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Journal ArticleDOI

Analysis of risk factors of interstitial lung disease and mortality rates in Chinese patients with idiopathic inflammatory myopathy

24 May 2021-International Journal of Rheumatic Diseases (John Wiley & Sons, Ltd)-Vol. 24, Iss: 6, pp 815-827
TL;DR: In this article, the authors investigated the risk factors for interstitial lung disease (ILD) and prognosis in patients with idiopathic inflammatory myopathy (IIM) and found that older age at disease onset, ILD, malignancy, and increased serum albumin level were independent predictors for mortality.
Abstract: Aim To investigate the risk factors for interstitial lung disease (ILD) and prognosis in patients with idiopathic inflammatory myopathy (IIM) Methods A retrospective longitudinal study was performed in patients diagnosed with IIM between January 2012 and December 2018 Results The study cohort included 91 men and 195 women who were classified as having dermatomyositis (DM, n = 183), polymyositis (PM, n = 77), or clinical amyopathic DM (CADM, n = 26) ILD was identified in 465% (n = 133) of patients with IIM The independent risk factors for ILD were age at disease onset, presence of anti-Ro-52 antibody, Gottron's papules, elevated serum immunoglobulin M levels and hypoalbuminemia Older age at disease onset, ILD, malignancy, and increased serum aspartate aminotransferase and neutrophil-to-lymphocyte ratio (NLR) were identified as the independent predictors for mortality, whereas elevated serum albumin level was associated with a better prognosis A total of 73 deaths (255%) occurred after a median follow-up time of 33 months Infection (493%) was the leading cause of death In the overall cohort, the 1-year, 5-year and cumulative survival rates were 832%, 742% and 694%, respectively The receiver operating characteristic curve indicated that the optimal cut-off value of NLR for predicting death in IIM was 611 Conclusion IIM patients have a poor prognosis with substantial mortality, especially in patients who have older age at onset, ILD, malignancy and higher NLR Close monitoring and aggressive therapies are required in patients having poor predictive factors
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Journal ArticleDOI
TL;DR: Myocardial involvement is not rare and is an independent poor prognostic factor of anti-MDA5 Ab+ DM/CADM patients, and cardiac abnormality screening is necessary for them.
Abstract: Objectives Studies concerning myocardial involvement (MI) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis (anti-MDA5 Ab+ DM/CADM) are scarce. We aimed to characterize MI in our anti-MDA5 Ab+ DM/CADM cohort and to investigate its association with prognosis. Methods In this single-center retrospective study, anti-MDA5 Ab+ hospitalized DM/CADM patients who underwent transthoracic echocardiography (TTE) were enrolled. Myocardial involvement was diagnosed according to abnormal cardiac structure and function detected by TEE. Clinical features and cardiac examination findings of patients with MI were analyzed. Clinical features, laboratory findings, complications, and treatments were compared between MI and non-MI, deceased, and survival patients. Logistic regression analysis was used to explore the independent risk factors for the occurrence of MI and prognostic factors for these patients. Results Seventy-six hospitalized patients with anti-MDA5 Ab+ DM/CADM were enrolled. Twelve (15.8%) patients were diagnosed with MI. Of the 12 patients, three underwent cardiac magnetic resonance imaging (CMR) and late gadolinium enhancement (LGE) were noted for them. TEE revealed that eight (66.7%) patients had left atrial and/or ventricular enlargement, three (25.0%) had cardiac hypertrophy, six (50.0%) had diffuse ventricular wall dyskinesia, and seven (58.3%) had diastolic dysfunction. Six (50.0%) patients with MI developed heart failure (HF) during treatment. Of the 12 patients, one patient died of HF caused by myocarditis, three died of infection, and four died of exacerbation of rapidly progressive interstitial lung disease (RP-ILD). Logistic regression analysis revealed that dysphagia (OR 3.923, 95% CI 1.085, 14.181), NT-proBNP >600 pg/ml (OR 18.333, 95% CI 1.508, 222.875), and increased peripheral white blood cells (OR 1.201, 95% CI 1.003, 1.438) were risk factors for the occurrence of MI, but plasma albumin (OR 0.892, 95% CI 0.796, 0.999) was a protective factor. Both MI (OR 5.984, 95% CI 1.174, 30.496) and RP-ILD (OR 11.875, 95% CI 2.796, 50.411) were independent risk factors for the mortality of these anti-MDA5 Ab+ DM/CADM patients. Conclusion Myocardial involvement is not rare and is an independent poor prognostic factor of anti-MDA5 Ab+ DM/CADM patients. Cardiac abnormality screening is necessary for them.

