Predictors of rapidly progressive interstitial lung disease and mortality in patients with autoantibodies against melanoma differentiation-associated protein 5 dermatomyositis.

Abstract

OBJECTIVE:

Anti-melanoma differentiation-associated protein 5 (MDA5)-positive DM is associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality. This multicentre retrospective study aimed to identify predictors of mortality and RP-ILD.

METHODS:

Anti-MDA5-positive DM patients were identified from the Hong Kong Myositis Registry and the Clinical Data Analysis and Reporting System. Clinical characteristics were reviewed. Risk factors for mortality and RP-ILD were identified.

RESULTS:

Among the 116 recruited patients, 100 (86.2%) had ILD, 47 (40.5%) had RP-ILD and 44 (37.9%) patients died. Cox regression analysis revealed RP-ILD [hazard ratio (HR) 9.735 (95% CI 3.905, 24.272)], age >52 years [HR 4.750 (95% CI 1.692, 13.333)], ferritin level >2800 pmol/l [HR 3.042 (95% CI 1.323, 6.997)] and lactate dehydrogenase (LDH) >400 IU/l [HR 2.290 (95% CI 1.009, 5.198)] were independent predictors of mortality. With regard to RP-ILD, analyses showed that potential predictors at baseline included age >50 years [HR 2.640 (95% CI 1.277, 5.455)], LDH >300 IU/l [HR 3.189 (95% CI 1.469, 6.918)], fever [HR 1.903 (95% CI 0.956, 3.790)] and neutrophil:lymphocyte ratio >7.0 [HR 1.967 (95% CI 0.942, 4.107)]. We proposed a prediction model based on fever, LDH, age and white cell count (FLAW) to stratify the risk of development of RP-ILD. The probability of RP-ILD in a patient with a score of 4 was 100%. A small internal validation cohort showed the odds of RP-ILD with FLAW scores of 0, 1, 2 and 3 were 0%, 0%, 42.9% and 75%, respectively.

CONCLUSIONS:

Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates. The FLAW model maybe useful to predict the development of RP-ILD.

• Authors: Jacqueline So, Ho So, Victor Tak-Lung Wong, Roy Ho, Tsz Yuen Wu, Priscilla Ching-Han Wong, Lydia Ho-Pui Tam, Chi Ho, Tommy Tsz-On Lam, Yuen Kwan Chung, Wai Ling Li, Chi Hung To, Chak Sing Lau, Chi Chiu Mok, Lai-Shan Tam
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 61 Issue 11 Pg. 4437-4444 (11 02 2022) ISSN: 1462-0332 [Electronic] England
• PMID: 35157042 (Publication Type: Multicenter Study, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Chemical References

• Autoantibodies
• Interferon-Induced Helicase, IFIH1
• L-Lactate Dehydrogenase

Topics

• Humans
• Middle Aged
• Dermatomyositis (complications)
• Autoantibodies
• Retrospective Studies
• Lung Diseases, Interstitial (etiology)
• Interferon-Induced Helicase, IFIH1
• Survival Rate
• L-Lactate Dehydrogenase
• Fever