Knowledge guides

LG21: Autoinflammatory disease

Key presentations and conditions

Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.

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Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.

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Investigations, procedures, and clinical assessment tools

Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.

Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.

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Important specific issues

Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.

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Presentations
  • Inflammation, persistent or recurrent, organ-based or systemic, in the absence of autoimmune disease, infection, or malignancy
Conditions
  • Adult-onset Still disease
  • Behçet disease
  • Familial Mediterranean fever
  • Macrophage activation syndrome
  • Sarcoidosis
  • Vacuoles, E1 enzyme, x-linked, autoinflammatory, somatic (VEXAS)

For each presentation and condition, Advanced Trainees will know how to:

Synthesise
  • recognise the clinical presentation
  • identify relevant epidemiology, pathophysiology, and clinical science
  • take a comprehensive clinical history
  • conduct an appropriate examination
  • establish a differential diagnosis
  • plan and arrange appropriate investigation
  • consider the impact of illness and disease on patients and families, and their quality of life
Manage
  • provide evidence-based management
  • prescribe therapies tailored to patients’ needs and conditions
  • recognise potential complications of disease and its management, and initiate preventative strategies
  • involve multidisciplinary teams
Consider other factors
  • identify individual and social factors and the impact of these on diagnosis and management
  • Epidemiology, management, pathophysiological mechanisms, and therapeutics of the listed conditions
  • Investigations and imaging relevant to the diagnosis of the listed conditions
  • Acknowledge when autoinflammatory disease should be considered as a diagnosis
  • Awareness of new autoinflammatory diseases being identified regularly, and the rapid evolution in their diagnostics, imaging, and therapeutics
  • Conduct preliminary investigations to diagnose and treat autoinflammatory diseases
  • Skills to consult current literature when presented with possible rarer forms of disease