Elsevier

Respiratory Medicine

Volume 103, Issue 6, June 2009, Pages 846-853
Respiratory Medicine

Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases

https://doi.org/10.1016/j.rmed.2008.12.019Get rights and content
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Summary

Background

Acute exacerbation (AE) is currently established as a distinct condition with acute deterioration of respiratory status in idiopathic pulmonary fibrosis (IPF). Recently, several studies have reported that AE also occurred in interstitial pneumonias other than IPF, such as collagen vascular disease-associated interstitial pneumonia (CVD-IP). However, the incidence of AE in CVD-IP and its clinical characteristics remain to be fully determined. This study was conducted to elucidate cumulative incidence of AE in CVD-IP and its clinical features.

Methods

We reviewed 83 biopsy-proven CVD-IP patients, estimated cumulative incidence of AE, and examined its clinical characteristics.

Results

Among 83 CVD-IP patients, six patients with a mean age of 65.7 years developed AE (overall incidence, 7.2%; 1-year incidence, 1.25%). Underlying CVDs included rheumatoid arthritis (RA) (n = 5; overall incidence, 20.0%) and primary Sjögren syndrome (n = 1; overall incidence, 5.9%). Patients with AE showed acute respiratory deterioration with severe hypoxemia (mean PaO2/FiO2 ratio, 131). Radiologically, ground-glass opacity was superimposed on the underlying reticular abnormalities. Preexisting histological patterns included three usual interstitial pneumonia (UIP) and two non-specific interstitial pneumonia (NSIP). Five (83.3%) of six patients died of respiratory failure despite intensive therapy. Univariate Cox's proportional hazards analysis showed that age and RA diagnosis were significantly associated with AE. Multivariate Cox's proportional hazards analysis indicated that age was an independent significant factor predicting AE.

Conclusions

These data suggest that AE can occur in CVD-IP, and this condition is closely similar to that of IPF with poor prognosis. AE is most common in RA, and associated with higher ages.

Keywords

Collagen vascular disease
Interstitial pneumonia
Acute exacerbation

Abbreviations

ADM
amyopathic dermatomyositis
CVD
collagen vascular disease
DM
dermatomyositis
MCTD
mixed connective tissue disease
NSIP
non-specific interstitial pneumonia
PM
polymyositis
pSS
primary Sjögren syndrome
RA
rheumatoid arthritis
SLE
systemic lupus
SSc
systemic sclerosis
UIP
usual interstitial pneumonia

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