Background: SITRAME (systemic inflammatory troncular recurrent acute macular eruption) syndrome is a newly identified systemic autoinflammatory disease (USAID) described in 2023. It is characterized by recurrent erythematomacular eruptions on the trunk, with systemic inflammation evidenced by elevated C-reactive protein (CRP) during flares. The initial report described 16 patients, and diagnostic criteria were published in 2024.

Objectives: The aim of this study is to describe the characteristics of the largest cohort of patients with SITRAME syndrome to date.

Methods: The cohort included patients followed up at the French National Reference Center for Adult Autoinflammatory Diseases, all fulfilling the diagnostic criteria for SITRAME syndrome.

Results: A total of 46 patients were reported with a median SITRAME score of 7; all adults of Caucasian descent, with 27 women (58.7%). The median age at inclusion was 56.3 years, ranging from 27 to 77 years. The median age at symptom onset was 34 years, and at diagnosis, it was 53.5 years, with a median diagnostic delay of 19.9 years. Flares of SITRAME syndrome lasted a median of 3 days, with annual recurrences ranging from 1 to 20 times. During flares, CRP levels were elevated to a median of 26.5 mg/L, normalizing in between. All patients met the major diagnostic criteria, and the most frequent minor criteria included triggering factors like infections, vaccinations, and intense physical activity (73.9%). During flares, all patients displayed an erythematous rash affecting the trunk, which was macular in 93.5% and pruritic in 10.9%. The rash extended beyond the trunk, reaching the arms in 26%, the thighs roots in20%, the legs in 11% and face in 9%. The rash covered between 10% and 50% of the body surface in 67.4% of patients. Symptoms associated with the rash included fever (50%), asthenia (47.8%), arthromyalgia (28.3%), headache (17.4%), and abdominal pain (13%). The most common triggers were ENT infections (63%), vaccinations (26%), physical activity (10.9%), and medications (17.4%). Regarding severity, 39.1% of patients had more than 50% of their body surface affected by the rash. Severe asthenia, affecting daily activities, was observed in 28.3% of cases. 8.7% of patients experienced at least one flare per month. Histological analysis of skin biopsies from 16 patients revealed no vasculitis or isolated neutrophilic infiltrates, but a mixed neutrophilic and eosinophilic infiltrate in 6 cases, and an eosinophilic infiltrate in 3 cases. Genetic analysis of 15 patients showed inconclusive results. Therapeutic interventions included colchicine for 19 patients (43.2%) at 1 mg/d: for 7 patients it decreased the intensity and length of flares; corticosteroids, which were ineffective in 3 patients; one patient received anakinra during a crisis, which was able to improve the symptoms; anti-IgE treatment (300 mg/month) were ineffective for two patients. Seven patients were followed for at least 12 months, and two for 24 months. No serious complications, such as inflammatory amyloidosis or death, were observed.

Conclusion: SITRAME syndrome is an individualized entity within USAID, affecting adults sporadically. This study confirms the original description suggesting that the syndrome is more common than previously thought, with already 46 French patients affected. Despite the frequent recurrence of attacks associated with asthenia and elevated CRP, no serious inflammatory complications are reported so far. Further research is needed to understand the pathophysiology, improve therapeutic management and prevention.

REFERENCES: [1] Soria et al, J Eur Acad Dermatol Venereol . 2023 Apr;37(4):e538-e542.

[2] Soria et al, J Eur Acad Dermatol Venereol . 2025 Jan;39(1):e87-e90.

Acknowledgements: NIL.

Disclosure of Interests: Angèle Soria: None declared, Cristina Lavilla: None declared, Emmanuelle Amsler: None declared, Yixiang Yves-Jean Zhu: None declared, Hassina Aloui: None declared, Mathieu Paul Rodero: None declared, Guilaine Boursier: None declared, Sophie Georgin-Lavialle SOBI and Novartis.

© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.