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Childhood interstitial lung disease

Childhood interstitial lung disease (chILD) is a broad collection of diverse interstitial lung diseases (ILDs) with an age of onset ranging from 0 to 18 years1-3

Childhood interstitial lung disease

PREVALENCE OF chILD

Prevalence of chILD in Europe is estimated to be between 1.3 and 46.5 cases per million children*4–6

Prevalence of chILD by age group in Spain

SIGNS, SYMPTOMS AND COMORBIDITIES OF chILD

The severity of chILD presentation is highly variable, ranging from mild nonspecific symptoms to very severe disease7

Despite the heterogeneity within chILD, there is significant overlap in its clinical manifestations7

The most common signs and symptoms of chILD include:7–27

the most common signs and symptoms of child

Comorbidities are seen in almost half of all patients with chILD (48.1%), and 27.4% report ≥1 comorbidity,17 with the most frequent comorbidities being:15–17,20

heart disease

Heart disease 
(17.4%–41.7%)
nervous system disease

Nervous system disease 
(8.0%–13.0%)
6.6-9percent.jpg

Diseases of musculoskeletal system (6.6%–9.0%)
4.8-8percent.jpg

Skin disease (4.8%–8.0%)

Pulmonary fibrosis in chILD

The proportion of patients with chILD classified as having pulmonary fibrosis ranges from 2% to 26%27–29

  • In a UK study, pulmonary fibrosis was reported using biopsy in 2% (n=2/93) of patients with chILD aged <2 years and in 7% (n=8/118) aged 2–18 years28
  • In a French study, pulmonary fibrosis was reported using HRCT and histology in 4% (n=4/119) of patients with chILD29
  • In a European study, pulmonary fibrosis was reported mostly using lung biopsy in 26% (n=46/177) of patients with chILD27

In ILDs in general, HRCT is essential for reaching an accurate diagnosis of pulmonary fibrosis30

Disease severity and progression

Disease severity in chILD can be assessed using the Fan severity score by establishing the patient history and undertaking a physical examination, whose increasing value is associated with significantly poorer outcomes, including decreased survival.24,31 Higher Fan severity scores have also been reported following ≥1 incident(s) of acute exacerbation of ILD (AE-ILD)10

Although there is no single validated threshold for clinically significant ILD progression in chILD, the clinical course of chILD can also be assessed using the Fan severity score, as well as monitoring the child’s nutritional status by observing their calorie count and growth parameters32

MORTALITY IN chILD

chILD can be associated with significant mortality15,33

Morbidity and mortality rates vary depending on the specific chILD disorder33

Risk factors for early mortality in chILD
  • Younger age at baseline15
  • Fan severity score24,31
  • Pulmonary hypertension34
  • Almost mature / immature gestational age10
  • High baseline severity24
  • Low baseline SpO215
  • Immunocompromised status21
  • Use of mechanical ventilation21

 

Health-related quality of life (HRQoL) in chILD

HRQoL is significantly impaired in patients with chILD vs. healthy population35

child-and-parent-report.jpg

Risk factors for impaired HRQoL in chILD
  • Younger age17
  • Higher disease severity17,35
  • ≥1 incident(s) of AE-ILD10
  • Extra-pulmonary localization of disease35
  • Use of non-pharmaceutical therapies17,35
  • Lower mental functioning of parents36

 

Caregiver burden in chILD

  • Parent HRQoL is impaired, especially mental functioning, which is lower when perception of their child’s HRQoL is also low36
  • 74%–91% of parents report anxiety or depression22,37
  • 44% of parents report productivity loss at the time of initial presentation of chILD, which continues to be the case in 33% of parents after 5 years of follow-up38
Footnotes
  • *
    The period prevalence range of chILD in Europe was derived for children aged 0 to 18 years: 1.3 cases per million in Germany (2009);4 3.6 cases per million in the UK and Ireland (2002);5 and 46.5 cases per million in Spain (2022).6
  • AE-ILD, acute exacerbation of interstitial lung disease; chILD, childhood interstitial lung disease; ES, effect size; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; ILD, interstitial lung disease; PedsQL, Pediatric Quality of Life Inventory; SpO2, peripheral oxygen saturation.

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