Utilize lung ultrasound B-lines and KL-6 to monitor anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis-associated interstitial lung disease: a case report and literature review

Wang, Yukai; Chen, Shaoqi; Lin, Zhangzhang; Lin, Jianqun; Xie, Xuezhen; Lin, Qisheng; Du, Guangyuan; Huang, Xiu‐Feng; Matucci‐Cerinic, Marco; Fürst, Daniel E.

doi:10.1007/s10067-019-04462-z

Cited by 6 publications

(7 citation statements)

References 20 publications

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“…A recent study reported a patient with anti‐MDA5 + CADM‐ILD successfully treated with aggressive immunotherapy and followed up using lung ultrasound and KL‐6 . This indicated that the combined use of lung ultrasound and KL‐6 might be a safe, helpful way for the assessment and follow‐up of ILD . The ROC curves in this study supported that AUC was better when KL‐6 was combined with ferritin or HRCT score compared with KL‐6 alone.…”

Section: Discussionsupporting

confidence: 67%

“…Elevated KL‐6 levels could indicate the possibility of ILD, helping to predict outcomes and evaluate treatment efficacy. A recent study reported a patient with anti‐MDA5 + CADM‐ILD successfully treated with aggressive immunotherapy and followed up using lung ultrasound and KL‐6 . This indicated that the combined use of lung ultrasound and KL‐6 might be a safe, helpful way for the assessment and follow‐up of ILD .…”

Section: Discussionmentioning

confidence: 99%

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Serum KL‐6 level is a prognostic marker in patients with anti‐MDA5 antibody‐positive dermatomyositis associated with interstitial lung disease

Yan

Wang

et al. 2019

Clinical Laboratory Analysis

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Objective This study aimed to investigate the clinical significance of Krebs von den Lungen‐6 (KL‐6) serum levels in patients with anti‐MDA5 antibody‐positive dermatomyositis (anti‐MDA5+ DM) having interstitial lung disease (ILD), especially in the amyopathic DM phenotype. Methods The serum KL‐6 level was measured using a chemiluminescence enzyme immunoassay (CLEIA) in patients with anti‐MDA5+ DM, including clinically amyopathic dermatomyositis (CADM)‐ILD and classic DM‐ILD, and healthy donors. The baseline and post‐treatment serum KL‐6 levels were determined in 39 patients with CADM‐ILD who experienced remission or acute exacerbation. The association between laboratory findings, high‐resolution computed tomography (HRCT) scores, pulmonary function tests (PFTs), and the predictive value of baseline KL‐6 level for death was analyzed. Results The serum KL‐6 levels were significantly higher in patients with CADM‐ILD (1339 ± 1329 U/mL) compared with DM‐ILD (642.3 ± 498.4 U/mL) and healthy donors (162.4 ± 54.01 U/mL). The KL‐6 levels correlated positively with chest HRCT scores, serum lactate dehydrogenase, serum ferritin levels, and PFTs, but not with erythrocyte sedimentation rate. During follow‐up, the post‐treatment serum KL‐6 levels significantly reduced in the remission/stable group, but increased in the acute exacerbation group. Higher levels of ferritin and KL‐6 and HRCT scores were independently associated with poor prognosis. The 1‐year survival rate was significantly lower in patients with high KL‐6 level than in those with low KL‐6 level. Conclusion The serum KL‐6 levels may be a useful marker for predicting and monitoring ILD in Chinese patients with anti‐MDA5+ DM, especially amyopathic DM phenotype.

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Section: Discussionsupporting

confidence: 67%

Section: Discussionmentioning

confidence: 99%

Serum KL‐6 level is a prognostic marker in patients with anti‐MDA5 antibody‐positive dermatomyositis associated with interstitial lung disease

Yan

Wang

et al. 2019

Clinical Laboratory Analysis

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“…Today, HRCT is still a ‘gold standard’ to diagnose and follow the ILD morphological changes. However, the radiological burden, and the large, non-portable equipment limit HRCT clinical application to closely monitor the lung [ 25 ]. Furthermore, respiratory failure due to acute exacerbation of ILD may sometimes inhibit patients from properly performing PFTs.…”

Section: Discussionmentioning

confidence: 99%

“…The treatment option was decided according to the close follow-up performed by monitoring B-lines numbers and serum KL-6 level. The patient was successfully treated and chest ultrasound was employed for detecting and following the rapidly progressing ILD, in particular combining LUS and serum biomarkers [ 25 ].…”