3 citations

Journal ArticleDOI
TL;DR: A separate group of IIM related to paramalignant phenomenon has been distinguished, known as cancer-associated myositis (CAM), and the evidence supporting the contribution of genetics to CAM will be discussed.
Abstract: Idiopathic inflammatory myopathies (IIMs) comprise a heterogeneous group of rare immune‐mediated disorders that primarily affect muscles but also lead to dysfunction in other organs. Five different clinical subphenotypes of IIM have been distinguished: dermatomyositis, polymyositis, inclusion body myositis, antisynthetase syndrome, and immune‐mediated necrotizing myopathy. Excess mortality and morbidity associated with IIM are largely attributed to comorbidities, particularly cancer. The risk of malignancy is not equally distributed among IIM groups and is particularly high among patients with dermatomyositis. The cancer risk peaks around 3 years on either side of the IIM diagnosis and remains elevated even 10 years after the onset of the disease. Lung, colorectal, and ovarian neoplasms typically arise before the onset of IIM, whereas melanoma, cervical, oropharyngeal, and nonmelanoma skin cancers usually develop after IIM diagnosis. Given the close temporal proximity between IIM diagnosis and the emergence of malignancy, it has been proposed that IIM could be a consequence rather than a cause of cancer, a process known as a paramalignant phenomenon. Thus, a separate group of IIMs related to paramalignant phenomenon has been distinguished, known as cancer‐associated myositis (CAM). Although the relationship between IIM and cancer is widely recognized, the pathophysiology of CAM remains elusive. Given that genetic factors play a role in the development of IIM, dissection of the molecular mechanisms shared between IIM and cancer presents an opportunity to examine the role of autoimmunity in cancer development and progression. In this review, the evidence supporting the contribution of genetics to CAM will be discussed.

2 citations

Journal ArticleDOI
TL;DR: Wang et al. as discussed by the authors used the logistic regression models to determine the association between the variables and DBS infection, and established a nomogram including BMI, blood glucose, and albumin, which were significant predictors of infection in patients with DBS surgery.
Abstract: Infection is one of the important and frequent complications following implantable pulse generator and deep brain stimulation (DBS) electrode insertion. The goal of this study was to retrospectively evaluate and identify potential risk factors for DBS infections.From January 2015 to January 2021 in Qingdao municipal hospital (training cohort) and The First Affiliated Hospital of the University of Science and Technology of China (validation cohort), the authors enrolled patients with Parkinson disease who had undergone primary DBS placement or implantable pulse generator replacement. The cases were divided into infection or no-infection groups according to the 6-month follow-up. The authors used the logistic regression models to determine the association between the variables and DBS infection. Depending on the results of logistic regression, the authors established a nomogram. The calibration curves, receiver operating characteristic curve analysis, and decision curves were used to evaluate the reliability of the nomogram.There were 191 cases enrolled in the no-infection group and 20 cases in the infection group in the training cohort. The univariate logistic regression showed that BMI, blood glucose, and albumin were all significant predictors of infection after DBS surgery (OR 0.832 [p = 0.009], OR 1.735 [p < 0.001], and OR 0.823 [p = 0.001], respectively). In the crude, adjust I, and adjust II models, the three variables stated above were all considered to be significant predictors of infection after DBS surgery. The calibration curves in both training and validation cohorts showed that the predicted outcome fitted well to the observed outcome (p > 0.05). The decision curves showed that the nomogram had more benefits than the "All or None" scheme. The areas under the curve were 0.93 and 0.83 in the training and validation cohorts, respectively.The nomogram included BMI, blood glucose, and albumin, which were significant predictors of infection in patients with DBS surgery. The nomogram was reliable for clinical application.

