II. Background

  1. Named in 1851 for wolf (lupus) bite-like facial rash
  2. First described by Moritz Kaposi

III. Epidemiology

  1. Prevalence
    1. U.S.: 40 to 50 per 100,000 persons (up to 1 in 1000)
    2. England: 200 per 100,000 women aged 18 to 65 years
  2. Over-diagnosed in United States
    1. Of 2 million U.S. cases, only 25% have true disease
  3. Age distribution
    1. Young adults with onset 15-64 years
    2. Children comprise 10-15% of cases
  4. More common in women (especially child-bearing age) by ratio of 9:1 (women 90%, men 10%)
  5. Ethnic predisposition
    1. Native American
    2. African American
    3. Hispanic
    4. Chinese
    5. Filipino

IV. Etiology

  1. Idiopathic
  2. Drug Induced lupus examples (more than 80 drugs associated, first described in 1945 related to sulfadiazine)
    1. Hydralazine (most common cause)
    2. Procainamide
    3. Isoniazid
    4. Minocycline
    5. Terbinafine
    6. Phenytoin
    7. Ethosuximide
    8. D-Penicillamine
    9. Proton Pump Inhibitors

V. Pathophysiology

  1. Tissue damage by Antibody and immune complex deposition
  2. Autoantibodies form to cell nucleus components

VI. Findings: Presentations

  1. Women of child-bearing age (age 15 to 50 years old)
    1. Fever
    2. Malar Rash
    3. Arthralgias (or myalgias)
  2. Men
    1. Less skin and joint involvement in men than women and children
    2. Hematologic disorder
    3. Renal disorder
    4. Neurologic disorder
  3. Children
    1. Fever
    2. Malar Rash
    3. Arthralgias
    4. Alopecia
    5. Hematologic disorder (Anemia, Leukocytopenia)
    6. Renal involvement
  4. Drug-Induced Lupus (occurs equally in men and women, typically closer to age 50 years old)
    1. Fever
    2. Rash
    3. Arthralgias and myalgias
    4. Pleuritic Chest Pain
    5. Rare renal involvement and rare CNS involvement
    6. Resolves in most cases after offending drug is stopped (see list of drug causes above)

VII. Symptoms

  1. Fatigue
  2. Fever
  3. Malaise
  4. Weight loss

VIII. Signs: General

  1. Dermatologic
    1. See Cutaneous Lupus Erythematosus
    2. Malar "butterfly" rash (50% of cases)
    3. Photosensitivity
    4. Vasculitis
    5. Alopecia
    6. Oral Ulcers
    7. Sicca Syndrome
  2. Rheumatologic
    1. Arthritis and Polyarthritis
    2. Myositis
  3. Abdominal
    1. Lymphadenopathy
    2. Splenomegaly
    3. Nephritis
    4. Mesenteric Vasculitis
  4. Neuropsychiatric
    1. Organic brain syndrome
    2. Seizures
    3. Psychosis
    4. Thrombotic Cerebrovascular Accident
    5. Transverse myelitis
    6. Cognitive dysfunction (spectrum from mild cognitive deficits to Dementia)
    7. Migraine Headaches
    8. Peripheral Neuropathy
  5. Cardiovascular
    1. Pericarditis
    2. Myocarditis
    3. Acute Coronary Syndrome
    4. Venous thrombosis (DVT)
      1. Suggests Antiphospholipid Antibody Syndrome
  6. Pulmonary
    1. Pleuritis
    2. Pneumonitis or Pneumonia
    3. Interstitial Lung Disease
    4. Pulmonary Arterial Hypertension
    5. Pulmonary Embolism
  7. Ocular changes (20% of SLE cases)
    1. Anterior eye disorders
      1. Keratoconjunctivitis Sicca
      2. Uveitis
      3. Episcleritis and Scleritis
      4. Keratitis
      5. Blepharitis-like Discoid Lupus eyebrow involvement
    2. Retinal disorders
      1. Cotton wool spots
      2. Retinal Hemorrhages
      3. Proliferative retinopathy
    3. Neurologic conditions
      1. Optic Neuritis
      2. Ischemic Optic Neuropathy
      3. Amaurosis Fugax

IX. Diagnosis: ACR requires 4 of 11 criteria

  1. Malar Rash (present in 50% of cases)
    1. Erythema (may be raised) over the cheeks and sparing the nasolabial folds
  2. Discoid rash
    1. Raised erythematous patches with adherent keratotic Scaling, follicular plugging and atrophic scarring in older patients
  3. Photosensitivity
    1. Atypical sunlight skin reactions
  4. Oral Ulcers
    1. Typically painless, oral or nasopharyngeal lesions
  5. Polyarthritis (present in 90% of cases)
    1. Non-erosive arthritis involving more than 2 joints
  6. Lupus Serositis (Pleuritis or Pericarditis)
    1. See cardiac complications and pulmonary complications described below
    2. Pleuritic Chest Pain, friction rub or Pleural Effusion
    3. Positional Chest Pain worse supine, improved sitting or leaning forward with a Pericardial Effusion or friction rub
    4. EKG changes with diffuse ST Elevation, PR depression and no reciprocal changes
  7. Renal disease or Lupus Nephritis (ultimately present in 60% of cases)
    1. Persistent Proteinuria > 500 mg/day (>3+ Urine Protein) or RBC cellular casts (or mixed casts)
  8. Neurologic disorder or Lupus Cerebritis (present in 75% of cases)
    1. Seizure disorder
    2. Psychosis
    3. Thrombotic Cerebrovascular Accident
    4. Transverse myelitis
    5. Cognitive dysfunction (spectrum from mild cognitive deficits to Dementia)
    6. More subtle, less specific or dramatic changes (Migraine Headache, Peripheral Neuropathy)
  9. Hematologic disorder
    1. Hemolytic Anemia
    2. Leukopenia or lymphopenia
    3. Thrombocytopenia <100,000/mm3
  10. Antinuclear Antibody titer positive (1:80 or higher)
    1. Titer over 1:320 is very suggestive
    2. Prior discriminatory value was 1:40 dilution
  11. Other immunologic disorders
    1. Anti-dsDNA positive
    2. Anti-Sm positive
    3. Antiphospholipid Antibody positive
    4. Previously also included criteria of Syphilis False Positive

