TY - JOUR
T1 - Under detection of interstitial lung disease in Juvenile Systemic Sclerosis (JSSC) utilizing pulmonary function tests. Results from the juvenile scleroderma inception cohort
AU - Foeldvari, Ivan
AU - Hinrichs, Bernd
AU - Torok, Kathryn S.
AU - Santos, Maria Jose
AU - Kasapcopur, Ozgur
AU - Adrovic, Amra
AU - Stanevicha, Valda
AU - Sztajnbok, Flavio
AU - Terreri, Maria Teresa
AU - Sakamoto, A. P.
AU - Alexeeva, Ekaterina
AU - Katsikas, M.
AU - Anton, Jordi
AU - Smith, Vanessa
AU - Avcin, Tadej
AU - Cimaz, Rolando
AU - Kostik, Mikhail
AU - Lehman, Thomas
AU - Sifuentes‐Giraldo, Walter‐Alberto
AU - Appenzeller, Simone
AU - Janarthanan, Mahesh
AU - Moll, M.
AU - Nemcova, D.
AU - Schonenberg‐Meinema, Dieneke
AU - Battagliotti, Cristina
AU - Berntson, Lillemor
AU - Bica, Blanca E.R.G.
AU - Brunner, Jurgen
AU - Reis, P. Costa
AU - Eleftheriou, Despina
AU - Harel, L.
AU - Horneff, Gerd
AU - Kallinich, Tilmann
AU - Lazarevic, D.
AU - Minden, Kirsten
AU - Nielsen, Susan
AU - Nuruzzaman, Farzana
AU - Patwardhan, Anjali
AU - Uziel, Yosef
AU - Helmus, Nicola
N1 - Conference code: 26
PY - 2020
Y1 - 2020
N2 - Introduction: Juvenile systemic sclerosis (jSSc) has a prevalence in
around 3 in a million children. Pulmonary involvement occurs in
approximately 40 % in the international juvenile systemic
scleroderma cohort (JSScC). Traditionally in jSSc, pulmonary function
testing (PFT) with FVC and DLCO are used for screening and
computed tomography (HRCT) was more reserved for those with
abnormal PFTs. More recently, it has become apparent that PFTs
might not be sensitive enough for detecting interstitial lung disease
(ILD) in children.
Objectives: To assess the sensitivity and specificity of FVC and DLCO
assessment to detect ILD
Methods: The international juvenile systemic scleroderma cohort
(JSScC) database was queried for available patients with recorded
PFT parameters and HRCT performed to determine sensitivity of PFTs
detecting disease process.
Results: Of 129 patients in the jSScC, 67 patients had both CT
imaging and an FVC reading from PFTs for direct comparison. DLCO
readings were also captured but not in as many patients with
tandem HRCT (n =55 DCLO and HRCT scan). Therefore, initial
analyses focused on the sensitivity, specificity and accuracy of the
FVC value from the PFTs to capture the diagnosis of interstitial lung
disease as determined by HRCT.
Overall, 49% of the patients had ILD determined by HRCT, with 60%
of patients having normal FVC (>80%) with positive HRCT findings,
and 24% of patients having normal DLCO (> 80%) with positive
HRCT findings. Fourteen percent (n = 3/21) of patients with both FVC
and DLCO values within the normal range had a positive HRCT
finding.
Conclusion: The sensitivity of the FVC in the JSScC cohort in
detecting ILD was only 39%. Relying on PFTs alone for screening for
ILD in juvenile systemic sclerosis would have missed the detection of
ILD in almost 2/3 of the sample cohort, supporting the use of HRCT
for detection of ILD in children with SSc. In addition, the cut off
utilized, of less than 80% of predicted FVC or DLCO could be too low
for pediatric patients to exclude beginning ILD. This pilot data needs
confirmation in a larger patient population.
Supported by the "Joachim Herz Stiftung
AB - Introduction: Juvenile systemic sclerosis (jSSc) has a prevalence in
around 3 in a million children. Pulmonary involvement occurs in
approximately 40 % in the international juvenile systemic
scleroderma cohort (JSScC). Traditionally in jSSc, pulmonary function
testing (PFT) with FVC and DLCO are used for screening and
computed tomography (HRCT) was more reserved for those with
abnormal PFTs. More recently, it has become apparent that PFTs
might not be sensitive enough for detecting interstitial lung disease
(ILD) in children.
Objectives: To assess the sensitivity and specificity of FVC and DLCO
assessment to detect ILD
Methods: The international juvenile systemic scleroderma cohort
(JSScC) database was queried for available patients with recorded
PFT parameters and HRCT performed to determine sensitivity of PFTs
detecting disease process.
Results: Of 129 patients in the jSScC, 67 patients had both CT
imaging and an FVC reading from PFTs for direct comparison. DLCO
readings were also captured but not in as many patients with
tandem HRCT (n =55 DCLO and HRCT scan). Therefore, initial
analyses focused on the sensitivity, specificity and accuracy of the
FVC value from the PFTs to capture the diagnosis of interstitial lung
disease as determined by HRCT.
Overall, 49% of the patients had ILD determined by HRCT, with 60%
of patients having normal FVC (>80%) with positive HRCT findings,
and 24% of patients having normal DLCO (> 80%) with positive
HRCT findings. Fourteen percent (n = 3/21) of patients with both FVC
and DLCO values within the normal range had a positive HRCT
finding.
Conclusion: The sensitivity of the FVC in the JSScC cohort in
detecting ILD was only 39%. Relying on PFTs alone for screening for
ILD in juvenile systemic sclerosis would have missed the detection of
ILD in almost 2/3 of the sample cohort, supporting the use of HRCT
for detection of ILD in children with SSc. In addition, the cut off
utilized, of less than 80% of predicted FVC or DLCO could be too low
for pediatric patients to exclude beginning ILD. This pilot data needs
confirmation in a larger patient population.
Supported by the "Joachim Herz Stiftung
UR - https://ped-rheum.biomedcentral.com/counter/pdf/10.1186/s12969-020-00469-y.pdf
M3 - Meeting Abstract
SN - 1546-0096
VL - 18
SP - 40
EP - 41
JO - Pediatric Rheumatology
JF - Pediatric Rheumatology
IS - Suppl.2
T2 - 26th European Paediatric Rheumatology Congress
Y2 - 23 September 2020 through 26 September 2020
ER -