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 |
3584 Outlook Avenue, Cincinnati OH, 45208