1 citations

Journal ArticleDOI
TL;DR:
Abstract: BACKGROUND Pneumocystis jiroveci pneumonia (PJP) is a serious opportunistic infection that occurs mostly in patients with immunodeficiency and long-term immunosuppressive therapy. In non-human immunodeficiency virus-infected patients, the most important risk factor for PJP is the use of glucocorticoids in combination with other immunosuppressive treatments. The management of glucocorticoids during the perioperative period in patients with dermatomyositis requires special care. CASE SUMMARY We report a case of PJP in the perioperative period. A 61-year-old woman with a history of anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis and interstitial pneumonia was administered with long-term oral methylprednisolone and cyclosporine. The patient underwent right total hip arthroplasty in the orthopaedic department for bilateral osteonecrosis of the femoral head. She was given intravenous drip hydrocortisone before anesthesia and on the first day after surgery and resumed oral methylprednisolone on the second postoperative day. On the fifth day after surgery, the patient suddenly developed dyspnea. The computed tomography scan showed diffuse grid shadows and ground glass shadows in both lungs. Polymerase chain reaction testing of bronchoalveolar lavage fluid was positive for Pneumocystis jiroveci. The patient was eventually diagnosed with PJP and was administered with oral trimethoprim-sulfamethoxazole. At the 6-mo review, there was no recurrence or progression. CONCLUSION Continued perioperative glucocorticoid use in patients with anti-MDA5-positive dermatomyositis may increase the risk of PJP.

1 citations

Journal ArticleDOI
TL;DR: In this paper , the authors investigated the risk factors and the predictive value of multiple risk factors for progressive pulmonary fibrosis (PPF) in patients with ILD on high-resolution computed tomography (HRCT).
Abstract: Abstract Objective Interstitial lung disease (ILD) is a common extramuscular manifestation of the anti-synthetase syndrome (ASS). Patients with ASS-ILD are at risk in developing a progressive fibrosing phenotype despite appropriate treatments. This study investigated the risk factors and the predictive value of multiple risk factors for progressive pulmonary fibrosis (PPF) in patients with ASS-ILD. Methods Ninety patients with a diagnosis of ASS and evidence of ILD on high-resolution computed tomography (HRCT) were recruited. Among them, 72 participants completed follow-up for more than 12 months. These patients were further divided into a PPF-ASS group ( n = 18) and a non-PPF-ASS group ( n = 54). Logistic regression analysis was performed to investigate the risk factors for PPF. The predictive value of the combined risk factors for predicting PPF were analyzed by a ROC curve. Results The PPF-ASS group had a higher rate of positive non-Jo-1 antibodies, a significantly higher neutrophil-to-lymphocyte ratio (NLR) and serum lactate dehydrogenase (LDH), and a significantly lower PaO 2 /FiO 2 ratio and diffusing capacity for carbon monoxide (DLCO%pred) than the non-PPF-ASS group. In addition, elevated serum Krebs von den Lungen-6 (KL-6) level and reticular opacities were significantly more common, and corticosteroid monotherapy at onset was administered more frequently in the PPF-ASS group. The median duration of follow-up was 37.4 months, survival was poorer in the PPF-ASS group, and the overall survival was 88.9%. Multivariate regression analysis further revealed that positive non-Jo-1 antibodies, NLR, and KL-6 were independent risk factors for PPF. These combined indexes had good accuracy (area under the curve = 0.874) in predicting PPF in patients with ASS-ILD. Conclusion Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors for PPF in patients with ASS-ILD. Monitoring these markers can potentially predict PPF in this group of patients. Key Points • Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors associated with PPF in patients with ASS-ILD. • Monitoring non-Jo-1 antibodies, NLR, and serum KL-6 can potentially predict PPF in patients with ASS-ILD.

