The Systemic JIA Foundation tracks clinical trials relevant for SJIA, AOSD, and MAS. The data is gathered from ClinicalTrials.gov & European Trial Registry and includes trials from USA, Europe, Asia and South America. This page was last updated in September 2023. Please email us (contact@systemicjia.org) to report any updates.
The tables below include:
- Trials not just for SJIA / AOSD but also for related diseases such as HLH or autoinflammatory diseases which share therapeutic approaches.
- Criteria trial used to define SJIA (can patients with systemic only symptoms be included or is arthritis required).
- A summary of the main types of drugs covered in the tables.
- IL1 & IL6 inhibitors: There are current trials for canakinumab in Germany & Japan. Additionally, there are trials for sarilumab, an IL6 biosimilar in Europe, United Kingdom, South America and Asia.
- IL18 inhibitors: There are three IL18 inhibitors coming up through the pipeline.
- Tadekinig Alfa from AB2 Bio continues to be in trial for NLRC4 & XIAP deficiency. (Note: SJIA Foundation is aware of a SJIA patient who earlier received the drug through compassionate use.)
- MAS-825 from Novartis is a bi-specific antibody against IL1 & IL18. The current trials for MAS-825 are in NLRC4 & XIAP. (Note: Foundation is aware of a few patients with SJIA, MAS & Lung Disease who have received the drug via compassionate use).
- Interferon gamma inhibitor: Emapalumab is an interferon-gamma (IFNy) blocking antibody from Sobi that is currently approved for HLH in the USA, and is in clinical trials for MAS in the USA and Europe. (Note: The SJIA Foundation is aware of several patients with SJIA, MAS & Lung Disease who have received emapalumab in life-threatening situations, outside clinical trials).
- Jak-inhibitors: Several jak-inhibitors are in trials for SJIA including tofacitinib, baricitinib and upadacitinib. Additionally, ruxolitinib and itacitinib are in trials for HLH.
- NLRP3 inflammasome inhibitors: A new class of drugs, NLRP3 Inflammasome inhibitors, which block both IL1 & IL18 pathways are in trials for autoinflammatory diseases such as CAPS, FCAS etc.
Table 1. Current Clinical Trials AOSD / SJIA
Drug | Type | Indication | Patient Group targeted | Trial Number | Status / Phase | Sponsors | Last Update | Locations |
---|---|---|---|---|---|---|---|---|
Baracitinib | Jak-Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | 2017-004495-60 | Recruiting | Eli Lilly & Company | Austria, Belgium, Czechia, Denmark... For full countries list, see link. | |
Baracitinib | Jak-Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | NCT04088396 | Recruiting / Phase 3 | Eli Lilly & Company | 9/13/2023 | India, Israel, Japan, Austria... For full countries list, see link. |
Tofacitinib | Jak-Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | NCT03000439 | Recruiting / Phase 3 | Pfizer | 9/5/2023 | United States, Argentina, Belgium, Brazil... For full countries list, see link. |
Tofacitinib | Jak-Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | 2017-002018-29 | Recruiting | Pfizer | Belgium, Germany, Hungary, Italy, Netherlands, Poland, Slovakia | |
Upadacitinib | Jak-Inhibitor | JIA / SJIA | SJIA with active arthritis | NCT03725007 | Recruiting / Phase 1 | AbbVie | 9/21/2023 | United States, Canada, Germany, Hungary... For full countries list, see link. |
Upadacitinib & Tocilizumab | Jak-Inhibitor +IL6 Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | NCT05609630 | Not yet recruiting / Phase 3 | AbbVie | 9/22/2023 | |
Canakinumab | IL1 Inhibitor | SJIA | SIJA - ILAR criteria (arthritis required) | 2018-004284-30 | Recruiting | Asklepios Klink Sankt Augustin | Germany | |
Canakinumab | IL1 Inhibitor | AOSD | SIJA - ILAR criteria (arthritis required) | NCT04717635 | Active, not recruiting / Phase 3 | Novartis Pharmaceuticals | 9/14/2023 | Japan |
Sarilumab | IL6 Inhibitor (Biosimilar) | SJIA | SIJA - ILAR criteria (arthritis required) | NCT02991469 | Recruiting / Phase 2 | Sanofi | 9/22/2023 | Argentina, Bulgaria, Canada, Czechia... For full countries list, see link. |
The second table summarizes current trials in MAS, Primary & Secondary HLH. Historically drugs first tested in HLH have been later used in MAS. As such, the foundation follows trials in Primary & Secondary HLH as well.
