology of skeletal muscle in systemic lupus erythematosus |journal=J. Rheumatol. |volume=17 |issue=2 |pages=210–9 |year=1990 |pmid=2319520 |doi= |url=}}</ref> =====
* Symptoms are listed based on their frequency in each organ system
{| class="wikitable"
{| class="wikitable"
!Organ system
!Organ system
!Disease
!Disease
!Sympton
!Sympton
!
!
|-
|-
| rowspan="7" |Gastrointestinal involvement
| rowspan="7" |Gastrointestinal involvement
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* Regurgitation
* Regurgitation
* Odynophagia
* Odynophagia
|
|
|-
|-
|Peptic ulcer disease
|Peptic ulcer disease
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* Early satiety
* Early satiety
* Nausea
* Nausea
|
|
|-
|-
|Intestinal pseudo-obstruction
|Intestinal pseudo-obstruction
Line 56: Line 73:
* Bloating
* Bloating
* Distension
* Distension
|
|
|-
|-
|Protein-losing enteropathy
|Protein-losing enteropathy
Line 64: Line 79:
* Hypoalbuminemia
* Hypoalbuminemia
* Severe diarrhea
* Severe diarrhea
|
|
|-
|-
|Acute pancreatitis
|Acute pancreatitis
|
|
* Severe persistent epigastric pain often radiating to the back
* Severe persistent epigastric pain often radiating to the back
|
|
|-
|-
|Mesenteric vasculitis
|Mesenteric vasculitis
Line 82: Line 93:
* Diarrhea
* Diarrhea
** Due to chronic mesenteric ischemia
** Due to chronic mesenteric ischemia
|
|
|-
|-
|Primary peritonitis
|Primary peritonitis
Line 92: Line 101:
* Constipation or the inability to pass gas
* Constipation or the inability to pass gas
* Anorexia
* Anorexia
|
|
|-
|-
| rowspan="7" |Pulmonary involvement
| rowspan="7" |Pulmonary involvement
Line 101: Line 108:
* Dyspnea
* Dyspnea
* Fever
* Fever
|
|
|-
|-
|Acute pneumonitis
|Acute pneumonitis
Line 109: Line 113:
* Fever
* Fever
* Cough (sometimes with hemoptysis)
* Cough (sometimes with hemoptysis)
* Dyspnea/ physical examination reveals tachypnea, tachycardia, basilar crackles (may be late inspiratory), and hypoxemia
* Dyspnea
|
|
|-
|-
|Pulmonary hemorrhage
|Pulmonary hemorrhage
Line 118: Line 120:
* Cough
* Cough
* Hemoptysis
* Hemoptysis
|
|
|-
|-
|Interstitial lung disease
|Interstitial lung disease
Line 127: Line 127:
* Dyspnea
* Dyspnea
* Decreased exercise tolerance
* Decreased exercise tolerance
|
|
|-
|-
|Thromboembolic disease 
|Thromboembolic disease 
Line 134: Line 132:
* Dyspnea
* Dyspnea
* Sharp chest pain that may become worse with deep breathing or coughing
* Sharp chest pain that may become worse with deep breathing or coughing
|
|
|-
|-
|Pulmonary hypertension
|Pulmonary hypertension
Line 147: Line 143:
* Edema
* Edema
* Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
* Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
|
|
|-
|-
|Shrinking lung
|Shrinking lung
Line 154: Line 148:
* Dyspnea
* Dyspnea
* Pleuritic chest pain (episodic)
* Pleuritic chest pain (episodic)
|
|
|-
|-
| rowspan="5" |Cardiac involvement
| rowspan="5" |Cardiac involvement
|Valvular disease
|Valvular disease
|
|
* most often valve thickening, nodules, and regurgitation, and less frequently vegetations or stenotic lesions, is noted echocardiographically, and the presence of valvular lesions may increase the risk of serious complications
* Tachycardia
|
* Syncope
|
* Dyspnea
* Peripheral edema
|-
|-
|Nonbacterial thrombotic endocarditis (Libman-Sacks, verrucous endocarditis)
|Nonbacterial thrombotic endocarditis
|
* May occur in patients with SLE and is associated with antiphospholipid antibodies (aPL).
|
|
|
* Fever and chills
* Arthralgias
* Persistent cough
|-
|-
|Acute pericarditis
|Acute pericarditis
Line 174: Line 167:
* Sharp, stabbing chest pain with a sudden start
* Sharp, stabbing chest pain with a sudden start
* Positional pain that decreases when sitting up and leaning forward
* Positional pain that decreases when sitting up and leaning forward
|
|
|-
|-
|Myocarditis 
|Myocarditis 
|
|
* Tachycardia
* Tachycardia
|
|
|-
|-
|Coronary heart disease
|Coronary heart disease
Line 188: Line 177:
* Dyspnea
* Dyspnea
* Nausea and vomiting
* Nausea and vomiting
|
|
|-
|-
| rowspan="6" |Neurological involvement
| rowspan="4" |Neurological involvement
|Cognitive dysfunction
|CNS small vessle vasculitis
|
|
|
|
* Cognitive dysfunction
* Headache
* Neuropathies
|-
|-
|Stroke
|Stroke
Line 201: Line 189:
* Speech disturbance
* Speech disturbance
* Weakness on one-half of the body
* Weakness on one-half of the body
|
|
|-
|-
|Seizures
|Seizures
|
|
* Tonic-clonic seizure
* Tonic-clonic seizure
|
|
|-
|-
|Psychosis
|Psychosis
|
|
* Hallucinations visual
* Hallucinations
* Auditory
** Visual
|
** Auditory
|
|-
|Headache
|
|
|
|-
|Neuropathies
|
|
|
|-
|-
|Genitourinary
|Genitourinary
Line 234: Line 207:
* Foamy urine
* Foamy urine
* Weight gain
* Weight gain
|
|
|-
|-
| rowspan="3" |Musculoskeletal
| rowspan="3" |Musculoskeletal
Line 243: Line 214:
* Arthralgias
* Arthralgias
* Decreased range of motion of both small and large joints
* Decreased range of motion of both small and large joints
* Morning stiffness
* Morning stiffness that will last less than 1 hour
|
|
|-
|-
|Avascular necrosis
|Avascular necrosis
|
|
* Asymptomatic
* Asymptomatic
* Mild to moderate pain in groin and lower abdomen
* Mild to moderate pain in groin and lower abdomen in case of femoral head AVN
* Secondary pain amplification
* Secondary pain amplification
|
|
|-
|-
|Osteoporosis 
|Osteoporosis 
Line 259: Line 226:
* Loss of height
* Loss of height
* Sudden back pain
* Sudden back pain
|
|
|-
|-
| rowspan="4" |Skin disorder
| rowspan="4" |Skin disorder
Line 268: Line 233:
* Generalized maculopapular rash
* Generalized maculopapular rash
* Discoid rash
* Discoid rash
* Vasculitic lesions
* Bullous lesions
* Bullous lesions
* Raynaud's phenomenon
* Raynaud's phenomenon
* Pyoderma gangrenosum
* Pyoderma gangrenosum
** Ulcerative cutaneous condition
* Erythema multiforme  
* Erythema multiforme  
* Nail fold infarcts
* Nail fold infarcts
|
|
|-
|-
|Photosensitivity
|Photosensitivity
|common theme for skin lesions associated with SLE
|Common theme for skin lesions associated with SLE
|
|
|-
|-
|oral and/or nasal ulcers
|oral and/or nasal ulcers
|usually painless
|Usually painless
|
|
|-
|-
|Nonscarring alopecia
|Nonscarring alopecia
|may occur at some point during the course of their disease
|May occur at some point during the course of their disease
|
|
|}
|}