X. Labs: Protocol

  1. Lab interpretation described specifically below
  2. Indications for ANA titer
    1. Unexplained involvement of Two or more organ systems
    2. ANA is very prevalent in normal population
  3. Initial Screening
    1. ANA titer positive if 1:80 dilution (prior cut-off was 1:40)
  4. Secondary testing if ANA titer positive
    1. Complete Blood Count
    2. Urinalysis
    3. Serum Creatinine
    4. Antiphospholipid Antibody
    5. Double Stranded DNA Antibody (Anti-dsDNA)
    6. Smith Antibody (Anti-Smith or Anti-Sm)

XI. Labs: Interpretation

  1. Complete Blood Count
    1. Anemia
    2. Neutropenia
    3. Thrombocytopenia
  2. Urinalysis
    1. Lupus Nephritis suggested by persistent Proteinuria > 500 mg/day (>3+ Urine Protein) or RBC cellular casts (or mixed casts)
    2. Consider 24 Hour Urine Protein
    3. Consider 24 hour Creatinine Clearance
  3. Coagulation Factors
    1. Prothrombin Time
    2. Partial Thromboplastin Time (PTT)
      1. Increased (prolonged) in Antiphospholipid Antibody Syndrome
  4. Primary Antinuclear Antibodies (and other autoantibodies)
    1. Antinuclear Antigen (ANA)
      1. Positive in 98% of true SLE cases
      2. Typically positive in Drug-Induced Lupus
      3. Only 5% of ANA positive patients have SLE
    2. Smith Antibody (Anti-Smith or Anti-Sm)
      1. Positive in 20-30% of SLE cases
      2. Highly specific for SLE (nearly pathognomonic)
      3. Does not correlate with disease activity (unlike Anti-dsDNA)
    3. Double Stranded DNA Antibody (Anti-dsDNA)
      1. Positive in 50-70% of SLE cases
      2. Specific for Systemic Lupus Erythematosus
      3. Associated with Lupus Nephritis
      4. Associated with Lupus CNS Involvement
      5. Also positive in Syphilis and Bacterial Endocarditis
  5. Antiphospholipid Antibody Syndrome related labs
    1. Antiphospholipid Antibody
    2. Anticardiolipin Antibody
    3. Partial Thromboplastin Time (PTT)
      1. Prolonged in Antiphospholipid Antibody Syndrome
  6. Other Autoantibodies
    1. Anti-ribosomal P (Lupus sensitivity: 20-30%)
      1. Highly specific for lupus erythematosus
      2. Associated with Lupus Psychosis
    2. Anti-Ro (Anti-SSA)
      1. Positive in 40% of SLE cases
    3. Anti-La (Anti-SSB)
      1. Positive in 10-15% of SLE cases
    4. Histone Antibody (Anti-histone)
      1. Positive in 50-70% of SLE cases
  7. Other labs used historically in Lupus evaluation
    1. Complement Levels
      1. Complement C3, Complement C4, Complement CH50
      2. Typically unreliable in predicting acute lupus flare
    2. Syphilis Serology (VDRL or RPR)
    3. Erythrocyte Sedimentation Rate elevated

XII. Diagnostics

  1. Electrocardiogram
    1. Pericarditis
      1. See EKG in Pericarditis
  2. Lumbar Puncture
    1. Evaluate differential diagnosis of Lupus Cerebritis (e.g. Meningitis or encephilitis, Multiple Sclerosis)
    2. Findings suggestive of increased CNS Lupus activity
      1. CSF White Blood Cells increased
      2. CSF Protein increased
      3. Immunoglobulin synthesis or Immunoglobulin G increased

XIII. Imaging

  1. Brain MRI
    1. Indicated in suspected Lupus Cerebritis or other neuropsychiatric findings
    2. Findings are typically non-specific
  2. Echocardiogram (or Bedside Ultrasound)
    1. Indicated for suspected Myocarditis or Pericarditis
    2. Evaluate for overall Left Ventricular Dysfunction and Pericardial Effusion

XIV. Complications

  1. Immunocompromised state
    1. Hyposplenism
    2. Immunosuppressants used to treat systemic lupus
  2. Lupus Nephritis
    1. Lupus Nephritis is a Glomerulonephritis of several different types (worst is diffuse proliferative nephritis)
    2. Lupus Nephritis is a predictor of increased mortality risk
  3. Lupus Cerebritis
    1. Various presentations (see above under diagnosis) of CNS disease are seen in 75% of Lupus patients
    2. Consider MRI and Lumbar Puncture (see above under imaging and diagnostics)
  4. Cardiovascular disease
    1. Coronary events
      1. Coronary atherosclerosis (secondary to autoantibody related Vasculitis and lupus medication adverse effects)
      2. Coronary Vasculitis with secondary ST segment elevation Myocardial Infarction (STEMI)
      3. Spontaneous Coronary Artery dissection
    2. Pericarditis (most common)
      1. Risk of Cardiac Tamponade (has even occurred in children)
    3. Myocarditis
      1. Presents with conduction abnormalities, arrhythmias
      2. Associated with Cardiomyopathy or Heart Failure
  5. Pulmonary disease
    1. Lung disease affects 50% of Lupus patients and is a presenting symptom in up to 5% of cases
    2. Pleuritis and pulmonary infections are most common presentations
    3. Interstitial Lung Disease, pneumonitis, Pulmonary Arterial Hypertension and Pulmonary Embolism may also occur
  6. Venous Thromboembolism (VTE)
    1. Higher risk by a factor of 100 in SLE (and at a younger age)
      1. Occurs in up to 26% of lupus patients (contrast with 0.2% in the general population)
    2. Increased risk with Antiphospholipid Antibody positive status
    3. D-Dimer has less less utility in SLE due to a high False Positive Rate in this population (less specific)
      1. However, a negative D-Dimer in SLE suggests a very low likelihood of VTE
      2. Wu (2008) Clin J Am Soc Nephrol 3(6): 1628-36
  7. Antiphospholipid Antibody Syndrome (Hughes Syndrome, Anticardiolipin Syndrome)
    1. Affects 15% of Lupus patients
    2. Risk of thromboembolic complications (despite prolonged PTT)
      1. Venous Thromboembolism
      2. Acute Coronary Syndrome
      3. Acute thrombotic stroke
      4. Acute Limb Ischemia
      5. Mesenteric Ischemia
      6. Renal artery thrombosis
      7. Raynaud's Phenomenon (with risk of digital ischemia)