1 citations

References
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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

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William D. Travis, Talmadge E. King, Eric D. Bateman, David A. Lynch, Frédrique Capron, Thomas V. Colby, Jean-François Cordier, Roland M. Dubois, Jeffrey R. Galvin, Philippe Grenier, David M. Hansell, Gary W. Hunninghake, Masanori Kitaichi, Nestor L. Müller, Jeffrey L. Myers, Sonoko Nagai, Andrew G. Nicholson, Ganesh Raghu, Benoit Wallaert, Christian Brambilla, Kevin K. Brown, Andrew L. Cherniaev, Ulrich Costabel, David B. Coultas, Gerald S. Davis, Maurits G. Demedts, William W. Douglas, Jim J. Egan, Anders Eklund, Leonarda M. Fabbri, Craig A. Henke, Richard Hubbard, Y. Inoue, Takateru Izumi, H. M. Jansen, Ian Johnston, Dong Soon Kim, Nasreen Khalil, Fiona R. Lake, Giuseppe Lungarella, Joseph P. Lynch, Douglas W. Mapel, Fernando J. Martinez, Richard A. Matthay, Lee S. Newman, Paul W. Noble, Ken Ohta, Dario Olivieri, Luis A. Ortiz, Venerino Poletti, Robert Rodriguez-Roisin, William N. Rom, Jay Hoon Ryu, Paulo Hilário Nascimento Saldiva, Raúl H Sansores, Marvin L. Schwarz, Moisés Selman, Cecelia M. Smith, Zhaohui Tong, Zarir F Udwadia, Dominique Valeyre, Athol U. Wells, Robert A. Wise, Antonio Xaubet, Emilio Alvarez Fernandez, Elisabeth Brambilla, Vera Luiza Capelozzi, Andrew Cherniaev, Peter Dalquen, Gerhard Dekan, Philip S. Hasleton, James C. Hogg, N. A. Jambhekar, Anna Luise A Katzenstein, Michael Koss, Osamu Matsubara, Klaus Michael Müller, F. B.J.M. Thunnissen, James A. Waldron, Wei Hua Li, Paul J. Friedman, Martin Remy-Jardin, Theresa C. McLoud 
TL;DR: The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis.
Abstract: Executive Summary Objectives Participants Evidence Validation Key Messages Introduction Rationale for a Change in the Approach to Classification of Idiopathic Interstitial Pneumonias Development of a New Classification of Idiopathic Interstitial Pneumonia Current Classification of IIP New ATS/ERS Classification Principles Guiding the Assessment of Patients with Idiopathic Interstitial Pneumonias The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis Clinical Features Radiologic Features Histologic Features IPF: Areas of Uncertainty Nonspecific Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features NSIP: Areas of Uncertainty Cryptogenic Organizing Pneumonia Clinical Features Radiologic Features Histologic Features COP: Areas of Uncertainty Acute Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features AIP: Areas of Uncertainty Respiratory Bronchiolitis-Associated Interstitial Lung Disease Clinical Features Radiologic Features Histologic Features RB-ILD: Areas of Uncertainty Desquamative Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features DIP: Areas of Uncertainty Lymphoid Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features LIP: Areas of Uncertainty References Appendix

3,591 citations

Journal ArticleDOI
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

Journal ArticleDOI
TL;DR: In this paper, the combined effects of inflammation and inadequate protein and caloric intake in patients with chronic disease such as chronic renal failure were identified as the cause of hypoalbuminemia.
Abstract: Hypoalbuminemia is the result of the combined effects of inflammation and inadequate protein and caloric intake in patients with chronic disease such as chronic renal failure. Inflammation and malnutrition both reduce albumin concentration by decreasing its rate of synthesis, while inflammation alone is associated with a greater fractional catabolic rate (FCR) and, when extreme, increased transfer of albumin out of the vascular compartment. A vicious cascade of events ensues in which inflammation induces anorexia and reduces the effective use of dietary protein and energy intake and augments catabolism of the key somatic protein, albumin. Hypoalbuminemia is a powerful predictor of mortality in patients with chronic renal failure, and the major cause of death in this population is due to cardiovascular events. Inflammation is associated with vascular disease and likely causes injury to the vascular endothelium, and hypoalbuminemia as two separate expressions of the inflammatory process. Albumin has a myriad of important physiologic effects that are essential for normal health. However, simply administering albumin to critically ill patients with hypoalbuminemia has not been shown to improve survival or reduce morbidity. Thus the inference from these clinical studies suggests that the cause of hypoalbuminemia, rather than low albumin levels specifically, is responsible for morbidity and mortality.

884 citations

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