Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity

Abstract

Objective Rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody potentially presents with a fatal clinical course and requires early intensive treatment. Recently, perilobular opacity was reported to pathologically correspond to the acute phase of diffuse alveolar damage in RP-ILD with anti-MDA5 antibody. We aimed to investigate whether or not perilobular opacity was a common radiological finding in RP-ILD patients with anti-MDA5 antibody. Methods We conducted a retrospective review of the medical records of eight consecutive patients with RP-ILD with anti-MDA5 antibody. The clinical features and radiological findings of follow-up computed tomography (CT) during the course of their disease were evaluated. Results Among eight RP-ILD patients with anti-MDA-5 antibody, six showed perilobular opacity in the lower lobes, and the remaining two had only consolidation on high-resolution CT. Of note, the perilobular opacity in all six patients thickened and progressed to consolidation with a loss of lung volume in a short period. Despite intensive treatment, 6 patients (75%) died within 100 days after the first visit. Notably, the two patients with consolidation presented with a very rapid clinical course and died in 13 days each. In the two survivors, the perilobular opacity and consolidation recovered with improvement in the loss of lung volume. Conclusion Rapidly progressive perilobular opacity that thickens and progresses to consolidation is characteristic of RP-ILD with anti-MDA5 antibody. Chest physicians should immediately check the status of anti-MDA-5 antibody in order to initiate early aggressive therapy in RP-ILD patients with rapidly progressive perilobular opacity.

A Computed Tomography Radiomics-Based Prediction Model on Interstitial Lung Disease in Anti-MDA5-Positive Dermatomyositis

“…Of note, in the population of MDA5 + DM-ILD, the organizing pneumonia pattern was found to be dominant in HRCT, with lower zone consolidation correlating with rapidly progressive ILD ( 28 ). Moreover, another study with pathological evidences discovered that a perilobular opacity transforming to consolidation corresponded to diffuse alveolar damage, which was presented as the main finding in MDA5 + DM associated with rapidly progressive ILD ( 29 ). Thus, these manifestations might result in the changes of CT values and texture, making these radiomic features important for diagnosis, risk stratification, and prognosis.…”

Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis

“…Pathological findings of anti-MDA5 antibody-positive ILD were also reported. In most of the reported cases, autopsy was performed, and the pathological pattern of ILD was that of diffuse alveolar damage [ [17] , [18] , [19] ]. Chino et al reported a patient with anti-MDA5 antibody-positive ILD who underwent SLB.…”

Editorial: Interstitial Lung Disease in the Context of Systemic Disease: Pathophysiology, Treatment and Outcomes

“…ILD is common in myositis, especially in patients with antibodies directed against different aminoacyl-tRNA synthetases, a group collectively called the antisynthetase syndrome (ASyS), but also those with anti-Ro52 antibodies ( 5 , 6 ). Anti-MDA5 antibodies are associated with clinically amyopathic dermatomyositis (CADM) which can present as a rapidly progressive ILD with high mortality despite aggressive immunosuppressive therapy ( 7 ). The following summarizes the papers publishes within this Research Topic.…”