Insights into the clinical presentation of juvenile systemic lupus erythematosus: the Paediatric Rheumatology group of the Milan area (PRAGMA) monocentric cohort of 177 patients
Authors' conclusion
These data from a large jSLE monocentric cohort allow gaining further insights into a rare disease with a still high morbidity burden.
Study context
- Population
- Patients with juvenile SLE (1997 ACR or 2012 SLICC criteria, onset before 18 years), Paediatric Rheumatology group of the Milan area (PRAGMA)155 females
- Participants
- 177 patients
- Outcome
- Clinical manifestations at onset and during follow-up
Methodological detailsmonocentric
- Monocentric
Main finding
As reported in the abstract
Disease manifestations
- Haematologic involvement (most common)
- 75%
- Joint involvement
- 70%
- Cutaneous involvement
- 57%
- Renal disease, 58 patients
- 32.8%
- Neurological complications, 26 cases
- 14.7%
- 3 clinical manifestations (most common)
- 32.8%
- 2 organ involvements, 54 patients
- 30.5%
- 4 organ involvements, 25 subjects
- 14.1%
Observational data: associations, not effects.
Further findings
Manifestations by age at onset
- Onset <10 years (n=49): less commonly articular involvement
- P = 0.02
- Age >14.8 years: less neurological manifestations
- P = 0.02
Disease progression during follow-up · median follow-up of 118 month
- Patients with disease progression
- 93
- Median new manifestations per patient
- 2
Low complement at diagnosis predicting new clinical manifestations
- C3
- P = 0.013
- C4
- P = 0.0004
SLEDAI over time
- Median SLEDAI at diagnosis
- 13
- Similar at 6 months, decreased at 12 months, stable at 18 months, further reduced at 24 months
- P < 0.0001
Original publication
Insights into the clinical presentation of juvenile systemic lupus erythematosus: the PRAGMA monocentric cohort of 177 patients. Clin Exp Rheumatol. Published 10 October 2022. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →