Overview VEXAS syndrome is a late-onset, adult autoinflammatory disease caused by acquired somatic mutations in the UBA1 gene, leading to dysregulated innate immunity, systemic inflammation, and overlapping haematologic and rheumatologic manifestations.  VEXAS stands for Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic. It predominantly affects older males due to its X-linked nature and typically presents with refractory […]

Overview Osteonecrosis of the hip, usually referring to osteonecrosis of the femoral head (ONFH), is a progressive disorder caused by compromised subchondral blood supply leading to death of osteocytes and marrow elements, failed structural repair, subchondral fracture, femoral head collapse, and eventually secondary hip osteoarthritis.[1-4]  It most often affects younger to middle-aged adults, commonly between […]

Overview Amyloidosis is a disorder in which misfolded proteins aggregate into insoluble beta-pleated sheet fibrils and deposit extracellularly in tissues, leading to progressive organ dysfunction. It may be systemic or localized.  In current practice, the most clinically important systemic forms are AL (light-chain), AA (serum amyloid A), and ATTR (transthyretin; hereditary or wild-type). The phenotype […]

Overview Behçet’s syndrome is a chronic, relapsing, multisystem inflammatory disorder and variable-vessel vasculitis characterized classically by recurrent oral ulcers, genital ulcers, ocular inflammation, and skin lesions. It can also involve the joints, vessels, nervous system, and gastrointestinal tract.  The disease is most prevalent along the historic “Silk Road” region, including Turkey, the Middle East, and […]

Overview Chronic spontaneous urticaria (CSU) is a mast-cell–driven skin disorder defined by recurrent wheals (hives), angioedema, or both for >6 weeks without a specific external trigger, with a point prevalence around 0.5–1% and a female predominance; median disease duration is ~2–5 years, but a subset persists longer and markedly impairs quality of life and work […]

Overview Anaphylaxis is a severe, systemic hypersensitivity reaction that is rapid in onset and potentially fatal, most often triggered by foods, medications, or venom; global incidence is ~50–112 per 100,000 person-years with rising emergency presentations in many regions, including Australia [1,2,4].  Mortality is low but non-trivial and is driven by airway obstruction, severe bronchospasm, or […]

Overview Adult Onset Still’s Disease (AOSD) is a complex multisystemic autoinflammatory disease characterised by recurrent episodes of spiking fever (> 39–40°C), pink–salmon transient skin rash, and the presence of arthralgia or polyarthritis. First described by Eric Bywaters in 1971 it closely resembles systemic onset juvenile idiopathic arthritis (Still’s disease). Pathophysiology AOSD is a polygenic and […]

Transient osteoporosis of the hip causes sudden, severe groin or buttock pain and reversible bone marrow edema—learn how to spot, diagnose, and manage it. In this clear, clinically focused video you’ll discover the defining features of transient osteoporosis of the hip, why it’s distinct from general osteoporosis and avascular necrosis, and how MRI identifies diffuse […]