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. 2024 Jan;59(1):72-80.
doi: 10.1002/ppul.26709. Epub 2023 Oct 16.

The SPEC score-A quantifiable CT scoring system for primary ciliary dyskinesia

Tarika Chowdhary  1 , Jennifer Bracken  2 , Lucy Morgan  3   4 , Andre Schultz  5   6 , Phil Robinson  1   2   7
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The SPEC score-A quantifiable CT scoring system for primary ciliary dyskinesia

Tarika Chowdhary et al. Pediatr Pulmonol. 2024 Jan.

Abstract

Background: Structural lung changes seen on computed tomography (CT) scans in persons with primary ciliary dyskinesia (pwPCD) are currently described using cystic fibrosis (CF) derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.

Methods: Chest CT scans from 30 pwPCD, were described for structural changes including bronchiectasis, bronchial wall thickening, mucous plugging, atelectasis, air trapping, and interlobar septal thickening and, additionally, changes previously described as being frequent in pwPCD including extensive tree-in-bud pattern of mucous plugging, bronchoceles or nodules, thickening of interlobar and interlobular septa and whole lobe atelectasis. Based on these findings a novel and unique scoring system, the Specific PCD Evaluation by CT (SPEC) score was constructed. Scans were then re-scored using the SPEC score and results compared to corresponding measurements of lung function to assess structure-function correlation.

Results: Total SPEC scores ranged from 0 to 60 (max possible score 90). There was a strong negative correlation between the SPEC score (SPEC) and forced vital capacity (FVC), forced expiratory volume over 1 s (FEV1 ) and FEV1 /FVC ratio (-r = .784, -.865, -.872 respectively).

Conclusions: Using PCD-derived data we describe the construct of a PCD-specific score for assessing lung structural damage on CT scans, the SPEC score. A strong correlation between the SPEC score and PFT variables was identified. The SPEC score holds the potential for describing longitudinal changes in CT scans and assessing the efficacy of interventive therapies in patients with PCD.

Keywords: CF scoring system; CT structural changes; primary ciliary dyskinesia; spirometry.

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References

REFERENCES

    1. Hannah WB, Seifert BA, Truty R, et al. The global prevalence and ethnic heterogeneity of primary ciliary dyskinesia gene variants: a genetic database analysis. Lancet Respir Med. 2022;10(5):459-468.
    1. Lucas JS, Barbato A, Collins SA, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017;49(1):1601090.
    1. Lucas JS, Burgess A, Mitchison HM, Moya E, Williamson M, Hogg C. Diagnosis and management of primary ciliary dyskinesia. Arch Dis Child. 2014;99(9):850-856.
    1. Boon M, Vermeulen FL, Gysemans W, et al. Lung structure-function correlation in patients with primary ciliary dyskinesia. Thorax. 2015;70:339-345.
    1. Brody AS, Molina PL, Klein JS, Rothman BS, Ramagopal M, Swartz DR. High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol. 1999;29(10):731-735.

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