Table 2. Current Clinical Trials in MAS / Primary & Secondary HLH
Drug | Type | Indication | Patient Group targeted | Trial Number | Status / Phase | Sponsors | Last Update | Locations |
---|---|---|---|---|---|---|---|---|
MAS-825 | IL1 + IL18 Inhibitor | NLRC4-GOF | NLRC4 mutation | NCT04641442 | Recruiting / Phase 2 | Novartis Pharmaceuticals | 7/13/2023 | Canada, United States, Japan, Turkey, ... For full countries' list, see link. |
Tadekinig alfa (IL-18BP) | IL18 Inhibitor | NLRC4 / XIAP deficiency | Genetic diagnosis of NLRC4 mutation or XIAP deficiency | NCT03113760 | Active, not recruiting / Phase 3 | AB2 Bio | 7/21/2023 | United States, Canada, Germany |
Emapalumab | IFNY inhibitor | AOSD / SJIA / MAS | SJIA / AOSD Diagnosis / MAS criteria described in trial | 2021-001577-24 | Recruiting | Sobi AG | Belgium, Czechia, France, Germany, ... For full countries' list, see link. | |
Emapalumab | IFNY inhibitor | AOSD/ SJIA / MAS / SLE | SJIA / AOSD Diagnosis / MAS criteria described in trial | NCT05001737 | Recruiting / Phase 3 | Sobi AG | 9/13/2023 | Canada, United States, China, Japan, ... For full countries' list, see link. |
Itacitinib | Jak-Inhibitor | HLH | NCT05063110 | Recruiting / Phase 2 | Hôpitaux de Paris / Incyte Corporation | 8/23/2022 | France, Bobigny | |
Ruxolitinib, Dexamethasone, Etoposide | Jak-Inhibitor / Corticosteroid / Chemotherapy Drug | HLH | Active HLH (diagnosis criteria described in trial) | NCT04551131 | Recruiting / Phase 1 (ruxo), Phase 2 (dexa) | St. Jude Children's Research Hospital | 9/25/2023 | United States |
ELA026 | Anti-SIRP monoclonal antibody | HLH | HLH (criteria HLH-2004) | NCT05416307 | Recruiting / Phase 1 | Electra Therapeutics | 3/20/2023 | Austria, Italy, Netherlands, Spain, United Kingdom |
Gemtuzumab Ozogamicin | Monoclonal anti-CD33 antibody | HLH / MAS | HLH or MAS (primary, secondary or refractory) | 2020-002428-36 | Recruiting / Phase 2 | University of Birmingham | United Kingdom |
The third table summarizes trials for Inflammasome inhibitors currently being tested in different Autoinflammatory Diseases such as CAPS, FCAS.
Table 3. Current Clinical Trials for Inflammasome Inhibitors in Autoinflammatory Disease
Drug | Type | Indication | Trial Number | Status / Phase | Sponsors | Last Update | Locations |
---|---|---|---|---|---|---|---|
ZYIL1 | Inflammasome Inhibitor | CAPS | NCT05186051 | Completed / Phase 2 | Zydus Lifesciences Limited | 07/11/2022 | Australia |
ATI-450 | Inflammasome Inhibitor | Sepsis | NCT04635878 | Recruiting | University Hospital, Toulouse | 06/09/2022 | France |
ATI-450 | Inflammasome Inhibitor | CAPS | NCT05292768 | Not yet recruiting | Hôpitaux de Paris,Institut Imagine | 03/23/2022 | France |
DFV890 | Inflammasome Inhibitor | FCAS | NCT04868968 | Completed / Phase 2 | Novartis Pharmaceuticals | 5/23/2023 | United States, France, Germany, Italy |
Depansutrile | Inflammasome Inhibitor | Schnitzler Syndrome | NCT03595371 | Unknown / Phase 2 | Olatec Therapeutics LLC / Radboud University Medical Center | 07/06/2021 | Netherlands |