Clinical Heterogeneity of Interstitial Lung Disease in Polymyositis and Dermatomyositis Patients With or Without Specific Autoantibodies.
TL;DR: MDA5‐ILD was associated with severe pulmonary manifestations, poor response to treatment and aggravated prognosis, and the ARS‐ILD group had favorable treatment response and prognosis.
About: This article is published in The American Journal of the Medical Sciences.The article was published on 2018-01-01. It has received 30 citations till now. The article focuses on the topics: Dermatomyositis & Polymyositis.
Citations
More filters
TL;DR: The idiopathic inflammatory myopathies, including polymyositis and dermatomyositis, are autoimmune connective tissue diseases with variable degrees of muscle inflammation and systemic involvement and the addition of other immunosuppressive therapy is typically necessary to achieve disease control.
66 citations
TL;DR: It is hoped that greaterawareness of the clinical features of this syndrome will allow for appropriate diagnosis and treatment of these potentially treatable patients as well as raise awareness of the need for multicenter collaboration to prospectively study how to manage this complex disease.
35 citations
TL;DR: Molecules that could be used as biomarkers for diagnosis and monitoring dermatomyositis disease activity are reviewed.
Abstract: Dermatomyositis pathophysiology is complex. In recent years, medical research has identified molecules associated with disease activity. Besides providing insights into the driving mechanisms of dermatomyositis, these findings could provide potential biomarkers. Activity markers can be used to monitor disease activity in clinical trials and may also be useful in daily practice. This article reviews molecules that could be used as biomarkers for diagnosis and monitoring dermatomyositis disease activity.
23 citations
TL;DR: In this article, a review of the pharmacologic managements of polymyositis (PM), dermatomyositis, and clinically amyopathic DM-associated ILD (PM/DM/CADM-ILD) is presented.
Abstract: Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. The management of PM-/DM-/CADM-associated ILD (PM/DM/CADM-ILD) requires careful evaluation of the disease severity and clinical subtype, including the ILD forms (acute/subacute or chronic), because of the substantial heterogeneity of their clinical courses. Recent studies have highlighted the importance of myositis-specific autoantibodies’ status, especially anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl tRNA synthetase (ARS) antibodies, in order to evaluate the clinical phenotypes and treatment of choice for PM/DM/CADM-ILD. Because the presence of the anti-MDA5 antibody is a strong predictor of a worse prognosis, combination treatment with glucocorticoids (GCs) and calcineurin inhibitors (CNIs; tacrolimus (TAC) or cyclosporin A (CsA)) is recommended for patients with anti-MDA5 antibody-positive DM/CADM-ILD. Rapidly progressive DM/CADM-ILD with the anti-MDA5 antibody is the most intractable condition, which requires immediate combined immunosuppressive therapy with GCs, CNIs, and intravenous cyclophosphamide. Additional salvage therapies (rituximab, tofacitinib, and plasma exchange) should be considered for patients with refractory ILD. Patients with anti-ARS antibody-positive ILD respond better to GC treatment, but with frequent recurrence; thus, GCs plus immunosuppressants (TAC, CsA, azathioprine, and mycophenolate mofetil) are often needed in order to achieve favorable long-term disease control. PM/DM/CADM-ILD management is still a therapeutic challenge for clinicians, as evidence-based guidelines do not exist to help with management decisions. A few prospective clinical trials have been recently reported regarding the treatment of PM/DM/CADM-ILD. Here, the current knowledge on the pharmacologic managements of PM/DM/CADM-ILD was mainly reviewed.
23 citations
References
More filters
TL;DR: (First of Two Parts)
Abstract: Laboratory Features Elevation of sarcoplasmic enzymes in serum (creatine phosphokinase, aldolase, transaminases and lactic dehydrogenase) is valuable both for diagnosis and for following the clinic...
4,394 citations
"Clinical Heterogeneity of Interstit..." refers methods in this paper
...The occurrence of PM (n = 26) and DM (n = 146) was diagnosed according to the Bohan and Peter criteria.(11) Patients diagnosed with clinical amyopathic DM (n 1⁄4 10) according to the Sontheimer criteria were also included....
[...]
Woolcock Institute of Medical Research1, University of Otago2, University of Cape Town3, Boston University4, University of California, San Francisco5, University of Wisconsin-Madison6, Creighton University7, University of Arizona8, University of Newcastle9, Erasmus University Rotterdam10, University of Groningen11, University of Edinburgh12, McMaster University13, Imperial College London14, University of Leicester15, University of Amsterdam16, University of Nevada, Reno17, University of Washington18, University of Aberdeen19, University of Pittsburgh20
TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
Abstract: Background: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical “gold standard” of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs.Purpose: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs.Methods: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011.Results: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis–interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific inte...
2,931 citations
"Clinical Heterogeneity of Interstit..." refers background or methods in this paper
...CT-scan patterns were classified as UIP, NSIP and OP by 2 experienced radiologists according to 2013 American Thoracic Society (ATS) and European Respiratory Society (ERS) policies.(13) Typical HRCT features of UIP included a reticular pattern, traction bronchiectasis and honeycombing appearance predominantly located in the subpleural region and in the lower lobe....
[...]
...RP-ILD was defined as worsening of radiologic interstitial changes with symptoms of progressive dyspnea and hypoxemia that occurred within 3 months of the onset of respiratory issues.(13,14)...
[...]
TL;DR: The Master said, "If names are not correct, language is not in accordance with the truth of things, affairs cannot be carried out to success, therefore a superior man considers it necessary that the names he uses be spoken appropriately, what the superior man requires, is just that in his words there may be nothing incorrect as mentioned in this paper.
