Low positive titer of anti-melanoma differentiation-associated gene 5 antibody is not associated with a poor long-term outcome of interstitial lung disease in patients with dermatomyositis

Sakamoto, Satoshi; Okamoto, Masaki; Kaieda, Shinjiro; Fujimoto, Kiminori; Nagata, Shuji; Tominaga, Masaki; Nakamura, Masayuki; Zaizen, Yoshiaki; Nouno, Takashi; Koga, Tetsuro; Kawayama, Tomotaka; Kuwana, Masataka; Ida, Hiroaki; Hoshino, Tomoaki

doi:10.1016/j.resinv.2018.07.007

Cited by 27 publications

(23 citation statements)

References 19 publications

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“…Chest HRCT scans of the patients were evaluated by trained pulmonologists (KT and TH) in a blinded and independent manner, in accordance with a previously reported classification . First, the pulmonologists classified chest HRCT results into the following patterns: usual interstitial pneumonia (UIP), probable UIP, nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), NSIP with OP, or unclassifiable by chest HRCT.…”

Section: Methodsmentioning

confidence: 99%

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

Tsuji

Nakashima

Hosono

et al. 2020

Arthritis & Rheumatology

234

168

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Objective Interstitial lung disease (ILD) accompanied by anti–melanoma differentiation–associated gene 5 (anti–MDA‐5)–positive dermatomyositis (DM) is often rapidly progressive and associated with poor prognosis. Because there is no established treatment, we undertook this study to prospectively evaluate the efficacy and safety of a combined immunosuppressive regimen for anti–MDA‐5–positive DM patients with ILD. Methods Adult Japanese patients with new‐onset anti–MDA‐5–positive DM with ILD (n = 29) were enrolled at multiple study centers from 2014 to 2017. They were treated with a regimen of high‐dose glucocorticoids (GCs), tacrolimus, and intravenous cyclophosphamide (IV CYC). Plasmapheresis was used if a patient's condition worsened after the regimen started. The primary end point was 6‐month survival, which was compared between this group of patients and a historical control group (n = 15) consisting of anti–MDA‐5–positive DM patients with ILD who received step‐up treatment (high‐dose GC and stepwise addition of immunosuppressant). Secondary end points were 12‐month survival rate, adverse events, and changes in laboratory data. Results The combined immunosuppressive regimen group showed significantly higher 6‐month survival rates than the step‐up treatment group (89% versus 33%; P < 0.0001). Over a period of 52 weeks, improvements in anti–MDA‐5 titers, serum ferritin levels, vital capacity, and chest high‐resolution computed tomography scores were observed. The combined immunosuppressive regimen group received IV CYC nearly 20 days earlier with shorter intervals and tended to receive plasmapheresis more often than patients undergoing step‐up treatment. Cytomegalovirus reactivation was frequently observed over 52 weeks. Conclusion A combined immunosuppressive regimen is effective for anti–MDA‐5–positive DM patients with ILD. Plasmapheresis can be used for additional effect in intractable disease. Patients should be carefully monitored for opportunistic infections during treatment.

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Section: Methodsmentioning

confidence: 99%

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

Tsuji

Nakashima

Hosono

et al. 2020

Arthritis & Rheumatology

234

168

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“…Indeed, our results indicate that even perilobular opacity itself is a sign of acute-phase DAD. Furthermore, some previous reports described the early prediction of lethal RP-ILD with MDA-5 antibody in patients with findings such as palmer papule, nail fold bleeding, and skin ulcers, which were present in almost all of our patients (16, 22-24). Given the present and previous findings, the status of MDA-5 antibody and the presence of a specific skin rash should be immediately evaluated in order to initiate early aggressive treatment including multiple immunosuppressive agents in RP-ILD patients with rapidly progressive perilobular opacity.…”

Section: Discussionmentioning

confidence: 54%

“…A few papers have described the radiological characteristics of RP-ILD patients with anti-MDA5 antibody. In those papers, the most common HRCT pattern was “unclassified” followed by “NSIP with OP” (15, 16), and neither intralobular reticular opacity nor lower reticulation was observed (17). Generally, perilobular opacity, which was observed in 6 of the 8 patients (75%) in our study, is reported in about a half of patients with cryptogenic organizing pneumonia (11-14).…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2019

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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.

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“…Hoa et al 17 found in a series of MDA5 (+) RP-ILD associated DM, that levels of ferritin were in the range of 370-13,878 ng/ml (NV < 200 ng/ml). Blood values higher than 1,000 ng/ml, seem to be associated with higher mortality in Caucasians and Asian ethnicities [18][19][20][21] ; moreover, ferritin values run in parallel to the activity of the disease 22 . Beside the ferritin, Krebs von den Lungen-6 (KL-6), a type II pneumocyte glycoprotein has been postulated as a biomarker of ILD in different ethnicities 23,24 .…”

Section: Diagnostic Accuracy and Rationale Of The Different Questions Methods Of Anti-mda5 Detection And Brief Description Of The Differementioning

confidence: 99%

Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease

Romero-Bueno

Campo

Trallero-Araguás

et al. 2020

Seminars in Arthritis and Rheumatism

134

114

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Low positive titer of anti-melanoma differentiation-associated gene 5 antibody is not associated with a poor long-term outcome of interstitial lung disease in patients with dermatomyositis

Cited by 27 publications

References 19 publications

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

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

Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease

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