XV. Management: General Principles

  1. Reevaluate every 3-6 months
  2. Employ measures to relieve Fatigue
  3. Sunscreen and other protection due to photosensitivity
  4. Reduce risk of infection (e.g. Immunizations)
  5. Birth Control is critical during exacerbations

XVI. Management: Hospitalization indications

  1. Acute lupus presentation
    1. Female aged 15 to 50 years old with fever, arthralgias and Malar Rash and associated findings (esp. nephritis)
    2. May benefit from admission for diagnosis and acute management
  2. Significant febrile illness
    1. Lower threshold for hospital admission given underlying immunocompromised state and potential Hyposplenism
  3. Acute lupus flare with severe pain
    1. Consider IV cytotoxic agents and Corticosteroids
  4. Lupus Nephritis (acute onset or worsening)
    1. Heralded by abnormal Urinalysis (Hematuria, 3+ Proteinuria, RBC cellular casts) or increased Serum Creatinine
    2. Renal biopsy is typically required for diagnosis of specific Glomerulonephritis type, which in turn drives management
    3. Nephritis is a predictor of increased mortality and morbidity, including progression to Renal Failure
    4. Acute management typically makes use of Systemic Corticosteroids and immunosuppressants
  5. Lupus Cerebritis (e.g. Seizures, Psychosis, Dementia)
    1. Initial evaluation typically with MRI Brain and Lumbar Puncture
    2. Hospitalization for further evaluation and acute management
  6. Acute cardiovascular conditions
    1. Acute Coronary Syndrome
    2. Pericarditis
    3. Myocarditis (may cause life threatening, Acute Heart Failure)
  7. Venous Thromboembolism
    1. Suggests Antiphospholipid Antibody Syndrome (higher risk of thrombotic complications)
  8. Acute Abdominal Pain
    1. Hospitalization indicated where diagnosis is unclear or for associated Lactic Acidosis
    2. Normal Complete Blood Count or non-diagnostic CT Abdomen does not exclude serious cause in SLE
    3. Acute Abdominal Pain in SLE presenting to the emergency department is associated with 57% morbidity and 11% mortality
      1. Vergara-Fernandez (2009) J Gastrointest Surg 13(7): 1351-7

XVII. Management: Medications

  1. Corticosteroids
    1. Indicated as a mainstay of acute management
    2. Systemic Corticosteroids in moderate to severe exacerbations
      1. Prednisone 0.5 to 1 mg/kg/day up to 4 weeks or
      2. Solu-medrol 15 mg/kg IV for 3 days
    3. Skin lesions (no significant evidence to support use)
      1. Topical Corticosteroids
      2. Intralesional Corticosteroids
  2. Salicylates and NSAIDs
    1. Indicated for mild to moderate pain from arthralgias, Headache or other lupus-related conditions
    2. Contraindicated in renal disease
    3. Preparations
      1. Enteric Coated ASA 650 mg PO every 4-6 hours prn
      2. Ibuprofen 400-800 mg PO tid-qid prn
  3. Cytotoxic agents (and other immunosuppressants)
    1. Hydroxychloroquine (Plaquenil)
      1. Indicated for Lupus Nephritis and lupus-related skin lesions
      2. Dosing: 400 mg/day
    2. Cyclophosphamide
      1. Indicated in Lupus Nephritis
      2. Daily dosing: 1.5-2.5 mg/kg/day or
      3. Monthly dosing: 10-15 mg/kg IV every 4 weeks
    3. Azathioprine (Imuran)
      1. Dosing 2-3 mg/kg/day
      2. Therapeutic response and toxicity monitored with thiopurine metabolites (6-MMP, 6-TGN)
    4. Mycophenolate mofetil
      1. Therapeutic response and toxicity monitored with mycophenolic acid (MPA)
  4. Anticoagulation
    1. Indicated in Antiphospholipid Antibody Syndrome (to prevent thrombotic complications)
    2. Warfarin (Coumadin) with goal INR of 2.5 to 3.0
  5. Additional measures
    1. Ophthalmology Consultation for dilated Eye Exam
      1. Initial exam on starting steroids or Plaquenil
      2. Repeat exam yearly in high risk patients

XVIII. Prognosis

  1. Drug-Induced Lupus typically resolves spontaneously after stopping the offending agent
  2. Overall five year survival: 91-97%
  3. Worse prognostic factors
    1. Seizure disorder
    2. Lupus Nephritis
    3. Azotemia
    4. Onset in childhood

XIX. Resources

  1. American Lupus Society (Ventura, CA)
    1. Phone: (800) 331-1802
  2. Lupus Foundation of America
    1. http://www.lupus.org

XX. References

  1. Edworthy in Ruddy (2001) Kelly's Rheumatology, 1105-19
  2. Green (2014) Crit Dec Emerg Med 28(10): 2-9
  3. Sercombe in Marx (2002) Rosen's Emergency, p. 1607-13
  4. Gill (2003) Am Fam Physician 68:2179-86

Images: Related links to external sites (from Google)

Ontology: Lupus Erythematosus, Systemic (C0024141)

Definition (MEDLINEPLUS)

If you have lupus, your immune system attacks healthy cells and tissues by mistake. This can damage your joints, skin, blood vessels and organs. There are many kinds of lupus. The most common type, systemic lupus erythematosus, affects many parts of the body. Discoid lupus causes a rash that doesn't go away. Subacute cutaneous lupus causes sores after being out in the sun. Another type can be caused by medication. Neonatal lupus, which is rare, affects newborns.

