systemic%20lupus%20erythematosus
SYSTEMIC LUPUS ERYTHEMATOSUS
Systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation.
It can be suspected when ≥2 organ systems are involved.
It is predominantly diagnosed in females of childbearing age, rarely diagnosed before 8 years old.
Clinical presentation varies in different patients and the disease activity varies over time in a single patient. Majority of patients have arthralgia of the hand.

Diagnosis

  • Diagnosis of systemic lupus erythematosus (SLE) is based on clinical symptoms & laboratory findings

Classification

Diagnosis based on the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE

  • ≥4 criteria (at least 1 clinical & 1 immunologic criteria) OR biopsy-proven lupus nephritis w/ positive antinuclear antibody (ANA) or Anti-DNA
  • Symptom/finding need not be present all at the same time
Clinical Criteria:
    1) Acute cutaneous lupus, including:
    • Lupus malar rash (do not count if malar discoid)
    • Bullous lupus
    • Toxic epidermal necrolysis variant of SLE
    • Maculopapular lupus rash
    • Photosensitive lupus rash (in the absence of dermatomyositis)
    • OR
    • Subacute cutaneous lupus (nonindurated psoriaform &/or annular polycyclic lesions that resolve without scarring, although occasionally w/ post-inflammatory dyspigmentation or telangiectasias)
    2) Chronic cutaneous lupus, including:
    • Classic discoid rash: localized (above the neck) or generalized (above & below the neck)
    • Hypertrophic (verrucous) lupus
    • Lupus panniculitis (profundus)
    • Mucosal lupus
    • Lupus erythematosus tumidus
    • Chillblains lupus
    • Discoid lupus/lichen planus overlap
    3) Oral Ulcers OR Nasal Ulcers
    • Oral: palate, buccal, tongue
    • In the absence of other causes, such as vasculitis, Behcet’s disease, infection (herpesvirus), inflammatory bowel disease, reactive arthritis, & acidic foods
    4) Nonscarring alopecia
    • Diffuse thinning or hair fragility w/ visible broken hairs
    • In the absence of other causes such as alopecia areata, drugs, iron deficiency, & androgenic alopecia
    5) Synovitis involving ≥2 joints
    • Characterized by swelling or effusion
    • OR tenderness in ≥2 joints & at least 30 minutes of morning stiffness
    6) Serositis
    • Typical pleurisy for >1 day OR pleural effusions OR pleural rub
    • Typical pericardial pain (pain w/ recumbency improved by sitting forward) for >1 day OR pericardial effusion OR pericardial rub OR pericarditis by electrocardiography
    • In the absence of other causes, such as infection, uremia, & Dressler’s pericarditis
    7) Renal
    • Urine protein–to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours OR red blood cell casts
    8) Neurologic
    • Seizures
    • Psychosis
    • Mononeuritis multiplex (in the absence of other known causes such as primary vasculitis)
    • Myelitis
    • Peripheral or cranial neuropathy (in the absence of other known causes such as primary vasculitis, infection, & diabetes mellitus)
    • Acute confusional state (in the absence of other causes, including toxic/metabolic, uremia, drugs)
    9) Hemolytic anemia

    10) Leukopenia (<4000/mm3) at least once, in the absence of other known causes such as Felty’s syndrome, drugs, & portal hypertension
         OR
          Lymphopenia (<1000/mm3) at least once, in the absence of other known causes such as corticosteroids, drugs, & infection
    11) Thrombocytopenia (<100,000/mm3)
    • At least once in the absence of other known causes such as drugs, portal hypertension, & thrombotic thrombocytopenic purpura
Immunologic Criteria:
    1.ANA level above laboratory reference range
    2.Anti-dsDNA antibody level above laboratory reference range [or >2-fold the reference range if tested by enzyme-linked immunosorbent assay (ELISA)]
    3.Anti-Sm: presence of antibody to Sm nuclear antigen
    4.Antiphospholipid antibody positivity, as determined by:
    1. Positive test for lupus anticoagulant
    2. False-positive test result for rapid plasma reagin
    3. Medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM)
    4. Positive test result for anti-B2-glycoprotein I (IgA, IgG, or IgM)
    5.Low complement (C3, C4, or CH50)
    6.Direct Coombs’ test (in the absence of hemolytic anemia)

