This reading list is a starting point for clinicians and readers who want to explore the medical literature on autoinflammatory diseases. For a plain-language introduction, visit The Basics. Families can also find support organizations on the Resources page.
Selected guidance and research
CAPS, TRAPS, MKD and DIRA
EULAR/ACR guidance on IL-1-mediated autoinflammatory diseases (2021 points to consider, published 2022). Guidance for clinicians on diagnosis, management and monitoring of cryopyrin-associated periodic syndromes (CAPS), TNF receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), and deficiency of the IL-1 receptor antagonist (DIRA). The full text is available through PubMed Central.
Familial Mediterranean fever
EULAR/PReS endorsed recommendations for the management of familial Mediterranean fever (FMF): 2024 update. Recommendations for health professionals covering treatment and follow-up. The publisher may require access for the full article; the recommendation is also listed in the EULAR management recommendations directory.
Autoinflammatory interferonopathies
EULAR/ACR guidance on CANDLE/PRAAS, SAVI and Aicardi-Goutières syndrome (2021 points to consider, published 2022). A specialist reference on diagnosis and management of these rare type I interferonopathies. The full text is available through PubMed Central.
Genetic testing and interpretation
ISSAID/EMQN best practice guidelines for the genetic diagnosis of monogenic autoinflammatory diseases (2020). Guidance on testing strategies, variant interpretation, and additional investigations such as testing for mosaicism.
PFAPA
CARRA consensus treatment plans for PFAPA (2020). A framework for comparing treatment approaches and responses; these consensus plans do not establish one preferred treatment for every patient.
Disease activity and the AIDAI
- Piram and colleagues: AIDAI validation for hereditary recurrent fever syndromes (2014). The foundational validation study.
- Koné-Paut and colleagues: AIDAI monitoring in patients treated with canakinumab (2022). Disease-activity monitoring in colchicine-resistant FMF, MKD, and TRAPS.
For the printable diary and patient instructions, see Fever and Symptom Diary: Using the AIDAI.
Using this reading list
These publications are written primarily for health professionals and are not a complete review of every autoinflammatory condition. Recommendations evolve. Families can discuss questions raised by an article with their care team; an individual research paper cannot determine a diagnosis or treatment plan.
Reading list updated September 29, 2026.