Anyone can get lupus, but women are most at risk. Lupus is also more common in African American, Hispanic, Asian and Native American women. The cause of lupus is not known.

Lupus has many symptoms. Some common ones are

  • Joint pain or swelling
  • Muscle pain
  • Fever with no known cause
  • Red rashes, often on the face (also called the "butterfly rash")

There is no one test to diagnose lupus, and it may take months or years to make the diagnosis. There is no cure for lupus, but medicines and lifestyle changes can help control it.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Definition (NCI) A chronic, inflammatory, connective tissue disease that can affect many organs including the joints, skin, heart, lungs, kidneys, and nervous system. It is marked by many different symptoms; however, not everyone with SLE has all of the symptoms.
Definition (MSH) A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Definition (CSP) chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes; it is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system; the disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Concepts Disease or Syndrome (T047)
MSH D008180
ICD9 710.0
ICD10 M32 , M32.9
SnomedCT 156450004, 201435004, 201439005, 55464009
English Lupus erythematosus, disseminated, SYSTEMIC LUPUS ERYTHEMATOSIS, LUPUS, ERYTHEMATOSUS, SYSTEMIC, Lupus Erythematosus Disseminatus, Lupus Erythematosus, Systemic, LE SYNDROME, DISSEMINATED LUPUS ERYTHEMATOSUS, LUPUS ERYTHEMATOSUS SYSTEMIC, LUPUS ERYTHEMATOSUS, DISSEMINATED, LUPUS ERYTHEMATOSUS, SYSTEMIC, LUPUS SYNDROME, Lupus erythematosus, Systemic, Lupus erythematosus, systemic, SYNDROME LUPUS, LUPUS ERYTHEMATOSIS DISSEMINATED, Disseminated lupus erythematos, SLE-System lupus erythematosus, Systemic lupus erythematos.NOS, Systemic lupus erythematosus NOS, Systemic lupus erythematosus, unspecified, SYNDROME DISSEMINATED LUPUS ERYTHEMATOSIS, LUPUS ERYTHEMATOSIS (NOS), SYSTEMIC LUPUS ERYTHEMATOSUS, SYSTEMIC LUPUS ERYTHEMATOSUS SYND, Systemic Lupus Erythematosus, disseminated lupus erythematosus, lupus, systemic lupus erythematosus (diagnosis), systemic lupus, systemic lupus erythematosus, Lupus, Lupus erythematosis disseminated, Syndrome disseminated lupus erythematosis, Systemic lupus erythematosis, Systemic lupus erythematosus synd, Lupus erythematosus systemic, LE syndrome, LE systemic, Lupus syndrome, Syndrome lupus, LUPUS, Syst lupus erythematosus, Systemic lupus erythematosus NOS (disorder), Systemic lupus erythematosus (SLE), SLE NOS, Lupus Erythematosus, Systemic [Disease/Finding], erythematosu lupus systemic, les syndrome, sles, syndrome lupus, systemic lupus erythematosus (SLE), lupus erythematosis, lupus syndrome, le syndrome, erythematosis lupus systemic, lupus erythematosus disseminatus, lupus syndromes, SLE, Systemic lupus erythematosus, Disseminated lupus erythematosus, SLE - Systemic lupus erythematosus, Systemic lupus erythematosus (disorder), erythematosus; lupus, systemic, lupus; erythematosus, systemic, system; lupus erythematosus, SLE - Lupus Erythematosus, Systemic
French LUPUS ERYTHEMATEUX DISSEMINE, Syndrome lupique, Syndrome du LE, LES, Syndrome de lupus érythémateux disséminé, Syndr de lupus érythémateux systémique, LE systémique, LUPUS ERYTHEMATEUX SYSTEMIQUE, SYNDROME DE LUPUS ERYTHEMATEUX, Lupus érythémateux généralisé, Lupus érythémateux disséminé, Lupus érythémateux systémique, LED (Lupus Érythémateux Disséminé)
Portuguese LUPUS ERITEMATOSO SISTEMICO, Síndrome de LE, Lúpus eritematoso disseminado, Lúpus eritematoso sistémico, LES, LE sistémico, Síndrome lúpica, Síndrome de eritematose lúpus disseminado, Síndrome de lúpus eritematoso sistémico, Lúpus Eritematoso Disseminado, Lúpus Eritematoso Sistêmico, LUPUS ERITEMATOSO SISTEMIC, SINDROMA DE LUPUS ERITEMAT, Lupus eritematoso sistémico, LUPUS ERITEMATOSO DISSEMINADO
