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Soraya Ayoub & Salim Larbani. Tous droits réservés.
LEARN AND EXPLORE
Choose an area to find its topics and related resources.
Compare hereditary recurrent fevers, understand Still disease and recognise VEXAS: diagnosis, complications, treatment and monitoring.
LEARNING PATHWAYS
5 topics · 40 added course sections · 5 teaching diagrams
Compare attack duration, organ involvement and the meaning of genetic results.
Build the diagnosis of Still disease and recognise macrophage activation syndrome.
Connect inflammation, cytopenias and somatic UBA1 testing.
Expandable chapters, comparison tables, organ monitoring and references attached to the relevant sections.
MICA · CLINICAL LEARNING
For unexplained recurrent or persistent inflammation, assess the reproducible clinical pattern and its complications. Exclude common causes, document episodes and obtain appropriate expertise before concluding that an autoinflammatory disease is present.
Record age at onset, episode duration and frequency, return to baseline, triggers and family history. Connect documented fever with skin features, joint pain, serosal involvement or digestive symptoms. A diary with contemporaneous records is more useful than late reconstruction. Familial Mediterranean fever, Cryopyrinopathies and TRAPS have distinct patterns; grouping them does not mean they share identical criteria.
[1][2]Consider infection, malignancy, autoimmune disease and drug causes according to context and presentation. New prolonged fever in an adult should not automatically be labelled hereditary. Organ failure, neurological change or rapidly worsening laboratory findings requires urgent assessment. In Still’s disease, systemic deterioration particularly warrants assessment for macrophage activation syndrome without waiting for a complete presentation.
[3][1]Document inflammation during an attack and during a clinically quiet interval. Assess accumulated damage, including kidney involvement, and treatment effects according to the disease. Symptom resolution does not always mean inflammation has stopped. Inflammatory amyloidosis is an important complication of some diseases: this warrants organised monitoring, but risk cannot be inferred from a single febrile episode.
[1][2]Discuss genetic testing with an experienced team based on phenotype rather than treating every variant as causal. Treatment response can inform monitoring without proving the diagnosis by itself. Goals include episode control, residual inflammation, damage prevention and quality of life. Review adherence, tolerability and understanding of the escalation plan with the patient.
[1][2][3]FICTIONAL SITUATION · WITHOUT A SCORE
Fictional situation: a patient reports longstanding febrile attacks but now presents with continuous fever and general deterioration.
Does the usual pattern justify waiting for this episode to end?
No. The altered pattern requires fresh clinical and laboratory assessment for infection and systemic complications. Comparison with documented attacks helps identify the change but cannot exclude an emergency. Specialist assessment should be coordinated with necessary immediate care rather than delaying it.
Original educational synthesis for adult internal medicine. Individual care depends on the clinical setting, applicable product information and local pathways. These notes are not an institutional protocol or a certification. Independent human scientific review remains to be confirmed.
