Pediatric rheumatology

Pediatric Sarcoidosis: Retrospective Analysis of Biopsy-Proven Patients

J RheumatolPublished Retrospective cohort study

Authors' conclusion

Multiorgan involvement, response to steroids, and chronic course are hallmarks of pediatric sarcoidosis. The phenotype significantly varies by age and race.

Study context

Population
Children with biopsies consistent with sarcoidosis, performed 2010–2020
Participants
48 children
Exposure
Disease onset before 5 years of age vs older children
Outcome
Phenotype, disease course and treatment of pediatric sarcoidosis
Methodological detailsbiopsy-proven, Patients' notes reviewed retrospectively
  • Biopsy-proven
  • Patients' notes reviewed retrospectively

Main finding

As reported in the abstract

Phenotype and treatment
Mean age at diagnosis (years)
9.5
Male to female ratio
0.71
Black race
72%
Multiorgan disease
94%
Average number of organs involved
4.8
Lymph node involvement
65%
Skin involvement
63%
Eye involvement
60%
Raised serum calcium
23%
Raised angiotensin-converting enzyme
76%
NOD2 mutations (of 14 tested)
6
Systemic steroids
81%
Conventional disease-modifying antirheumatic drugs (DMARDs)
90%
Biologic added, mostly anti-tumor necrosis factor (anti-TNF)
25%
Weaned off steroids
58%
Remained on long-term DMARDs
85%

Observational data: associations, not effects.

Further findings

OutcomeP value
Features by ageUnder 5 years vs Older children · splenomegaly more oftenP = 0.001
Features by ageUnder 5 years vs Older children · spleen involvement more oftenP = 0.003
Features by ageUnder 5 years vs Older children · higher CRPP = 0.10

Features by age

Weight loss more common in adolescents (P = 0.006)

Organ involvement in Black patients

Kidney involvement more common in Black patients (P = 0.004)
Eye involvement more common in Black patients (P = 0.005)
Liver involvement more common in Black patients (P = 0.03)

Predictors of treatment outcome (regression)

No single factor associated with positive treatment outcomes

Original publication

Pediatric Sarcoidosis: Retrospective Analysis of Biopsy-Proven Patients. J Rheumatol. Published 15 August 2022. doi:10.3899/jrheum.220164

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