Spanish LUPUS ERITEMATOSO SISTEMICO, LUPUS ERITEMATOSO DISEMINADO, Lupus eritematoso diseminado, Síndrome similar al lupus, LE sistémico, Síndrome lúpico, Síndrome de lupus eritematoso diseminado, Síndrome LE, Systemic lupus erythematosus, LUPUS ERITEMATOSO, SINDROME, LES, SAI, LES, lupus eritematoso diseminado, lupus eritematoso sistémico (trastorno), lupus eritematoso sistémico, SAI (trastorno), lupus eritematoso sistémico, SAI, lupus eritematoso sistémico, Lupus eritematoso sistémico, Lupus Eritematoso Diseminado, Lupus Eritematoso Sistémico, Lupus Eritematoso Sistemico
Dutch lupus erythematodes disseminatus, SLE, lupus erythematodes systemisch, lupussyndroom, lupus erythematosus disseminatus, LE syndroom, systemische lupus erythematosis, LE systemisch, gegeneraliseerd lupus erythematosus syndroom, systemisch lupus erythematosus syndroom, erythematodes; lupus, systemisch, lupus; erythematodes, systemisch, systeem; lupus erythematodes, Lupus erythematodes disseminatus [LED], niet gespecificeerd, gegeneraliseerde lupus erythematosus, Lupus erythematodes disseminatus [LED], LED, Lupus erythematosus disseminatus, Systemische lupus erythematosus
German Lupus-Syndrom, Syndrom, disseminierter Lupus erythematosis, LE-Syndrom, systemischer Lupus erythematosus synd, LE systemisch, systemischer Lupus erythematosus, diseminierter Lupus erythematosus, Lupus erythematosus systemisch, SLE, Syndrom Lupus, Lupus erythematosus disseminiert, LE SYNDROM, LUPUS ERYTHEMATODES DISSEMINATUS, LUPUS ERYTHEMATODES SYSTEMISCH, SYSTEMISCHES LUPUS ERYTHEMATODES, Systemischer Lupus erythematodes, nicht naeher bezeichnet, Systemischer Lupus erythematodes, systemischer Lupus erythematodes, Lupus erythematodes disseminatus, Lupus erythematodes, systemischer
Italian Sindrome da lupus eritematoso, LES, Sindrome da lupus eritematoso disseminato, Sindrome da lupus, Sindrome lupoide, Sindrome da lupus eritematoso sistemico, Lupus eritematoso disseminato, Lupus eritematoso sistemico
Japanese 全身性エリテマトーデス症候群, LE症候群, SLE, ゼンシンセイエリテマトーデス, ハシュセイエリテマトーデス, LEショウコウグン, SLE, ゼンシンセイエリテマトーデスショウコウグン, 全身性LE, ゼンシンセイLE, ループスショウコウグン, 播種性エリテマトーデス症候群, ハシュセイエリテマトーデスショウコウグン, ループス症候群, 急性び漫性紅斑性狼瘡, 紅斑性狼瘡-全身性, 全身性紅斑性狼瘡, リブマン-サックス症候群, Libman-Sacks病, 播種状紅斑性狼瘡, 急性エリテマトーデス, 全身性エリテマトーデス, 播種状エリテマトーデス, リブマン-サックス病, エリテマトーデス-播種性, 播種性エリテマトーデス, Libman-Sacks症候群, エリテマトーデス-全身性
Swedish Systemisk lupus erythematosus
Czech lupus erythematosus systémový, Lupus erytematosus disseminovaný, SLE, Syndrom lupusu, Systémový lupus erytematosus, Systémový LE, Lupus erytematosus systémový, Lupusový syndrom, Diseminovaný lupus erytematosus, Syndrom difuzního lupusu erytematodu, LE syndrom, Sytémový lupus erytematosus -syndrom
Finnish Systeeminen lupus erythematosus
Russian LIBMANA-SAKSA BOLEZN', ENDOKARDIT VOLCHANOCHNYI, VOLCHANKA KRASNAIA SISTEMNAIA, VOLCHANKA ERITEMATOZNAIA SISTEMNAIA, ВОЛЧАНКА КРАСНАЯ СИСТЕМНАЯ, ВОЛЧАНКА ЭРИТЕМАТОЗНАЯ СИСТЕМНАЯ, ЛИБМАНА-САКСА БОЛЕЗНЬ, ЭНДОКАРДИТ ВОЛЧАНОЧНЫЙ
Korean 상세불명의 전신 홍반성 루푸스, 전신 홍반성 루푸스
Croatian LUPUS, ERITEMSKI SISTEMSKI, SISTEMSKI ERITEMSKI LUPUS
Polish Liszaj rumieniowaty narządowy, Liszaj rumieniowaty trzewny, Toczeń rumieniowaty układowy, Toczeń trzewny
Hungarian LE-syndroma, Lupus erythematosus, disszeminált, Disseminált lupus erythematosus, Systemás LE, SLE, Szisztémás lupus erythematosus, Systemás lupus erythematosus, Systemás lupus erythematosus syndroma, Lupus-syndroma, Disseminált lupus erythematosus syndroma, Lupus syndroma

