Systematic review and meta-analysis of prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease

Objective

To clarify prognostic factors for idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD).

Design

Systematic review and meta-analysis using the Grades of Recommendation, Assessment, Development and Evaluation system.

Data sources

Medline, EMBASE and Science Citation Index Expanded were searched through 9 August 2018.

Eligibility criteria for selecting studies

The review includes primary studies addressing all-cause mortality of IIM-associated ILD. Potential prognostic factors were any clinical information related to the outcome.

Data extraction and synthesis

Two reviewers extracted relevant data independently and assessed risk of bias using the Quality in Prognostic Studies tool. Meta-analysis was conducted using a random effects model and if inappropriate the results were reported qualitatively. Prognostic factors were determined based on statistically significant results derived from multivariate analysis.

Results

Of a total of 5892 articles returned, 32 were deemed eligible for analysis and cumulatively, these studies reported 28 potential prognostic factors for all-cause mortality. Each study was subject to certain methodological constraints. The four prognostic factors, which demonstrated statistically significant results on both univariate and multivariate analyses, were as follows: age (MD 5.90, 3.17–8.63/HR 1.06, 1.02–1.10 and 2.31, 1.06–5.06), acute/subacute interstitial pneumonia (A/SIP) (OR 4.85, 2.81–8.37/HR 4.23, 1.69–12.09 and 5.17, 1.94–13.49), percentage of predicted forced vital capacity (%FVC) (OR 0.96, 0.95–0.98/HR 0.96, 0.93–0.99) and anti-Jo-1 antibody (OR 0.35, 0.18–0.71/HR 0.004, 0.00003–0.54) (univariate/multivariate, 95% CI). Other prognostic factors included ground glass opacity/attenuation (GGO/GGA) and extent of radiological abnormality. The quality of the presented evidence was rated as either low or very low.

Conclusions

Older age, A/SIP, lower value of %FVC, GGO/GGA and extent of radiological abnormality were demonstrated to predict poor prognosis for IIM-associated ILD while a positive test for anti-Jo-1 antibody indicated better prognosis. However, given the weak evidence they should be interpreted with caution.

Trial registration number

CRD42016036999.

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