6 Months and older, any sex, with Nakajo-Nishimura Syndrome or Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature Syndrome. Patients with the condition only — healthy volunteers not accepted.
Results — posted to ClinicalTrials.gov
Per-arm endpoint measurements with 95% confidence intervals where reported. Source: trial results section.
Change From Baseline in Mean Daily Diary Scores in Participants With CANDLE (Primary Treatment Period)Primary· Baseline, 20 weeks
Diaries were specific to individual indications or conditions (i.e., NNS/CANDLE, SAVI, or AGS). For NNS/CANDLE, participant or caregiver was instructed to rate each symptom (fever, rash, musculoskeletal pain, headache and fatigue in the diary on a scale from 0 to 4 (where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms, 3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\]. The mean daily score range was 0-4 with the higher score indicating a more severe symptom. Total score was not utilized.
Group
Value
95% CI
CANDLE
-0.217
± 0.586
Change From Baseline in Mean Daily Diary Scores in Participants With SAVI (Primary Treatment Period)Primary· Baseline, 32 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For SAVI, participant or caregiver was instructed to rate each symptom (fever, rash, musculoskeletal pain, fatigue, respiratory/breathing problems, and ulcers/ischemic lesions in the diary on a scale from 0 to 4 (where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms,3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\].The mean daily score range was 0-4 with the higher score indicating a more severe symptom. Total score was not utilized.
Group
Value
95% CI
SAVI
-0.23
± 0.238
Change From Baseline in Mean Daily Diary Scores in Participants With AGS (Primary Treatment Period)Primary· Baseline, 32 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For AGS, participant or caregiver was instructed to rate each symptom (rating) (neurologic disability (0, 5, 7,10) crying (0, 1, 2, 3), length of uninterrupted sleep (0, 1, 2, 3), generalized seizure (0, 8), fever (0,1), excessive irritability (0, 1, 2, 3), skin findings(body) (0, 1, 2, 3), and skin findings (hands, feet, and ears) (0, 1, 2, 3) with a higher score for each symptom indicating a more severe symptom. The mean daily diary score was the average of all symptom scores and the range was 0 -
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
-0.045
± 0.164
Change From Baseline in Mean Daily Diary Scores in Participants With CANDLE (Primary Treatment and Maintenance Period)Secondary· Baseline, 191.1 weeks
Diaries were specific to individual indications or conditions (i.e., NNS/CANDLE, SAVI, or AGS). For NNS/CANDLE, participant or caregiver was instructed to rate each symptom (fever, rash, musculoskeletal pain, headache and fatigue in the diary on a scale from 0 to 4 (where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms, 3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\]. The mean daily score range was 0-4 with the higher score indicating a more severe symptom. Total score was not utilized.
Group
Value
95% CI
CANDLE
-0.24
± 0.613
Change From Baseline in Mean Daily Diary Scores in Participants With SAVI (Primary Treatment and Maintenance Period)Secondary· Baseline, 202.9 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For SAVI, participant or caregiver was instructed to rate each symptom (fever, rash, musculoskeletal pain, fatigue, respiratory/breathing problems, and ulcers/ischemic lesions in the diary on a scale from 0 to 4 (where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms,3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\].The mean daily score range was 0-4 with the higher score indicating a more severe symptom. Total score was not utilized.
Group
Value
95% CI
SAVI
-0.286
± 0.333
Change From Baseline in Mean Daily Diary Scores in Participants With AGS (Primary Treatment and Maintenance Period)Secondary· Baseline, 206.1 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For AGS, participant or caregiver was instructed to rate each symptom (rating) (neurologic disability (0, 5, 7,10) crying (0, 1, 2, 3), length of uninterrupted sleep (0, 1, 2, 3), generalized seizure (0, 8), fever (0,1), excessive irritability (0, 1, 2, 3), skin findings(body) (0, 1, 2, 3), and skin findings (hands, feet, and ears) (0, 1, 2, 3) with a higher score for each symptom indicating a more severe symptom. The mean daily diary score was the average of all symptom scores and the range was 0 -
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
-0.08
± 0.114
Number of Participants With Decrease in Daily Dose of Corticosteroids in Participants With CANDLE, SAVI and AGS (Primary Treatment Period)Secondary· CANDLE: Week 20, SAVI and AGS: Week 32
Decrease was defined as total steroid dose at the visit \<0.15 mg/kg/day (prednisone-equivalent) or \>=50% decrease from baseline.
Group
Value
95% CI
CANDLE
4
SAVI
1
Aicardi-Goutières Syndrome (AGS)
1
Number of Participants With Decrease in Daily Dose of Corticosteroids in Participants With CANDLE, SAVI and AGS (Primary Treatment and Maintenance Period)Secondary· CANDLE: Week 191.1; SAVI: 202.9 and AGS: Week 206.1
Decrease was defined as total steroid dose \<0.15 mg/kg/day (prednisone-equivalent) or \>=50% decrease from baseline.
Group
Value
95% CI
CANDLE
3
SAVI
1
Aicardi-Goutières Syndrome (AGS)
1
Change From Baseline in Patient's Symptom Specific Daily Diary Scores For Participants With CANDLE (Primary Treatment Period)Secondary· Baseline, 20 weeks
Diaries were specific to individual indications or conditions (i.e, NNS/CANDLE,SAVI, or AGS). For NNS/CANDLE, participant or caregiver was instructed to rate each symptom (fatigue, fever, headache, musculoskeletal pain and rash in the diary on a scale from 0 to 4, where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms, 3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\]). The mean daily score range was 0-4 with the higher score indicating a more severe symptom.