Abstract: The Master said,… If names are not correct, language is not in accordance with the truth of things. If language is not in accordance with the truth of things, affairs cannot be carried out to success.... Therefore a superior man considers it necessary that the names he uses be spoken appropriately., What the superior man requires, is just that in his words there may be nothing incorrect.
384 citations
TL;DR: Both serum ferritin and anti-MDA5 antibody are powerful indicators for the early diagnosis of A/SIP with DM and Ferritin also predicts disease severity and prognosis for patients with anti-mda5 antibody.
Abstract: Objective. The aim of this study is to evaluate the clinical manifestation and prognostic factors of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-associated interstitial lung disease (ILD) with DM. Methods. Fourteen patients who presented with anti-MDA5 antibody and 10 patients with anti-aminoacyl-tRNA synthetase (ARS) antibody were enrolled. All patients were diagnosed as having DM with ILD. Clinical manifestations in the patients with anti-MDA5 antibody were compared with those in the patients with anti-ARS antibody. Results. The frequencies of acute/subacute interstitial pneumonia (A/SIP) and fatal outcome were significantly higher in the subset with anti-MDA5 antibody. The creatine kinase (CK) value was significantly lower and the γ-glutamyl transpeptidase and ferritin values were significantly higher in the subset with anti-MDA5 antibody. Significant correlations were found between PaO 2 /F i O 2 and ferritin (r s =-0.59, P=0.035), alveolar-arterial oxygen difference (A-aDO 2 ) and KL-6 (r s =0.73, P=0.016) and A-aDO 2 and ferritin (r s =0.66, P=0.013) in the subset with anti-MDA5 antibody. The most significant prognostic factor was ferritin. The cumulative survival rate was significantly lower (P < 0.0001) in the subset with ferritin ≥ 1600 ng/ml than that in the subset with ferritin <1600 ng/ml in anti-MDA5 antibody-associated ILD. Conclusion. Both serum ferritin and anti-MDA5 antibody are powerful indicators for the early diagnosis of A/SIP with DM. Ferritin also predicts disease severity and prognosis for patients with anti-MDA5 antibody. Intensive treatment should be administered to cases that have anti-MDA5 antibody-associated ILD with DM showing hyperferritinaemia, especially if the ferritin level is ≥ 1600 ng/ml.
241 citations
TL;DR: Anti-MDA5 and anti-TIF1-gamma antibodies were detected based on their ability to immunoprecipitate biotinylated recombinant MDA5, and were closely associated with life-threatening complications in DM.
Abstract: Objectives. Myositis-specific autoantibodies are useful for diagnosing PM/DM. Recently, two new myositis-specific autoantibodies against melanoma differentiation-associated gene 5 (MDA5) and transcriptional intermediary factor 1-g (TIF1-g) were identified in DM. Here, we detected these autoantibodies in patient sera using new assays with recombinant MDA5 and TIF1-g, and associated clinical features with the presence of anti-MDA5 or anti-TIF1-g antibodies. Methods. We screened 135 Japanese patients with various CTDs, including 82 with DM. DM patients were classified as clinically amyopathic DM (CADM), cancer-associated DM or classical DM without cancer. Anti-MDA5 and anti-TIF1-g antibodies were detected by their ability to immunoprecipitate biotinylated recombinant proteins. Results. Sera from 21 (26%) of 82 DM patients immunoprecipitated MDA5, and every anti-MDA5-positive patient had DM (except one patient with SSc). Sera from 20 (65%) of 31 CADM patients reacted with MDA5. Notably, anti-MDA5-positive DM patients had significantly more interstitial lung disease than anti-MDA5-negative DM patients (95 vs 32%, P < 0.001). Sera from 12 (15%) of 82 DM patients immunoprecipitated TIF1-g, and anti-TIF1-g antibodies were only detected in DM patients. Strikingly, 7 (58%) of 12 patients with cancer-associated DM had sera that reacted with TIF1-g. Anti-TIF1-g-positive DM patients had significantly more internal malignancies than anti-TIF1-g-negative DM patients (58 vs 9%, P < 0.001). Conclusions. Anti-MDA5 and anti-TIF1-g antibodies were confirmed to be serological DM subset markers. Anti-MDA5 and anti-TIF1-g antibodies were detected based on their ability to immunoprecipitate biotinylated recombinant MDA5 and TIF1-g, and were closely associated with life-threatening complications in DM.
232 citations
"Clinical Heterogeneity of Interstit..." refers background in this paper
...These finding are in line with those from Hoshino et al,4,20 who demonstrated that the presence of anti-MDA5 antibodies was correlated with RP-ILD....
[...]
...Therefore, further research could explore whether other ILD-related autoantibodies exist in the patients with MSN-ILD....
[...]
...Patients with MSN-ILD have mild pulmonary manifestation and less frequency of RPILD; nevertheless, no difference in prognosis was observed compared to patients with ARS-ILD....
[...]
...The antiaminoacyl-tRNA synthetase (ARS) and antimelanoma differentiation-associated gene 5 (MDA5) antibodies are 2 kinds of myositis-specific autoantibodies (MSA) associated with ILD.(4,5) Distinct clinical features and prognoses of PM/DM-ILD with anti-MDA5 or anti-ARS antibodies have been reported in several different studies....
[...]
...Certain studies from Japan26,27 have stated that bronchoalveolar lavage fluid from patients with RP-ILD contained higher numbers of lymphocytes compared to that of patients with chronic ILD....
[...]