Ontology: Systemic lupus erythematosus (SMQ) (C1869047)

Definition (MDRCZE) Chronická zánětlivá autoimunitní porucha zasahující mnoho tělesných systémů s různou závažností charakterizovanou různými protilátkami.Protilátky dvouvláknové DNA a polypeptidů Sm Protilátky antifosfolipidů (včetně antikoagulantu lupusu, protilátek antikardiolipinu a protilátek, které vedou k falešně pozitivním výsledkům testu na syfilis)Aktivace komplementu vede k formaci imunního komplexu. Léky mohou vyvolat symptomy systémového erytematózního lupusu (SLE) de novo anebo prohloubit existující symptomy. Klinické obrazy se liší závažností a nástupem.Systémové symptomy: anorexie, hubnutí, nevolnost, myalgie, artralgie a horečkaKožní manifestace (např. makulární „motýlí" erytém, diskovité léze, makulopapulární léze, erytém po stranách dlaní, červené nebo fialové makulární léze na povrchu prstů, plešatost, periungvální erytém, léze membrán sliznic, purpura a citlivost na světloPolyartritida Nefritida může dojít k proteinurii, difúzní membranoproliferativní glomerulonefritidě, abnormálnímu rozboru moči (hematurie, pyurir, válečky v moči) nebo zvýšení kreatininu v séru, hypertenzi a nefrotickému syndromu.Manifestace centrálního nervového systémuBolesti hlavy, epilepsie, psychózy, organický mozkový syndrom a změny osobnosti, mrtvice nebo přechodné ischemické ataky kvůli vaskulární okluziOstatní manifestace / komplikaceKardiovaskulární: perikarditida, myokarditida, fibrinová (Libman-Sachsova) endokarditida a nedomykavosti chlopníPulmonální: pleuritida s výtokem nebo bez nějHematologické: anemie, leukopenie a trombocytopenie kvůli hemolýzeGastrointestinální: bolesti břicha, nevolnost, zvracení a průjemOční: syndrom vysoušení, nespecifický zánět spojivky, zánět sítnice a zánět zrakového nervu
Definition (MDRGER) Chronisch-entzündliche Autoimmunerkrankung, die viele Körpersysteme mit mit verschiedenen Autoantikörpern mit unterschiedlicher Schwere angreift o Antikörper mit Doppel-Strang-DNA und Sm-Polypeptide o Antiphospholipid-Antikörper (einschließlich Lupus-Antikoagulanz, Antikardiolipin-Antikörper und solche, die einen falsch positiven Syphilistest verursachen) o Die Komplementaktivierung führt zur Immunkomplexablagerung in Blutgefäßen Arzneimittel können Symptome von systemischem Lupus erythematosus (SLE) neu auslösen bzw. die Verschlimmerung bestehender Symptome verursachen. Klinische Präsentationen sind unterschiedlich in Bezug auf Schwere und Ausbruch. o Systemische Symptome: Magersucht, Gewichtsabnahme, Unwohlsein, Myalgie, Arthralgien und Fieber o Kutanmanifestationen (z.B. makuläres „Schmetterling"-Erythem, diskoide Läsionen, makulapapuläre Läsionen, Erythem seitlich an den Handflächen; rote oder lila makulare Läsionen an den volaren Fingeroberflächen, Alopezia, periunguales Erythem, Schleimhautläsionen, Purpura und Photosensibilität o Polyarthritis o Nephritis Proteinurie, diffuse membranproliferative Glomerulonephritis, anomale Urinanalyse (Hämaturie, Pyrurie, Harnzylinder) oder Kreatinin erhöht im Serum; Bluthochdruck und nephrotisches Syndrom möglich o Zentralnervensystem betreffende Manifestationen Kopfschmerzen, Epilepsie, Psychosen, organisches Hirnsyndrom, Persönlichkeitsänderungen, Schlaganfall und transiente ischämische Attacken o Andere Manifestationen/Komplikationen Perikarditis, Myokarditis, fibrinöse (Libman-Sachs) Endokarditis und Ventilerkrankungen Pleuritis mit oder ohne Effusion Anämia, Leukopenie und Thrombozytopenie durch Hämolyse Bauchschmerzen, Übelkeit, Erbrechen und Diarrhoe Sicca-Syndrom, unspezifische Konjunktivitis, retinale Vaskulitis und optische Neuritis
Definition (MDRJPN) 種々の自己抗体によって特徴づけられる様々な重症度を呈する多数の身体器官に及ぶ慢性の炎症性自己免疫疾患である。 2本鎖DNAおよびSmポリペプチドに対する抗体 抗リン脂質抗体(ループス性抗凝固因子、抗カルジオリピン抗体、および梅毒の偽陽性検査結果をもたらす抗体を含む) 免疫複合体を形成する補体の活性化。 薬剤によって全身性エリテマトーデス(SLE)の新規症状の誘発や既存の症状の悪化が起こる可能性がある。 臨床症状は重症度や発症によってさまざまである。 全身症状:食欲不振、体重減少、倦怠感、筋肉痛、関節痛、および発熱‐皮膚症状(例、斑状蝶形紅斑、円板状病変、斑状丘疹性病変、手のひら辺縁の紅斑、掌側の指の表面の赤色または紫色の斑状病変、脱毛症、爪周囲の紅斑、粘膜病変、紫斑、および光線過敏性)。 多発性関節炎‐腎炎‐蛋白尿、びまん性膜増殖性糸球体腎炎、尿検査の異常(血尿、膿尿、尿円柱)、または血清クレアチニン値上昇の他に、高血圧やネフローゼ症候群が起こることもある。 中枢神経系症状:頭痛、てんかん、精神病、器質性脳症候群、人格変化、脳卒中、および一過性脳虚血発作。 その他の症状/合併症:心膜炎、心筋炎、線維性(リブマン‐サックス)心内膜炎、および弁膜不全‐滲出を伴うまたは伴わない胸膜炎‐溶血に起因する貧血、白血球減少症、および血小板減少症‐腹痛、悪心、嘔吐、および下痢‐乾燥症候群、非特異性結膜炎、網膜血管炎、および視神経炎。
Definition (MDRHUN) Krónikus gyulladásos autoimmun betegség, ami számos szervrendszert érinthet különböző súlyossági fokkal, és melyet különböző auto-antitestek jelenléte jellemez. Antitestek duplaszálú DNS molekulákhoz és Sm polypeptidekhez. Az antifoszfolipid antitestek (ideértve a lupus anticoagulánst, az anticardiolipin antitesteket, és azokat is, melyek szifilisz fals-pozitív teszteket produkálnak).A komplemens aktiválása immunkomplex formációhoz vezet. A gyógyszerek de novo beindíthatnak systemás lupus erythematosus (SLE) tüneteket, vagy a meglévőeket súlyosbíthatják. A klinikai megjelenés eltérő lehet a súlyos és éppen megjelenő között. Systemás symptomák: anorexia, súlyvesztés, rossz közérzet, izomfájdalom, ízületi fájdalom és láz. Cután megjelenések (vagyis pl. maculosus "pillangó" erythema, discoid laesiók, maculopapulosus laesiók, erythema a tenyér oldalakon, vörös, vagy lila maculosus laesiók a tenyér ujj felületeken, alopecia, periungualis erythema, mucus membran laesiók, purpura és photosensitivitas; polyarthritis; nephritis. Proteinuria, diffúz membranoproliferativ glomerulonephritis, vizeletvizsgálattal kóros (haematuria, pyruia, vizelet cilinderek), vagy emelkedett szérum kreatinin; hypertensió és nephrosis syndroma fordulhat elő. Központi idegrendszeri manifesztációk: fejfájás, epilepszia, psychosis, organikus agy syndroma, személyiség megváltozása, sztrók és átmeneti ischaemiás rohamok. Egyéb manifesztációk/komplikációk: pericarditis, myocarditis, fibrosus (Libman-Sachs) endocarditis, és billentyű elégtelenség. Pleuritis effusióval, vagy a nélkül. Hemolízis miatti anaemia, leukopenia és thrombocytopenia. Abdominalis fájdalom, nausea, hányás és hasmenés. Sicca syndroma, nem-specifikus conjunctivitis, retinalis vasculitis és opticus neuritis.
