Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Diagnostic Process
2.2.1. Clinical Evaluation
2.2.2. Laboratory
2.2.3. Chest High-Resolution Computed Tomography (HRCT)
2.2.4. Pulmonary Function Tests (PFTs)
2.2.5. Transthoracic Echocardiography
2.3. Patient Classification
2.4. Follow-Up
- Deterioration of lung function on PFTs, defined by an absolute decline in forced vital capacity (FVC) of ≥5% predicted or in DLCO of 10% predicted [31].
- Radiological progression on HRCT: the trend of ILD (stable, deteriorated, or ameliorated) was established by the most expert thoracic radiologist, blinded to the clinical and functional evaluation, through a semi-quantitative analysis. Particularly evaluated were the presence or increase of the extent of traction bronchiectasis/bronchiolectasis, ground glass opacities, reticulation, and honeycombing as well as the increase of the lobar volume loss [31]. Figure 2 reports an example of ILD progression on HRCT.
- New chronic need for supplemental oxygen.
- Death due to ILD.
- Acute exacerbations, determining an acute respiratory insufficiency and/or a hospital admission and/or a broad-spectrum antibiotic therapy.
2.5. Statistical Analysis
3. Results
3.1. Baseline Characteristics
3.2. Follow-Up
3.3. Predictive Factors for ILD Progression at T1
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Post-Hoc Analysis | |||||||
---|---|---|---|---|---|---|---|
IPAF | CTD-ILD | UIPAF | p-Value | IPAF vs. CTD-ILD | IPAF vs. UIPAF | CTD-ILD vs. UIPAF | |
No. of subjects | 37 (36.6) | 53 (52.5) | 11 (10.9) | ||||
Age (years) | 71 (62.5–75.5) | 69 (60.5–74) | 68 (66–79) | 0.64 | |||
Female | 20 (54.1) | 37 (69.8) | 8 (72.7) | 0.27 | |||
Never-smokers | 13 (35.1) | 23 (43.4) | 4 (36.4) | 0.77 | |||
Months from symptoms’ onset | 15 (7–39.5) | 11 (6–34.5) | 12 (6–17) | 0.64 | |||
Systemic hypertension | 31 (83.8) | 30 (56.6) | 5 (45.5) | <0.01 | 0.011 | 0.017 | 0.526 |
Left-sided heart failure | 9 (24.3) | 3 (5.7) | 2 (18.2) | 0.03 | 0.024 | 1.00 | 0.201 |
Type 2 diabetes | 7 (18.9) | 10 (18.9) | 2 (18.2) | 1 | |||
Hypercholesterolemia | 17 (45.9) | 17 (32.1) | 4 (36.4) | 0.41 | |||
BMI (kg/m2) | 27.5 (24.8–31) | 25.6 (22.9–29) | 25.5 (24–28.9) | 0.09 | |||
OSAS | 6 (16.2) | 3 (5.7) | 1 (9.1) | 0.25 | |||
GERD | 12 (32.4) | 17 (32.1) | 5 (45.5) | 0.68 | |||
Concurrent autoimmune disease | 12 (32.4) | 19 (35.8) | 0 (0) | 0.04 | 0.824 | 0.041 | <0.01 |
Autoimmune thyroiditis | 7 (18.9) | 11 (20.8) | 0 (0) | ||||
Pulmonary hypertension (PH) | 12 (32.4) | 13 (24.5) | 2 (18.2) | 0.61 | |||
Pre-capillary PH | 3 (8.1) | 6 (11.3) | 0 (0) | ||||
ARDS at onset | 2 (5.4) | 3 (5.6) | 0 (0) | 1 | |||
Corticosteroid therapy | 31 (83.8) | 49 (92.5) | 8 (72.7) | 0.12 | |||
With an immunosuppressant | 18 (48.6) | 26 (49.1) | 1 (9.1) | 0.04 | 0.970 | 0.032 | 0.018 |
With an anti-fibrotic agent | 4 (10.8) | 6 (11.3) | 8 (72.7) | <0.01 | 0.940 | <0.01 | <0.01 |
Post-Hoc Analysis | |||||||
---|---|---|---|---|---|---|---|
IPAF | CTD-ILD | UIPAF | p-Value | IPAF vs. CTD-ILD | IPAF vs. UIPAF | CTD-ILD vs. UIPAF | |
Pattern | |||||||
UIP | 4 (10.8) | 17 (32.1) | 11 (100) | <0.01 | 0.023 | <0.01 | <0.01 |
NSIP | 21 (56.8) | 23 (43.4) | 0 (0) | <0.01 | 0.212 | <0.01 | <0.01 |
OP | 4 (10.8) | 2 (3.8) | 0 (0) | 0.3 | |||
ILD + Multi-compartment involvement ꝉ | 7 (18.9) | 10 (18.9) | 0 (0) | 0.35 | |||
Other patterns | 1 (2.7) | 1 (1.9) | 0 (0) | 1 | |||
PFTs | |||||||
FEV1 (% predicted) | 83 (76–102.5) | 84 (75–101) | 92 (76–115) | 0.55 | |||
FVC (% predicted) | 86 (73–97) | 91 (80–110) | 94 (69–106) | 0.3 | |||
DLCO (% predicted) | 61 (49–70) | 49.5 (34.8–75) | 46 (36.5–60.5) | 0.36 | |||
