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    Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

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    Prakash
    The document discusses neuropsychiatric manifestations in systemic lupus erythematosus (NPSLE) based on revised American College of Rheumatology (ACR) criteria, including varied presentations seen in patients at University Malaya Medical Centre (UMMC). Diagnosis of NPSLE can be challenging due to subtle symptoms and differential diagnoses. Treatment approaches discussed include high dose corticosteroids with or without cyclophosphamide depending on disease severity.

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    Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

    Uploaded by

    Prakash
    68 views17 pages
    The document discusses neuropsychiatric manifestations in systemic lupus erythematosus (NPSLE) based on revised American College of Rheumatology (ACR) criteria, including varied presentations seen in patients at University Malaya Medical Centre (UMMC). Diagnosis of NPSLE can be challenging due to subtle symptoms and differential diagnoses. Treatment approaches discussed include high dose corticosteroids with or without cyclophosphamide depending on disease severity.

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    The document discusses neuropsychiatric manifestations in systemic lupus erythematosus (NPSLE) based on revised American College of Rheumatology (ACR) criteria, including varied presentations seen in patients at University Malaya Medical Centre (UMMC). Diagnosis of NPSLE can be challenging due to subtle symptoms and differential diagnoses. Treatment approaches discussed include high dose corticosteroids with or without cyclophosphamide depending on disease severity.

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    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    68 views17 pages

    Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

    Uploaded by

    Prakash
    The document discusses neuropsychiatric manifestations in systemic lupus erythematosus (NPSLE) based on revised American College of Rheumatology (ACR) criteria, including varied presentations seen in patients at University Malaya Medical Centre (UMMC). Diagnosis of NPSLE can be challenging due to subtle symptoms and differential diagnoses. Treatment approaches discussed include high dose corticosteroids with or without cyclophosphamide depending on disease severity.

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    Neuropsychiatric

    Systemic Lupus
    Erythematosus (NPSLE)
    Case presentations and topic
    discussion
    The Rheumatology Unit UMMC
    experience
    References
     Sanna G, Bertolaccini ML. Neuropsychiatric
    manifestations in systemic lupus erythematosus:
    prevalence and association with antiphospholipid
    antibodies. J Rheumatology 2003; 30: 985-992.
     The American College of Rheumatology
    nomenclature and case definitions for
    neuropsychiatric lupus syndromes. Arthritis
    Rheum 1999;42:599-608
     Bruyn GA. Controversies in lupus: Nervous
    system involvement. Rheum Dis 1995: 54:
    159-167
    CNS Lupus – Current scenario in
    UMMC
     From the beginning of 2005 an
    average of 1 admission a month
    (new case).
     Varied presentations closely following
    the revised ACR criteria ( 19 NPSLE
    definitions)
     Problems with establishing a
    concrete diagnosis and we lack a
    clear treatment protocol.
    CNS Lupus
     Nervous system manifestations are
    present in up to 70% of patients with
    SLE.
     There are 19 definitions which are
    components of NPSLE. The earlier
    classifications only recognized 2
    clinical entities – seizures and
    psychoses
    NPSLE based on the revised ACR
    criteria
     Acute confusional  Cranial nerve
    state disorders
     Anxiety disorders  Mononeuropathy

     Cognitive function  Plexus neuropathy


    impairment  Vegetative
     Affective disorders neuropathy
     psychoses  Myasthenia

     AIDP

     Polyneuropathy
    NPSLE component entities
     Epilepticattacks
     Headaches and migraines

     Cereberovascular diseases

     Demyelinating syndromes

     Aseptic meningitis

     Chorea

     myelopathy
    CNS Lupus – problems in
    diagnosis
     Subtle presentations – wouldn’t I be
    depressed or anxious (or both) if I
    was diagnosed with SLE ?
     Effect of corticosteroids

     Other differential diagnoses?


    Infections and metabolic diseases
     access to imaging facilities
    NPSLE specific antibodies
     Anti ribosomal P antibody
     Anti neuronal antibodies
    Imaging
     MRI by far the most superior
     SPECT scans have also been found
    to be useful
     In the UMMC experience the MRI
    has shown to be of great diagnostic
    value
    MRI Images of 2 patients
     Case 1 – 14 years old Indian girl who
    presented to Kuantan with dense
    right sided hemiplegia who showed
    remarkable recovery with
    intravenous Methyl Prednisolone.
     Low complements, anti dsDNA 276
    iu/mL, Ig ACL 4 units, LA negative
    MRI Images of 2 patients
     12 years old Chinese girl presented
    with seizures while under treatment
    for class IV lupus nephritis.
     Low complements anti dsDNA 167
    iu/mL, IgG ACL 4 units, LA negative.
     Cases presented today had CT scans
    with contrast which were normal but
    with low complements and anti ds
    DNA in the thousands
    NPSLE - CSF
     Varied findings among samples from
    our cases, an indicator which we use
    is the CSF proteins (tend to be
    higher then 0.45)
     More useful to rule out infections.
    Always send for TB culture.
    Therapeutic approach in CNS lupus
    – mild disease
     Symptomatic therapy
     Analgesics
     Anxiolytics
     Antidepressants
     Tricyclics
     Fluoxetine
     Anti convulsants
     Anti psychotics
     Low dose corticosteroids
    Therapeutic approach in CNS Lupus –
    severe CNS disease: diffuse/nonthrombotic
    disease
     Acute treatment
     High dose corticosteroids
     Iv pulse methylprednisolone
     Iv pulse cyclophosphamide
     Plasmapharesis
     Iv immunoglobulins
     Intrathecal methotrexate
     Azathioprine
     Mycophenolate mofetil
    Therapeutic approach in CNS Lupus –
    severe CNS disease: diffuse/nonthrombotic
    disease
     Chronic treatment

     Taper corticosteroids

     Iv pulse cyclophosphamide

     Methotrexate

     Azathioprine

     Mycophenolate mofetil
    Current practice at UMMC
     Intravenous methylprednisolone
    500mg daily for 3 days followed by
    one of 2 cyclophosphamide regimes
     1) monthly courses of 1.0grams/BSA
    for 6 months followed by 3 monthly
    courses.
     2) 2 weekly courses of 500mg total
    of 6 doses (3 grams in total)
    Current practice at UMMC
     Symptomatic treatment is accompanied by
    intravenous methylprednisolone and high
    dose 1mg/kg of oral prednisolone daily
    which is tapered. Usually a steroid sparing
    agent such as azathioprine is added.
     Hydroxychloroquine

     Role for cyclophosphamide in such


    patients? At which doses – 1 or 2?

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