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, where he is Deputy Director (Education). He is a Clinical Adjunct Associate Professor at Monash University, the Lead for the  Clinician Educator Incubator programme, and a CICM First Part Examiner.

He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives. He was one of the founders of the FOAM movement (Free Open-Access Medical education) has been recognised for his contributions to education with awards from ANZICS, ANZAHPE, and ACEM.

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

On Bluesky, he is @precordialthump.bsky.social and on the site that Elon has screwed up, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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