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Am J Respir Crit Care Med. 2019 Jul 16. doi: 10.1164/rccm.201903-0486OC. [Epub ahead of print]

Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.

Author information

1
Oslo University Hospital, 155272, Oslo, Norway.
2
University of Oslo, 6305, Oslo, Norway ; a.m.hoffmann-vold@medisin.uio.no.
3
University of Oslo, 6305, Oslo, Norway.
4
Haukeland University Hospital, 60498, Bergen, Norway.
5
University Hospital of North Norway, 60519, Tromso, Norway.
6
Hospital of Southern Norway, Kristiansand, Norway.
7
Saint Olavs Hospital University Hospital in Trondheim, 60510, Trondheim, Norway.
8
Norwegian University of Science and Technology, 8018, Trondheim, Norway.
9
Lillehammer Hospital for Rheumatic diseases, Lillehammer, Norway.

Abstract

RATIONALE:

Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies.

OBJECTIVE:

Evaluate impact of ILD in a unique, nationwide, population based SSc cohort.

METHODS:

ILD was assessed prospectively in the Norwegian SSc cohort (Nor-SSc) including all 815 SSc patients resident in the country in 2000-2012. Lung HRCTs were available for fibrosis quantification at baseline (n=650, 80%) and follow-up. Pulmonary function tests were assessed at baseline (n=703, 86%) and follow-up. Vital status and standardized mortality rates (SMR) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched controls. Cumulative survival rates were computed.

MEASUREMENTS/MAIN RESULTS:

At baseline, 50% of the SSc patients (n=324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with >25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal range baseline FVC (80-100%) the 5- and 10-years survival rates correlated with presence or absence of lung fibrosis, being 83% and 80% with no fibrosis; and 69% and 56% with lung fibrosis (p=0.03).

CONCLUSION:

The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all SSc patients should undergo baseline PFT and lung HRCT screening to diagnose ILD early and tailor further management.

KEYWORDS:

interstitial lung disease; management; mortality; screening; systemic sclerosis

PMID:
31310156
DOI:
10.1164/rccm.201903-0486OC

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