Section: Discussionmentioning

confidence: 99%

Lung ultrasound B-lines and serum KL-6 correlate with the severity of idiopathic inflammatory myositis-associated interstitial lung disease

et al. 2019

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Objective Idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) significantly increases morbidity and mortality. Lung ultrasound B-lines and Krebs von den Lungen-6 (KL-6) are identified as new sonographic and serum markers of ILD, respectively. The aim of our work was to assess the role of B-lines and KL-6 as markers of the severity of IIM-ILD. For this purpose, the correlation among B-lines score, serum KL-6 levels, high-resolution CT (HRCT) score, and pulmonary function tests were investigated in IIM-ILD patients. Methods Thirty-eight patients with IIM-ILD underwent chest HRCT scans, lung ultrasound and pulmonary function tests (independently performed within 1 week) examination. To assess severity and extent of ILD at HRCT, the Warrick score was used. The B-lines score denoting the extension of ILD was calculated by summing the number of B-lines on a total of 50 scanning sites. Serum KL-6 levels (U/ml) was measured by chemiluminescent enzyme immunoassay. Results A significant correlation was found between the B-lines score and serum KL-6 levels (r = 0.43, P < 0.01), and between the Warrick score and serum KL-6 levels (r = 0.45, P < 0.01). A positive correlation between B-lines score and the Warrick score (r = 0.87, P < 0.0001) was also confirmed. Both B-lines score and KL-6 levels inversely correlated to diffusion capacity for carbon monoxide (r = −0.77, P < 0.0001 and r = −0.42, P < 0.05, respectively) and total lung capacity (r = −0.73, P < 0.0001 and r = −0.36, P < 0.05, respectively). Moreover, B-lines correlated inversely with forced vital capacity (r = −0.73, P < 0.0001), forced expiratory volume in 1 s (r = −0.69, P < 0.0001). Conclusion B-lines score and serum KL-6 levels correlate with HRCT findings and pulmonary function tests, supporting their use as measures of IIM-ILD severity.

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“…The significant relationship was confirmed in 38 patients with IIM-ILD ( r = 0.43, p < 0.01) [ 18 ]. In a recent case, B-lines and KL-6 were utilized to closely detect and follow a patient with anti-MDA-5-positive, clinically amyopathic dermatomyositis associated with rapidly progressive ILD [ 79 ]. Changes in B-line numbers and serum levels of KL-6 were consistent with the changes in HRCT findings and clinical presentation.…”

Section: Review Of Relevant Medical Literaturementioning

confidence: 99%

The role of lung ultrasound B-lines and serum KL-6 in the screening and follow-up of rheumatoid arthritis patients for an identification of interstitial lung disease: review of the literature, proposal for a preliminary algorithm, and clinical application to cases

et al. 2021

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Screening and follow-up of interstitial lung disease associated with rheumatoid arthritis (RA-ILD) is a challenge in clinical practice. In fact, the majority of RA-ILD patients are asymptomatic and optimal tools for early screening and regular follow-up are lacking. Furthermore, some patients may remain oligosymptomatic despite significant radiological abnormalities. In RA-ILD, usual interstitial pneumonia (UIP) is the most frequent radiological and pathological pattern, associated with a poor prognosis and a high risk to develop acute exacerbations and infections. If RA-ILD can be identified early, there may be an opportunity for an early treatment and close follow-up that might delay ILD progression and improve the long-term outcome.In connective tissue disease–associated interstitial lung disease (CTD-ILD), lung ultrasound (LUS) with the assessment of B-lines and serum Krebs von den Lungen-6 antigen (KL-6) has been recognized as sensitive biomarkers for the early detection of ILD. B-line number and serum KL-6 level were found to correlate with high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and other clinical parameters in systemic sclerosis–associated ILD (SSc-ILD). Recently, the significant correlation between B-lines and KL-6, two non-ionizing and non-invasive biomarkers, was demonstrated. Hence, the combined use of LUS and KL-6 to screen and follow up ILD in RA patients might be useful in clinical practice in addition to existing tools. Herein, we review relevant literature to support this concept, propose a preliminary screening algorithm, and present 2 cases where the algorithm was used.

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Utilize lung ultrasound B-lines and KL-6 to monitor anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis-associated interstitial lung disease: a case report and literature review

Cited by 6 publications

References 20 publications

Serum KL‐6 level is a prognostic marker in patients with anti‐MDA5 antibody‐positive dermatomyositis associated with interstitial lung disease

Serum KL‐6 level is a prognostic marker in patients with anti‐MDA5 antibody‐positive dermatomyositis associated with interstitial lung disease

Lung ultrasound B-lines and serum KL-6 correlate with the severity of idiopathic inflammatory myositis-associated interstitial lung disease

The role of lung ultrasound B-lines and serum KL-6 in the screening and follow-up of rheumatoid arthritis patients for an identification of interstitial lung disease: review of the literature, proposal for a preliminary algorithm, and clinical application to cases

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