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Subclinical ILD is frequent and progresses across different
connective tissue diseases
Anna-M ari a H offma nn-Vo ld, He len a Ande rs son, Silje Reise ter, Hå vard
Fret hei m, Imon Ba rua , Torhild Ga ren, Øyvin d Midtvedt, Ra gna r
Gunnarss on, Mic hae l Durhe im, Trond M ogens Aaløkke n, Øyvind
Molbe rg
Oslo Unive rsity Hospital
Conflict of interest disclosure
 I have no real or perceived conflicts of interest that relate to this presentation.
 I have the following real or perceived conflicts of interest that relate to this presentation:
Affiliation / Financial interest Commercial company
Grants/research support: Boehringer Ingelheim

Honoraria or consultation fees: Actelion, ARXX therapeutics, Bayer, Boehringer Ingelheim, Jansen&Jansen, Lilly, Medscape, Merck Sharp &
Dohme and Roche

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Background

Interstitial lung abnormalities (ILAs) are common incidental findings on HRCT, progress
frequently and are associated with mortality¹

ILAs preclinical identified in patients with connective tissue diseases (CTD) should be
defined as preclinical interstitial lung disease (ILD)¹

The concept of pre (sub)clinical disease is universal in medicine

The symptoms-define-disease argument would in CTD mean that ILD is not a disease
until patients become symptomatic, which is frequently first present in advanced stages
Aims / Learning objectives

1. To identify the prevalence of subclinical ILD across different

CTD
2. Assess the rate of lung fibrosis progression
 Patient cohort and ILD definitions

All CTD patients, including systemic sclerosis (SSc)¹, anti-synthetase syndrome (ASS)² and mixed
connective tissue disease (MCTD)³ from the Oslo University Hospital diagnosed were included and ILD
assessed by semi-quantitative assessment¹

Clinical ILD
Subclinical ILD
ILD extent >5% on HRCT
ILD extent <5% by semi-quantitative assessment
ILD extent <5% on HRCT
Preserved lung function with FVC >80% predicted
-with respiratory symptoms
No respiratory symptoms
-with FVC<80%

¹Hoffmann-Vold et al. Arthritis Rheumatol. 2015 May;67(8):2205-12; ²Andersson et al. J Rheumatol. 2016 Jun;43(6):1107-13; ³Reiseter et al. Rheumatology (Oxford). 2018 Feb 1;57(2):255-262
 Methodology

ILD progression
Increasing extent of ILD from baseline to follow-up HRCT¹

Statistical approach
Descriptive statistical analyses were conducted
Time to event assessed with Kaplan-Meier estimates and log reg test

¹Hoffmann-Vold et al. Am J Respir Crit Care Med. 2019 Nov 15;200(10):1258-1266

We identified 525 CTD patients assessed with
HRCT for the presence of ILD

ASS
94 (18%)

SSc
MCTD 296 (56%)
135 (26%)
The prevalence of subclinical ILD varied between
10% in MCTD and ASS and 15% in SSc
Subclinical ILD
67 (13%)
No ILD
231 (43%)

MCTD (13)

ASS (9)

SSc (45)

ILD
227 (44%)
Clinical characteristics, demographics and
outcome of CTD without ILD, with subclinical
and clinical ILD varied

  No ILD Subclinical ILD Clinical ILD

(n=227) (n=67) (n=231)
Age, y (SD) 50 (15.4) 51 (14.4) 52 (15.3)
Male sex, n (%) 89 (39) 22 (33) 111 (48)
Deceased, n (%) 50 (22) 12 (18) 91 (39)
Observation period, y median (range) 13.7 (18.6) 13.9 (17.9) 11.5 (17.1)
FVC% (SD) 97 (18.6) 99 (17.9) 81 (20.9)
FVC decline% (SD) -0.70 (11.1) -0.81 (16.5) -1.61 (15.9)
DLCO% (SD) 73 (19.4) 73 (16.9) 55 (17.4)
Extent of ILD% (SD) 0 (0) 2.3 (1.5) 19.3 (16.8)
ILD progression% (SD) 0.08 (1.0) 3.1 (6.2) 3.6 (9.9)
Subclinical ILD progressed in 38% of patients with CTD

Over a median time of 4.5 years

95/395 (24%) showed lung fibrosis
progression
72 (26%) SSc
23 (19%) MCTD patients
Disease progression was frequently
present in
subclinical ILD (38%)
ILD (51%) patients
Conclusion

Subclinical ILD is frequently present across CTDs

Subclinical ILD progresses over time in a substantial subgroup of patients,

comparable to patients with clinical ILD

Our findings question the terms sub- and preclinical ILD, which may potentially
lead to a suboptimal “watchful waiting management strategy”
List of references

1. Hatabu et al. Lancet Respir Med 2020 Jul;8(7):726-737

2. Hoffmann-Vold et al. Arthritis Rheumatol. 2015 May;67(8):2205-12
3. Andersson et al. J Rheumatol. 2016 Jun;43(6):1107-13
4. Reiseter et al. Rheumatology (Oxford). 2018 Feb 1;57(2):255-262
5. Hoffmann-Vold et al. Am J Respir Crit Care Med. 2019 Nov 15;200(10):1258-1266