Predictors of Rapidly Progressive Interstitial Lung Disease and Mortality in Patients with Autoantibodies Against Melanoma Differentiation-Associated Protein 5 Dermatomyositis

Jacqueline So¹, Lai-Shan Tam², Priscilla Wong¹, Lydia Ho-Pui Tam¹, Tsz On Lam¹, Chi Chiu Mok³, Chi Hung To⁴, Yuen Kwan Chung⁴, Victor Tak Lung Wong⁵, Tsz Yuen Wu⁶, Roy Ho⁷, Wai Ling Li⁸, Chi Ho⁹ and Ho So¹⁰, ¹Prince of Wales Hospital, Hong Kong, Hong Kong, ²Department of Medicine & Therapeutics, The Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China (People's Republic), ³Tuen Mun Hospital, Hong Kong, China, ⁴Pok Oi Hospital, Hong Kong, Hong Kong, ⁵Kwong Wah Hospital, Hong Kong, Hong Kong, ⁶Princess Margaret Hospital, Hong Kong, Hong Kong, ⁷Queen Elizabeth Hospital, Hong Kong, Hong Kong, ⁸Queen Mary Hospital, Hong Kong, Hong Kong, ⁹Alice Ho Miu Ling Nethersole Hospital, Hong Kong, Hong Kong, ¹⁰CUHK, Hong Kong, China

Meeting: ACR Convergence 2021

Keywords: interstitial lung disease, Myositis

Session Information

Date: Sunday, November 7, 2021

Title: Muscle Biology, Myositis & Myopathies Poster (0683–0722)

Session Type: Poster Session B

Session Time: 8:30AM-10:30AM

Background/Purpose: Anti-melanoma differentiation-associated protein 5 (MDA5) positive dermatomyositis (DM) is associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality. This multi-centre retrospective study aimed to evaluate whether RP-ILD was associated with increased mortality in anti-MDA5 positive DM in Hong Kong and to identify predictors for 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. Diagnosis of DM was based on Bohan and Peter’s criteria or EULAR/ ACR 2017 classification criteria. Diagnosis of CADM was defined as typical cutaneous features of DM confirmed by rheumatologist or dermatologist but minimal or no clinical features of myositis (hypomyopathic and amyopathic) with reference to the Sontheimer’s criteria. Detailed clinical characteristics were reviewed. RP-ILD was defined as rapid progression of dyspnea or imaging finding of ILD within 1 month of onset of respiratory symptoms. Risk factors for mortality and RP-ILD were identified.

Results: Among the 101 recruited patients, 87 (86.1%) had ILD and 42 (41.6%) had RP-ILD. Over a period of 19.1±21.9 months, 38 (37.6%) patients died, in which 31 patients (81.6%) died within three months of diagnosis. Multivariate Cox regression analysis revealed that RP-ILD (HR 17.6, 95%CI 5.96-51.9), smoking (HR 6.40, 95%CI 1.43-28.7), ferritin level >2800pmol/L (HR 6.37, 95%CI 2.40-16.9) and fever at diagnosis (HR 3.11, 95%CI 1.14-8.53) were independent predictors of mortality. Early rituximab use was associated with better survival (HR 0.10, 95% CI 0.01-0.82). With regards to RP-ILD, multivariate Cox regression analyses showed that independent predictors included age >50 years (HR 3.02, 95%CI 1.43-6.38), baseline neutrophil to lymphocyte ratio (NLR) >7.5 (HR 2.45, 95%CI 1.05-5.71) and lactate dehydrogenase (LDH) level >300IU/L (HR 3.34, 95%CI 1.36-8.21). We proposed a prediction model, based on NLR >7.5, age >50 years old and LDH >300IU/L (“NAL”), to stratify risk of development of RP-ILD.

Conclusion: Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates. The “NAL” model maybe useful to predict the development of RP-ILD in anti-MDA5 positive DM patients.

Patients’ demographic, clinical features, treatments and outcomes

Results of multivariate Cox regression analyses for RP-ILD

Prediction model for RP-ILD based on age >50, NLR >7.5 and LDH >300 IU/L at diagnosis

Disclosures: J. So, None; L. Tam, Janssen, 2, 5, Pfizer, 2, 5, GlaxoSmithKline, 5, AbbVie, 2, Novartis, 5, Amgen, 5, Boehringer Ingelheim, 2, 5, Eli Lilly, 2, Sanofi, 2; P. Wong, None; L. Tam, None; T. Lam, None; C. Mok, None; C. To, None; Y. Chung, None; V. Wong, None; T. Wu, None; R. Ho, None; W. Li, None; C. Ho, None; H. So, None.

To cite this abstract in AMA style:

So J, Tam L, Wong P, Tam L, Lam T, Mok C, To C, Chung Y, Wong V, Wu T, Ho R, Li W, Ho C, So H. Predictors of Rapidly Progressive Interstitial Lung Disease and Mortality in Patients with Autoantibodies Against Melanoma Differentiation-Associated Protein 5 Dermatomyositis [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/predictors-of-rapidly-progressive-interstitial-lung-disease-and-mortality-in-patients-with-autoantibodies-against-melanoma-differentiation-associated-protein-5-dermatomyositis/. Accessed .

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