Development and Implementation of the AIDA International Registry for Patients With Undifferentiated Systemic AutoInflammatory Diseases

Casa, Francesca Della; Vitale, A.; Lopalco, Giuseppe; Ruscitti, Piero; Ciccia, Francesco; Emmi, Giacomo; Cattalini, Marco; Więsik-Szewczyk, Ewa; Maggio, Maria Cristina; Ogunjimi, Benson; Sfikakis, Petros P.; Tufan, Abdurrahman; Al‐Mayouf, Sulaiman M.; Giudice, Emanuela Del; Aragona, Emma; Torre, Francesco La; Sota, Jurgen; Colella, Sergio; Cola, Ilenia Di; Iacono, Daniela; Mattioli, Irene; Jahnz-Różyk, Karina; Joos, Rik; Laskari, Katerina; Gaggiano, Carla; Abbruzzese, Anna; Cipriani, Paola; Rozza, Gelsomina; Alsaleem, Alhanouf; Yıldırım, Derya; Tarsia, Maria; Ragab, Gaafar; Ricci, Francesca; Cardinale, Fabio; Korzeniowska, Marcelina; Frassi, Micol; Caggiano, Valeria; Saad, Moustafa Ali; Pereira, Rosa Maria Rodrigues; Berlengiero, Virginia; Gentileschi, Stefano; Guerriero, Silvana; Giani, Teresa; Gelardi, Viviana; Iannone, Florenzo; Giardini, Henrique Ayres Mayrink; Almaghlouth, Ibrahim; Kardaş, Rıza Can; Ait-Idir, Djouher; Frediani, Bruno; Balistreri, Alberto; Fabiani, Claudia; Rigante, Donato; Cantarini, Luca

doi:10.3389/fmed.2022.908501

Cited by 6 publications

(5 citation statements)

References 22 publications

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“…This makes further efforts necessary to better understand the nature and the correct treatment of unexplained periodic fever episodes. In this context, the AutoInflammatory Disease Alliance (AIDA) project has recently supported the development of an international registry dedicated to undifferentiated autoinflammatory conditions, paving the way to suitable clinical research possibly leading to the identification of new clinical entities, among all patients with unexplained recurrent fever episodes ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

“…Despite these preliminary clues, it is not clear whether recurrent fever episodes should be considered as an expression of extra-articular inflammatory involvement or whether the axial SpA could have been part of a larger pathological picture. Based on a relatively wide cohort of patients from the AIDA Network USAID registry, we have described the clinical, laboratory and therapeutic features of these patients ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

“…The patients were consecutively identified and enrolled from April 2021 to November 2022. Data were collected in the International AIDA (AutoInflammatory Disease Alliance) network registry for Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) ( 16 ). This registry represents an observational study collecting data obtained from patients managed and treated according to the best standard of care, based on patients’ history, and tailored on clinical and laboratory manifestations.…”

Section: Methodsmentioning

confidence: 99%

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Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)

et al. 2023

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BeckgroundDespite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment.Materials and methodsPatients’ data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network.ResultsA total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 ± 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42°C, with a mean temperature of 38.8 ± 1.1°C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARDs were more useful when combined with biotechnological agents.ConclusionSigns and symptoms referring to axial SpA should be inquired in patients with apparently unexplained recurrent fever episodes. The specific treatment for axial SpA may lead to a remarkable improvement in the severity and/or frequency of fever episodes in patients with unexplained fevers and concomitant axial SpA.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)

et al. 2023

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“…Creating a worldwide network of experts (international registries) who can share their experiences will potentially be useful for overcoming some of the unmet needs of patients with AIDs ( 100 ).…”

Section: Discussionmentioning

confidence: 99%

Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study

Alexeeva,

Shingarova,

Dvoryakovskaya

et al. 2023

Front. Med.