Fatigue
Group
Value
95% CI
CANDLE
-0.029
± 0.997
Fever
Group
Value
95% CI
CANDLE
0
± 0
Headache
Group
Value
95% CI
CANDLE
0.057
± 0.128
Musculo-skeletal Pain
Group
Value
95% CI
CANDLE
-0.514
± 1.128
Rash
Group
Value
95% CI
CANDLE
-0.6
± 0.894
Change From Baseline in Patient's Symptom Specific Daily Diary Scores For Participants With SAVI (Primary Treatment Period)Secondary· Baseline, 32 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For SAVI, participant or caregiver was instructed to rate each symptom (fatigue, fever, musculoskeletal pain, rash, respiratory/breathing problems, and ulcers/ischemic lesions in the diary on a scale from 0 to 4, where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms, 3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\]). The mean daily score range was 0-4 with the higher score indicating a more severe symptom.
Fatigue
Group
Value
95% CI
SAVI
-0.381
± 0.541
Fever
Group
Value
95% CI
SAVI
-0.095
± 0.165
Musculo-skeletal Pain
Group
Value
95% CI
SAVI
-0.238
± 0.412
Rash
Group
Value
95% CI
SAVI
-0.333
± 0.577
Respiratory / Breathing Symptoms
Group
Value
95% CI
SAVI
-0.333
± 0.577
Ulcers / Ischemic Lesions
Group
Value
95% CI
SAVI
0
± 0
Change From Baseline in Patient's Symptom Specific Daily Diary Scores For Participants With AGS (Primary Treatment Period)Secondary· Baseline, 32 weeks
Diaries were specific to individual indications or conditions (i.e. NNS/CANDLE, SAVI, or AGS). For AGS, participant or caregiver was instructed to rate each symptom (rating) (crying (0, 1, 2, 3), excessive irritability (0, 1, 2, 3), fever (0,1), generalized seizure (0, 8), length of uninterrupted sleep (0, 1, 2, 3), neurologic disability (0, 5, 7,10), skin findings(body) (0, 1, 2, 3), and skin findings (hands, feet, and ears) (0, 1, 2, 3) with a higher score for each symptom indicating a more severe symptom.
Crying
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
0
± 0
Excessive Irritability
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
0
± 0
Fever
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
-0.071
± 0.101
Generalized Seizure
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
0
± 0
Length of Uninterrupted Sleep
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
0.286
± 0.404
Neurologic Disability
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
0
± 0
Skin Findings (Body)
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
-0.5
± 0.707
Skin Findings (Hands, Feet, Ears)
Group
Value
95% CI
Aicardi-Goutières Syndrome (AGS)
-0.071
± 0.101
Change From Baseline in Patient's Symptom Specific Daily Diary Scores For Participants With CANDLE (Primary Treatment and Maintenance Period)Secondary· Baseline, 191.1 weeks
Diaries were specific to individual indications or conditions (i.e, NNS/CANDLE,SAVI, or AGS). For NNS/CANDLE, participant or caregiver was instructed to rate each symptom (fatigue, fever, headache, musculoskeletal pain and rash in the diary on a scale from 0 to 4, where a score of 0 = no symptoms, 1 = mild symptoms, 2 = moderate symptoms, 3 = more severe symptoms, and 4 = severe symptoms \[equivalent to "worst" symptoms\]). The mean daily score range was 0-4 with the higher score indicating a more severe symptom.
Fatigue
Group
Value
95% CI
CANDLE
-0.057
± 1.172
Fever
Group
Value
95% CI
CANDLE
0
± 0
Headache
Group
Value
95% CI
CANDLE
0.057
± 0.128
Musculo-skeletal Pain
Group
Value
95% CI
CANDLE
-0.514
± 1.128
Rash
Group
Value
95% CI
CANDLE
-0.686
± 0.842
Adverse events — posted to ClinicalTrials.gov
Time frame: CANDLE: Baseline Up to 191.1 Weeks; SAVI: Baseline Up to 202.9 Weeks and AGS: Baseline Up to 206.1 Weeks.
Reporting threshold: 0%.
Adverse-event reports describe events observed during the trial — not all are caused by the drug.
The main purpose of this study is to evaluate the efficacy and safety of baricitinib in adult and pediatric Japanese participants with Nakajo-Nishimura Syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (NNS/CANDLE), STING-associated vasculopathy with onset during infancy (SAVI), and Aicardi-Goutières Syndrome (AGS).
Publications & conference data
8 peer-reviewed publications reference this trial (live from Europe PMC):
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Publications: Europe PMC API search by NCT ID, retrieved 10 June 2026
Drug + disease cross-links: matched in real time against Drug Landscape's normalised drug + company + condition tables
Sponsor: as reported to ClinicalTrials.gov by Eli Lilly and Company
Last refreshed: 10 July 2025
Drug Landscape aggregates and links these public records for informational use only. Always verify against the primary source before clinical or regulatory decisions. Canonical URL: https://druglandscape.com/trial/NCT04517253.