Revision as of 23:43, 5 July 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Systemic lupus erythematosus (SLE) one of several diseases known as the great imitator[1] because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.

A positive history of familial lupus, skin rashes (especially photo-sensitive skin rashes), arthritis, and fatigue may be suggestive of systemic lupus erythematosus. The most common symptoms of SLE include .

History

Obtaining the history is the most important aspect of making a diagnosis of systemic lupus erythematosus. It provides insight into disease course and severity, precipitating risk factors and associated comorbid conditions. Complete history will help determine the correct complication diagnosis, and helps in determining the prognosis as well. Specific histories about the symptoms (duration, onset, progression), associated symptoms, and drug usage have to be obtained. Specific areas of focus when obtaining the history, are outlined below:

  • Onset, duration and progression of symptoms
  • History of weight changes (gain or loss)
  • Anorexia
  • Arthritis, arthralgias, or muscle pain
  • Morning stiffness
  • Skin rashes and their association to flare ups, especially after exposure to sunlight
  • Medications and their association to flare ups
  • Infections especially mononucleosis
  • Sores in the mouth, nose, or other mucous membranes
  • Symptoms of other organ failure
    • Renal failure: Recent extremity edema and weight gain
    • Cardiac involvement: Tachycardia, dyspnea, chest pains
  • History of having a pet
  • Hair loss or thinning
  • Job history
  • Co-morbid conditions include:
  • Seizures, convulsions, or other nervous system symptoms
  • Family history of rheumatologic diseases

Common Symptoms

The early manifestations of systemic lupus erythematosus include common constitutional symptoms that may be seen in may other diseases as well.