Definition (MDR) - Chronic inflammatory autoimmune disorder affecting many body systems with variable severity characterized by various autoantibodies o Antibodies to double-stranded DNA and Sm polypeptides o Antiphospholipid antibodies (including lupus anticoagulant, anticardiolipin antibodies, and those that cause false-positive tests for syphilis) o Complement activation leads to immune complex formation - Drugs may trigger systemic lupus erythematosus (SLE) symptoms de novo or exacerbate existing symptoms - Clinical presentations vary in severity and onset o Systemic symptoms: anorexia, weight loss, malaise, myalgia, arthralgias and fever o Cutaneous manifestations (e.g., macular "butterfly" erythema, discoid lesions, maculopapular lesions, erythema on sides of the palms, red or purple macular lesions on volar finger surfaces, alopecia, periungual erythema, mucus membrane lesions, purpura and photosensitivity o Polyarthritis o Nephritis - Proteinuria, diffuse membranoproliferative glomerulonephritis, abnormal urinalysis (hematuria, pyruia, urinary casts) or increased serum creatinine; hypertension and nephrotic syndrome can occur o Central nervous system manifestations - Headaches, epilepsy, psychoses, organic brain syndrome, personality changes, stroke and transient ischemic attacks d o Other manifestations/complications - Pericarditis, myocarditis, fibrinous (Libman-Sachs) endocarditis, and valve insufficiencies - Pleurisy with or without effusion - Anemia, leukopenia and thrombocytopenia due to hemolysis - Abdominal pain, nausea, vomiting and diarrhea - Sicca syndrome, nonspecific conjunctivitis, retinal vasculitis and optic neuritis
Definition (MDRSPA) • Es un trastorno autoinmune inflamatorio y crónico que afecta muchos sistemas del organismo con gravedad variable caracterizado por varios autoanticuerpos o Anticuerpos anti-ADN de doble cadena y anti-polipéptidos Sm. o Anticuerpos antifosfolípidos (incluyendo anticoagulante lúpico, anticuerpos anticardiolipina y aquellos que causan falsos positivos en pruebas de sífilis) o La activación del complemento deriva en depósitos de inmunocomplejos • Los fármacos pueden desencadenar síntomas de Lupus eritematoso sistémico (LES) de novo o exacerbar los síntomas existentes • Las presentaciones clínicas varían en gravedad e inicio o Síntomas sistémicos: anorexia, pérdida de peso, malestar general, mialgia, artralgias y fiebre o Manifestaciones cutáneas (p.ej. exantema macular en forma de "alas de mariposa", lesiones discoides, lesiones maculopapulares, eritema en los costados de las palmas; lesiones maculares de color rojo o púrpura en la superficie volar de los dedos de la mano, alopecia, eritema periungueal, lesiones en la membrana mucosa, púrpura y fotosensibilidad o Poliartritis o Nefritis Proteinuria, glomerulonefritis membranoproliferativa difusa, análisis de orina anormal (hematuria, piuria, cilindros urinarios), o creatinina sérica elevada, puede ocurrir hipertensión y síndrome nefrótico o Manifestaciones en el sistema nervioso central Cefaleas, epilepsia, psicosis, síndrome cerebral orgánico y cambios de la personalidad, ictus o accidente isquémico transitorio o Otras manifestaciones/complicaciones Pericarditis, miocarditis, endocarditis fibrinosa (de Libman-Sachs) e insuficiencias valvulares Pleuritis con o sin derrame Anemia, leucopenia y trombocitopenia debido a hemólisis Dolor abdominal, náuseas, vómitos y diarrea Síndrome sicca, conjuntivitis inespecífica, vasculitis retiniana y neuritis óptica
Definition (MDRITA) • Patologia infiammatoria cronica autoimmunitaria che interessa molti sistemi del corpo con gravità variabile caratterizzata da vari autoanticorpi. o Anticorpi contro la doppia elica del DNA e i polipeptidi Sm. o Anticorpi antifosfolipidi (inclusi gli anticorpi anticardiolipina anticoagulanti del lupus, e quelli che causano test falsamente positivi per la sifilide) L'attivazione del complemento induce al deposito di complessi immunitari. • I farmaci possono causare i sintomi del lupus eritematoso sistemico de novo oppure esacerbare sintomi esistenti. • Le presentazioni cliniche variano per gravità e manifestazione. o Sintomi sistemici: anoressia, perdita di peso, malessere, mialgia, artralgia e febbre. o Manifestazioni cutanee (ad es., eritema maculare a "farfalla" , lesioni discoidali e maculopapulari, eritema sui lati dei palmi, lesioni maculari rosse o viola sulle superfici delle dita palmari e plantari, alopecia, eritema periunguale, lesioni alle membrane mucose, porpora e fotosensibilità o Poliartrite o Nefrite: proteinuria, glomerulonefrite membranoploriferativa diffusa, urinanalisi anormale (ematuria, piuria, sedimenti urinari), o creatinina sierica aumentata; si possono verificare ipertensione e sindrome nefrotica o Manifestazioni del sistema nervoso centrale Mal di testa, epilessia, psicosi, sindrome cerebrale organica e alterazioni della personalità, ictus o attacchi ischemici transitori o Altre manifestazioni/complicazioni pericardite, miocardite, endocardite fibrinosa (Libman-Sachs) e insufficienze valvolari pleurite con o senza effusione anemia, leucopenia e trombocitopenia dovuta a emolisi dolore addominale, nausea, vomito e diarrea sindrome sicca, congiuntivite aspecifica, vasculite retinica e nevrite ottica