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IntroductionThe blockade of interleukine-1 (anakinra and canakinumab) is a well-known highly effective tool for monogenic autoinflammatory diseases (AIDs), such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinaemia D syndrome, and cryopyrin-associated periodic syndrome, but this treatment has not been assessed for patients with undifferentiated AIDs (uAIDs). Our study aimed to assess the safety and efficacy of canakinumab for patients with uAIDs.MethodsInformation on 32 patients with uAIDs was retrospectively collected and analyzed. Next-generation sequencing and Federici criteria were used for the exclusion of the known monogenic AID.ResultsThe median age of the first episode was 2.5 years (IQR: 1.3; 5.5), that of the disease diagnosis was 5.7 years (IQR: 2.5;12.7), and that of diagnostic delay was 1.1 years (IQR: 0.4; 6.1). Patients had variations in the following genes: IL10, NLRP12, STAT2, C8B, LPIN2, NLRC4, PSMB8, PRF1, CARD14, IFIH1, LYST, NFAT5, PLCG2, COPA, IL23R, STXBP2, IL36RN, JAK1, DDX58, LACC1, LRBA, TNFRSF11A, PTHR1, STAT4, TNFRSF1B, TNFAIP3, TREX1, and SLC7A7. The main clinical features were fever (100%), rash (91%; maculopapular predominantly), joint involvement (72%), splenomegaly (66%), hepatomegaly (59%), lymphadenopathy (50%), myalgia (28%), heart involvement (31%), intestinal involvement (19%); eye involvement (9%), pleuritis (16%), ascites (6%), deafness, hydrocephalia (3%), and failure to thrive (25%). Initial treatment before canakinumab consisted of non-biologic therapies: non-steroidal anti-inflammatory drugs (NSAID) (91%), corticosteroids (88%), methotrexate (38%), intravenous immunoglobulin (IVIG) (34%), cyclosporine A (25%), colchicine (6%) cyclophosphamide (6%), sulfasalazine (3%), mycophenolate mofetil (3%), hydroxychloroquine (3%), and biologic drugs: tocilizumab (62%), sarilumab, etanercept, adalimumab, rituximab, and infliximab (all 3%). Canakinumab induced complete remission in 27 patients (84%) and partial remission in one patient (3%). Two patients (6%) were primary non-responders, and two patients (6%) further developed secondary inefficacy. All patients with partial efficacy or inefficacy were switched to tocilizumab (n = 4) and sarilumab (n = 1). The total duration of canakinumab treatment was 3.6 (0.1; 8.7) years. During the study, there were no reported Serious Adverse Events (SAEs). The patients experienced non-frequent mild respiratory infections at a rate that is similar as before canakinumab is administered. Additionally, one patient developed leucopenia, but it was not necessary to stop canakinumab for this patient.ConclusionThe treatment of patients with uAIDs using canakinumab was safe and effective. Further randomized clinical trials are required to confirm the efficacy and safety.

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“…Finally, there is a group of diseases, named unclassified AIDs, that do not fit in any of the clinically defined systemic autoinflammatory conditions and do not have any known pathogenetic mutations in genes ( 25 ). For this reason, the treatment of these diseases is based on expert opinion and similarities with other diseases ( 49 ).…”

Section: Use Of Canakinumab In Specific Diseasesmentioning

confidence: 99%

Off-label use of canakinumab in pediatric rheumatology and rare diseases

Giudice

Sota²,

Orlando³

et al. 2022

Front. Med.

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Since the first success of interleukin-1 blockade in cryopyrin-associated periodic syndrome, the use of interleukin-1 inhibitors has expanded to other disorders, including off-label indications. In particular, canakinumab has been employed in an off-label fashion in several diseases such as rare monogenic autoinflammatory diseases and multifactorial autoinflammatory diseases, disclosing an excellent efficacy and good safety profile in pediatric patients unresponsive to standards of care. In addition, hyperferritinemic syndromes and complex disorders, as well as Kawasaki disease, uveitis, and other pediatric rare disorders, represent additional areas where canakinumab efficacy is worth exploring. Altogether, the results summarized below are of paramount importance in pediatric patients where a considerable proportion of treatments are prescribed off-label. This review focuses on the off-label use of canakinumab in pediatric patients affected by systemic immune-mediated diseases.

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Development and Implementation of the AIDA International Registry for Patients With Undifferentiated Systemic AutoInflammatory Diseases

Cited by 6 publications

References 22 publications

Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)

Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)

Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study

Off-label use of canakinumab in pediatric rheumatology and rare diseases

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