  • Constitutional symptoms[2][3][4]
    • Fatigue
    • Fever
    • Myalgia
    • Muscle tenderness
    • Muscle weakness
    • Weight change
      • Weight gain due to increase in water retention and increase in appetite
      • Weight loss due to medication side effect and gastro-intestinal problems
    • Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.
Organ system based signs and symptoms[5][6]
  • Symptoms are listed based on their frequency in each organ system
Organ system Disease Sympton
Gastrointestinal involvement Dysphagia 
  • Retrosternal chest pain
  • Heartburn
  • Regurgitation
  • Odynophagia
Peptic ulcer disease
  • Epigastric pain
  • Food-provoked epigastric discomfort and fullness
  • Early satiety
  • Nausea
Intestinal pseudo-obstruction
  • Abdominal pain
  • Bloating
  • Distension
Protein-losing enteropathy
  • Profound edema
  • Hypoalbuminemia
  • Severe diarrhea
Acute pancreatitis
  • Severe persistent epigastric pain often radiating to the back
Mesenteric vasculitis
  • Abdominal pain
  • Food aversion
  • Weight loss
  • Nausea
  • Vomiting
  • Diarrhea
    • Due to chronic mesenteric ischemia
Primary peritonitis
  • Abdominal bloating or distention
  • Nausea and vomiting
  • Diarrhea
  • Constipation or the inability to pass gas
  • Anorexia
Pulmonary involvement Pleural disease
  • Cough
  • Dyspnea
  • Fever
Acute pneumonitis
  • Fever
  • Cough (sometimes with hemoptysis)
  • Dyspnea
Pulmonary hemorrhage
  • Dyspnea
  • Cough
  • Hemoptysis
Interstitial lung disease
  • May be asymptomatic
  • Chronic nonproductive cough
  • Dyspnea
  • Decreased exercise tolerance
Thromboembolic disease 
  • Dyspnea
  • Sharp chest pain that may become worse with deep breathing or coughing
Pulmonary hypertension
  • Dyspnea
  • Palpitations
  • Fatigue
  • Impaired exercise tolerance
  • Weakness
  • Syncope
  • Edema
  • Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
Shrinking lung
  • Dyspnea
  • Pleuritic chest pain (episodic)
Cardiac involvement Valvular disease
  • Tachycardia
  • Syncope
  • Dyspnea
  • Peripheral edema
Nonbacterial thrombotic endocarditis
  • Fever and chills
  • Arthralgias
  • Persistent cough
Acute pericarditis
  • Sharp, stabbing chest pain with a sudden start
  • Positional pain that decreases when sitting up and leaning forward
Myocarditis 
  • Tachycardia
Coronary heart disease
  • Chest pain
  • Dyspnea
  • Nausea and vomiting
Neurological involvement CNS small vessle vasculitis
  • Cognitive dysfunction
  • Headache
  • Neuropathies
Stroke
  • Speech disturbance
  • Weakness on one-half of the body
Seizures
  • Tonic-clonic seizure
Psychosis
  • Hallucinations
    • Visual
    • Auditory
Genitourinary Nephrotic syndrome
  • Hypertension
  • Peripheral edema
  • Foamy urine
  • Weight gain
Musculoskeletal Arthritis
  • Mostly symmetrical
  • Arthralgias
  • Decreased range of motion of both small and large joints
  • Morning stiffness that will last less than 1 hour
Avascular necrosis
  • Asymptomatic
  • Mild to moderate pain in groin and lower abdomen in case of femoral head AVN
  • Secondary pain amplification
Osteoporosis 
  • Loss of height
  • Sudden back pain
Skin disorder Cutaneous lupus erythematosus
  • Malar rash
  • Generalized maculopapular rash
  • Discoid rash
  • Bullous lesions
  • Raynaud's phenomenon
  • Pyoderma gangrenosum
    • Ulcerative cutaneous condition
  • Erythema multiforme
  • Nail fold infarcts
Photosensitivity Common theme for skin lesions associated with SLE
oral and/or nasal ulcers Usually painless
Nonscarring alopecia May occur at some point during the course of their disease

References

  1. ↑ Lupus: The Great Imitator
  2. ↑ Tench CM, McCurdie I, White PD, D'Cruz DP (2000). "The prevalence and associations of fatigue in systemic lupus erythematosus". Rheumatology (Oxford). 39 (11): 1249–54. PMID 11085805.
  3. ↑ McKinley PS, Ouellette SC, Winkel GH (1995). "The contributions of disease activity, sleep patterns, and depression to fatigue in systemic lupus erythematosus. A proposed model". Arthritis Rheum. 38 (6): 826–34. PMID 7779127.
  4. ↑ Wang B, Gladman DD, Urowitz MB (1998). "Fatigue in lupus is not correlated with disease activity". J. Rheumatol. 25 (5): 892–5. PMID 9598886.
  5. ↑ Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR (2003). "Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients". Medicine (Baltimore). 82 (5): 299–308. doi:10.1097/01.md.0000091181.93122.55. PMID 14530779.
  6. ↑ Finol HJ, Montagnani S, Márquez A, Montes de Oca I, Müller B (1990). "Ultrastructural pathology of skeletal muscle in systemic lupus erythematosus". J. Rheumatol. 17 (2): 210–9. PMID 2319520.

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