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Systemic Lupus Erythematosus

OVERVIEW

  • Systemic Lupus Erythematosus (SLE) = chronic, multi-system disease commonest in young females
  • wide spread antibodies -> produce tissue damage
  • polyclonal B-cell secretion -> formation of immune complexes with deposit in a variety of sites
  • may be triggered by EBV
  • long term risks: OP and CVS disease

CLINICAL FEATURES

History

  • remitting and relapsing illness
  • malaise, fatigue, myalgia, fever
  • lymphadenopathy
  • weight loss
  • multisystem symptoms

Examination

  • SKIN: skin and joint involvement common, oral and pharyngeal ulceration, alopcecia
  • CARDIOVASCULAR: pericarditis 15%, myocarditis, endocarditis (Libman-Sacks syndrome), Raynauds phenomenon, IHD, arteritis -> ischaemia
  • RESPIRATORY: infections and PE’s, pleuralitis, pleural effusion, pulmonary fibrosis
  • NEUROLOGICAL: cranial and peripheral nerve lesions, transverse myelitis -> weakness and paraplegia, depression, psychosis, seizures, CVA
  • RENAL: glomerulonephritis -> renal failure
  • HAEMATOLOGICAL: clotting disorders, hypercoagulable state, anti-phospholipid syndrome

INVESTIGATIONS

  • thrombocytopaenia
  • coagulopathy
  • U+E
  • CXR
  • ECHO
  • CT/MRI

DIAGNOSTIC CRITERIA (require >4)

  • malar rash (butterfly)
  • discoid rash
  • photosensitivity
  • oral ulcers
  • non-erosive arthritis
  • serositis: pleuritis, pericarditis
  • renal: proteinuria, casts
  • central nervous: seizures, psychosis
  • haematological: haemolytic anaemia, leucopenia, thrombocytopenia
  • immune: anti-dsDNA, anti-smooth muscle antibody, anti-phospholipid antibody, anti-nuclear anti-body

MANAGEMENT

  • cutaneous: topical steroids, sunblock
  • NSAIDS
  • hydroxychloroquine
  • low dose steroids
  • azathioprine
  • methotrexate
  • mycophenolate
  • severe flare: cyclophosphamide + high dose steroids
  • B cell depletion: rituximab

Influence on ICU Management

  • proportional to severity, stability and stability of disease
  • immunosuppressed
  • skin care
  • VTE risk
  • risk of renal injury
  • adrenal suppression
  • haematological abnormalities
  • may have restrictive lung disease

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also a Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of three amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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