DLCO <35% and/or FVC <50% | 5 (13.5) | 14 (26.4) | 3 (27.7) | 0.28 | |||
6MWT | |||||||
Distance (m) | 495 (378–540) | 459 (394–539) | 461 (351–500) | 0.53 | |||
Lowest SpO2 (%) | 92 (89–96) | 91 (83–96) | 86 (76–93) | 0.12 |
Post-Hoc Analysis | |||||||
---|---|---|---|---|---|---|---|
IPAF | CTD-ILD | UIPAF | p-Value | IPAF vs. CTD-ILD | IPAF vs. UIPAF | CTD-ILD vs. UIPAF | |
No. of subjects | 35 (36.1) | 51 (52.6) | 11 (11.3) | ||||
PFTs | |||||||
progression | 9 (26.5) | 22 (43.1) | 7 (63.6) | 0.07 | |||
improvement | 9 (25.7) | 9 (17.6) | 0 (0) | 0.14 | |||
stable | 16 (47.1) | 20 (39.2) | 4 (36.4) | 0.72 | |||
DLCO < 35% and/or FVC < 50% | 4 (11.4) | 20 (39.2) | 3 (27.3) | 0.01 | <0.01 | 0.337 | 0.516 |
HRCT | |||||||
progression | 6 (17.1) | 18 (35.3) | 5 (45.5) | 0.09 | |||
improvement | 9 (24.3) | 4 (7.8) | 0 (0) | 0.02 | 0.029 | 0.087 | 1.00 |
stable | 19 (54.3) | 29 (56.9) | 6 (54.5) | 0.95 | |||
Supplementary oxygen | 3 (8.6) | 16 (31.4) | 4 (36.4) | 0.01 | 0.017 | 0.050 | 0.735 |
At least one exacerbation | 7 (20.0) | 17 (33.3) | 2 (18.2) | 0.35 | |||
Composite endpoint of ILD progression | 11 (32.3) | 30 (58.8) | 8 (72.7) | 0.02 | 0.017 | 0.033 | 0.461 |
Univariate | Multivariate | |||
---|---|---|---|---|
Characteristics | OR (95% CI) | p-Value | OR (95% CI) | p-Value |
Age (years) | 1.00 (0.97–1.04) | 0.91 | 0.98 (0.94–1.03) | 0.53 |
Female sex | 0.81 (0.34–1.88) | 0.62 | 0.96 (0.34–2.74) | 0.94 |
Never-smoker | 0.85 (0.37–1.95) | 0.71 | ||
Pulmonary hypertension | 2.36 (0.92–6.48) | 0.12 | ||
ARDS at onset | 1.14 (0.13–9.83) | 0.91 | ||
IPAF | 0.30 (0.12–0.72) | <0.01 | 0.28 (0.09–0.79) | 0.02 |
UIP pattern | 4.31 (1.73–11.7) | <0.01 | 3.80 (1.33–11.9) | 0.01 |
ANA | 0.76 (0.33–1.22) | 0.51 | ||
RF ≥ 2 times above the upper limit | 5.77 (1.41–39.0) | 0.03 | 9.13 (1.8–53.3) | 0.02 |
Anti-CCP | 0.96 (0.17–5.41) | 0.96 | ||
Anti-Ro (SSA) | 0.48 (0.14–1.51) | 0.22 | ||
Anti-Scl-70 | 0.96 (0.04–24.7) | 0.98 | ||
Anti-tRNA synthetase | 0.68 (0.21–2.13) | 0.51 | ||
Anti-PM/Scl | 0.70 (0.13–3.36) | 0.65 | ||
Mechanic’s hand | 0.47 (0.21–5.06) | 0.54 | ||
Arthritis or joint stiffness ≥ 60 min | 1.02 (0.45–2.30) | 0.97 | ||
Palmar teleangiectasia | 2.06 (0.76–6.03) | 0.17 | ||
Raynaud phenomenon | 1.74 (0.72–4.35) | 0.23 | ||
Sicca syndrome | 0.49 (0.19–1.23) | 0.14 | ||
Corticosteroid therapy | 1.81 (0.56–6.41) | 0.33 | ||
With immunosuppressant | 1.00 (0.45–2.26) | 0.98 | ||
With an anti-fibrotic agent ꝉ | 9.00 (2.32–59.8) | <0.01 |
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Bozzao, F.; Tomietto, P.; Baratella, E.; Kodric, M.; Cifaldi, R.; Della Porta, R.; Prearo, I.; Pirronello, S.M.G.; Confalonieri, P.; Ruaro, B.; et al. Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria. Medicina 2023, 59, 794. https://doi.org/10.3390/medicina59040794
Bozzao F, Tomietto P, Baratella E, Kodric M, Cifaldi R, Della Porta R, Prearo I, Pirronello SMG, Confalonieri P, Ruaro B, et al. Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria. Medicina. 2023; 59(4):794. https://doi.org/10.3390/medicina59040794
Chicago/Turabian StyleBozzao, Francesco, Paola Tomietto, Elisa Baratella, Metka Kodric, Rossella Cifaldi, Rossana Della Porta, Ilaria Prearo, Silvia Maria Grazia Pirronello, Paola Confalonieri, Barbara Ruaro, and et al. 2023. "Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria" Medicina 59, no. 4: 794. https://doi.org/10.3390/medicina59040794
APA StyleBozzao, F., Tomietto, P., Baratella, E., Kodric, M., Cifaldi, R., Della Porta, R., Prearo, I., Pirronello, S. M. G., Confalonieri, P., Ruaro, B., Fischetti, F., & Fabris, B. (2023). Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria. Medicina, 59(4), 794. https://doi.org/10.3390/medicina59040794