Definition (MDRFRE) • Affection autoimmune inflammatoire chronique affectant plusieurs systèmes du corps selon différents degrés de sévérité et caractérisé par divers anticorps o Anticorps anti-ADN double brin et anti-polypeptides Sm o Anticorps antiphospholipides (comprenant l'anticoagulant lupique, les anticorps anticardiolipine et ceux produisant des résultats faux positifs pour la syphilis) o L'activation complémentaire entraîne la formation de complexe immun • Les médicaments peuvent déclencher les symptômes de novo du lupus érythémateux systémique (LES) ou exacerber les symptômes existants • La sévérité et la manifestation des symptômes varient selon les présentations cliniques o Symptômes systémiques : anorexie, perte de poids, malaise, myalgie, arthralgies et fièvre o Manifestations cutanées (par exemple, érythème maculaire en ailes de papillon, lésions discoïdes, lésions maculopapulaires, érythème des côtés des paumes, lésions maculaires rouges ou pourpres sur la face palmaire des doigts, alopécie, érythème périunguéal, lésions de la muqueuse, purpura et photosensibilité o Polyarthrite o Néphrite Protéinurie, glomérulonéphrite membranoproliférative diffuse, urines anormales (hématurie, pyurie, cylindres urinaires) ou augmentation de la créatinine sérique ; hypertension et syndrome néphrotique peuvent survenir o Manifestations du système nerveux central Céphalées, épilepsie, psychoses, syndrome de cerveau organique et changements de la personnalité, accident vasculaire cérébral ou crises ischémiques transitoires o Autres manifestations/complications péricardite, myocardite, endocardite fibrineuse (Libman-Sachs) et insuffisances de valves Pleurésie avec ou sans effusion Anémie, leucopénie et thrombocytopénie dues à l'hémolyse Douleurs abdominales, nausées, vomissements et diarrhée syndrome de Gougerot-Sjögren, conjonctivite non précisée, vascularite rétinienne et névrite optique
Definition (MDRDUT) • Chronische inflammatoire auto-immuunziekte die vele lichaamsstelsels met variabele mate van ernst beïnvloeden, gekenschetst door diverse auto-antistoffen o Antistoffen voor dubbelstrengig DNA en SM polypeptiden o Antifosfolipiden-antistoffen (met inbegrip van lupus-anticoagulans, anticardiolipine-antistoffen en die welke valspositieve tests voor syfilis veroorzaken) o Complement-activering leidt tot de depositie van immuuncomplex • Geneesmiddelen kunnen de novo symptomen van systemische lupus erythematodes triggeren of bestaande symptomen verergeren • Klinische presentaties variëren wat ernst en onset betreft o Systemische symptomen: anorexie, gewichtsverlies, malaise, myalgie, artralgieën en koorts o Cutane manifestaties (bijv. maculair vlindervormig erytheem, discoïde laesies, maculopapulaire laesies, erytheem aan zijden van de palmen; rode of paarse maculaire laesies op volaire vingeroppervlakken, alopecie, periunguaal erytheem, slijmvlieslaesies, purpura en lichtgevoeligheid o Polyartritis o Nefritis proteïnurie, diffuse membranoproliferatieve glomerulonefritis, abnormale urineanalyse (hematurie, pyurie, urinesediment) of verhoogd serumcreatinine; hypertensie en nefrotisch syndroom kunnen zich voordoen o Manifestaties van het centraal zenuwstelsel Hoofdpijn, epilepsie, psychosen, organisch hersensyndroom, persoonlijkheidsveranderingen, beroerte en transient ischaemic attacks o Andere manifestaties/complicaties Pericarditis, myocarditis, fibrineuze (Libman-Sachs) endocarditis en klepinsufficiënties Pleuritis met of zonder effusie Anemie, leukopenie en trombocytopenie als gevolg van hemolyse Buikpijn, misselijkheid, braken en diarree Sicca-syndroom, niet-specifieke conjunctivitis, retinavasculitis en neuritis optica
Definition (MDRPOR) • É uma afecção autoimune inflamatória e crónica que afecta muitos sistemas do organismo com gravidade variável caracterizada por vários autoanticorpos ou Anticorpos anti-ADN de cadeia dupla e anti-polipéptidos Sm. o Anticorpos antifosfolípidos (incluindo anticoagulante lúpico, anticorpos anticardiolipina e aqueles que causam falsos positivos em testes de sífilis) o A activação do complemento leva à formação de depósitos de imunocomplexos • Os fármacos podem desencadear sintomas de Lupus eritematoso sistémico (LES) de novo ou exacerbar os sintomas existentes • A apresentações clínicas variam em gravidade e início o Sintomas sistémicos: anorexia, perda de peso, mal-estar geral, mialgia, artralgias e febre o Manifestações cutâneas (p. ex. Eritema macular em forma de "asas de borboleta", lesões discóides, lesões maculopapulares, eritema nos lados das palmas; lesões maculares de cor vermelha ou púrpura na superfície palmar dos dedos da mão, alopecia, eritema periungueal, lesões na membrana mucosa, púrpura e fotossensibilidade o Poliartrite o Nefrite Proteinúria, glomerulonefrite membranoproliferativa difusa, análise da urina anormal (hematúria, piúria, cilindros urinários), ou creatinina sérica aumentada; pode ocorrer a hipertensão e a síndrome nefrótica o Manifestações do sistema nervoso central Cefaleias, epilepsia, psicose, síndrome cerebral orgânica e mudanças da personalidade, acidente vascular cerebral e ataques isquémicos transitórios o Outras manifestações/complicações Pericardite, miocardite, endocardite fibrinosa (de Libman-Sachs) e insuficiências valvulares Pleurite com ou sem derrame Anemia, leucopenia e trombocitopenia devido a hemólise Dor abdominal, náuseas, vómitos e diarreia Síndrome sicca, conjuntivite não específica, vasculite retiniana e neurite óptica
Concepts Classification (T185)
English Systemic lupus erythematosus (SMQ)
Dutch Systemische lupus erythematodes (SMQ)
French Lupus érythémateux systémique (SMQ)
German Systemischer Lupus erythematodes (SMQ)
Italian Lupus eritematoso sistemico (SMQ)
Spanish Lupus eritematoso sistémico (SMQ)
Portuguese Lúpus eritematoso sistémico (SMQ)
Czech Systémový lupus erythematosus (SMQ)
Japanese 全身性エリテマトーデス(SMQ)
Hungarian Systemás lupus erythematosus (SMQ)