Complications

Comorbidities & Complications of Systemic Lupus Erythematosus (SLE)
  • Comorbidities for which SLE patients are at an increased risk include infections, hypertension, atherosclerosis, dyslipidemia, diabetes, osteoporosis, avascular necrosis & malignancies
Lupus Nephritis
  • Patients suspected to have lupus nephritis should immediately undergo renal biopsy to confirm diagnosis, evaluate severity, determine prognosis & therapy
  • Indications for renal biopsy in SLE patients
    • Unexplained increase in serum creatinine in the absence of alternative causes (eg sepsis, hypovolemia, medications)
    • Confirmed proteinuria (≥1.0 gm/24 hours)
    • Presence of the following (result of 2 tests done w/in a short period of time, w/o other causes):
      • Protenuria of >5.0 gm/24 hours & hematuria (≥5 RBCs/hpf) OR
      • Protenuria of >5.0 gm/24 hours & cellular casts
  • Recommended tests used for monitoring of lupus nephritis:
  • Active nephritis at onset of treatment

    Previous active nephritis, none currently

    Pregnant w/ active glomerulonephritis at onset of treatment

    Pregnant w/ previous nephritis, none currently

    No prior or current nephritis

    Blood pressure

    1

    3

    1

    1

    3

    Urinalysis

    1

    3

    1

    1

    6

    Protein/Creatinine ratio

    1

    3

    1

    3

    6

    Serum creatinine

    1

    3

    1

    3

    6

    C3/C4 levels

    2

    3

    1

    3

    6

    Anti-DNA

    1

    3

    1

    1

    3

    *Values are at monthly intervals indicating minimum time at which the test should be measured
    Modified from: 2012 American College of Rheumatology Guidelines for Screening, Treatment, and Management of Lupus Nephritis
Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)
  • Monitor SLE patients for neurological &/or psychiatric manifestations as in non-NPSLE patients
  • Usually appears w/in 1 year from the time of diagnosis; may also appear before or at the time of diagnosis
  • Diagnostic work-up may include the following:
    • Lumbar puncture
    • Nerve conduction studies (NCS)
    • Cerebrospinal fluid (CSF) analysis
    • Electroencephalography (EEG)
    • Neuropsychological assessment of cognitive function
    • Neuroimaging: T1/T2 MRI, diffusion-weighted imaging, gadolinium-enhanced T1 sequences
  • Patients found to have NPSLE should be referred to a team of psychiatrists, psychologists, neurologist & rheumatologist
Toxicity
  • Patients on long-term glucocorticoids should be monitored for:
    • Electrolyte, glucose & lipid levels to identify metabolic conditions
    • Bone densitometry to identify osteoporosis & monitor response to treatment
  • Patients on Hydroxychloroquine should have ophthalmological exam every 6-12 months to detect retinal toxicity
  • Patients on immunosuppressants should be monitored for hematologic, liver & renal toxicity, & occurrence of infection
Others
  • Patients w/ severe hemolytic anemia require hematocrit & reticulocyte measurement weekly at disease onset
  • Patients w/ severe thrombocytopenia require platelet count weekly at disease onset
  • Primary & secondary prevention of thrombosis is needed in SLE patients w/ antiphospholipid antibodies
    • Use of low dose aspirin is recommended
  • Pregnancy may increase SLE activity & cause flares
Hospital Admission
  • Hospital admission is advised for:
    • Those w/ life-threatening or serious organ system involvement
    • Those requiring high-dose corticosteroid
    • Those needing chemotherapy
    • Those w/ severe hypertension
    • Those w/ unexplained fever to rule out sepsis
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