scholarly journals Interpretation of autoantibody positivity in interstitial lung disease and lung-dominant connective tissue disease

2013 ◽  
Vol 39 (6) ◽  
pp. 728-741 ◽  
Author(s):  
Daniel Antunes Silva Pereira ◽  
Alexandre de Melo Kawassaki ◽  
Bruno Guedes Baldi
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Current Knowledge ◽  
Pulmonary Involvement ◽  
Pathophysiological Role ◽  
History Of

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

scholarly journals The role of biologics in treatment of connective tissue disease-associated interstitial lung disease

QJM ◽  
2015 ◽  
Vol 108 (9) ◽  
pp. 683-688 ◽  
Author(s):  
C. Sharp ◽  
N. Dodds ◽  
L. Mayers ◽  
A.B. Millar ◽  
H. Gunawardena ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease

scholarly journals Cardiovascular Involvement in Connective Tissue Disease: The Role of Interstitial Lung Disease

PLoS ONE ◽  
2015 ◽  
Vol 10 (3) ◽  
pp. e0121976
Author(s):  
XiaoBing Wang ◽  
MeiNa Lou ◽  
Yongji Li ◽  
WenJing Ye ◽  
ZhiYong Zhang ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Cardiovascular Involvement

scholarly journals Interstitial lung disease and gastroesophageal reflux disease: key role of esophageal function tests in the diagnosis and treatment

2011 ◽  
Vol 48 (2) ◽  
pp. 91-97 ◽  
Author(s):  
Renato Vianna Soares ◽  
Anne Forsythe ◽  
Kyle Hogarth ◽  
Nadera J. Sweiss ◽  
Imre Noth ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Gastroesophageal Reflux Disease ◽  
Connective Tissue ◽  
Gastroesophageal Reflux ◽  
Lung Disease ◽  
Esophageal Function ◽  
Respiratory Disorders ◽  
Function Tests ◽  
History Of

CONTEXT: Gastroesophageal reflux disease (GERD) is common in patients with respiratory disorders and interstitial lung fibrosis from diverse disease processes. However, a cause-effect relationship has not been well demonstrated. It is hypothesized that there might be more than a coincidental association between GERD and interstitial lung damage. There is still confusion about the diagnostic steps necessary to confirm the presence of GERD, and about the role of effective control of GERD in the natural history of these respiratory disorders. OBJECTIVES: To determine the prevalence of GERD in patients with respiratory disorders and lung involvement; the sensitivity of symptoms in the diagnosis of GERD; and the role of esophageal function tests (manometry and 24- hour pH monitoring) in the diagnosis and treatment of these patients. METHODS: Prospective study based on a database of 44 patients (29 females) with respiratory disorders: 16 patients had idiopathic pulmonary fibrosis, 11 patients had systemic sclerosis associated interstitial lung disease, 2 patients had polymyositis associated interstitial lung disease, 2 patients had Sjögren associated interstitial lung disease, 2 patients had rheumatoid artrithis associated interstitial lung disease, 1 patient had undifferentiated connective tissue diseases associated interstitial lung disease and 10 patients had sarcoidosis. The average forced vital capacity (% predicted) was 64.3%. All patients had esophageal function tests. RESULTS: Thirty patients (68%) had pathologic reflux (average DeMeester score: 45, normal <14.7). The average number of reflux episodes recorded 20 cm above the lower esophageal sphincter was 24. Sensitivity and specificity of heartburn were 70% and 57%, of regurgitation 43% and 57%, and of dysphagia 33% and 64%. Twelve patients with GERD underwent a laparoscopic fundoplication which was tailored to the manometric profile: three patients in which peristalsis was normal had a total fundoplication (360°) and nine patients in which the peristalsis was absent had a partial anterior fundoplication (180°). CONCLUSIONS: The results of our study show that: (a) abnormal reflux was present in about 2/3 of patients with respiratory disorders (idiophatic pulmonary fibrosis, connective tissue disorders and sarcoidosis), and it extended to the upper esophagus in most patients; (b) the sensitivity and specificity of reflux symptoms was very low; and (c) esophageal function tests were essential to establish the diagnosis of abnormal reflux, to characterize the esophageal function and guide therapy. Long term follow-up will be necessary to determine if control of reflux alters the natural history of these respiratory disorders.

scholarly journals AB0026 DECREASE OF ANGIOGENIC T CELLS IN CONNECTIVE TISSUE DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1046.3-1047
Author(s):  
V. Pulito-Cueto ◽  
S. Remuzgo Martinez ◽  
F. Genre ◽  
B. Atienza-Mateo ◽  
V. M. Mora-Cuesta ◽  
...  
Keyword(s):  
T Cells ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Cell Subset ◽  
Vascular Damage ◽  
Type I ◽  
Research Support ◽  
Angiogenic T Cells

Background:Interstitial lung disease (ILD) is one of the most significant complications of connective tissue diseases (CTD), leading to an increase of the morbidity and mortality in patients with CTD [1]. A specific T cell subset termed angiogenic T cells (TAng), that promote endothelial repair and revascularization, have been involved in the pathogenesis of CTD [2-4]. However, to the best of our knowledge, no information regarding the role of TAng in CTD-ILD+ is available.Objectives:To study, for the first time, the potential role of TAng related to vascular damage in CTD-ILD+.Methods:Peripheral venous blood was collected from 40 patients with CTD-ILD+ and three comparative groups: 44 CTD-ILD- patients, 21 idiopathic pulmonary fibrosis (IPF) patients and 20 healthy controls (HC). All subjects were recruited from the Rheumatology and Pneumology departments of Hospital Universitario Marqués de Valdecilla, Santander, Spain. Quantification of TAng was performed by flow cytometry. TAng were considered as triple-positive for CD3, CD31 and CXCR4.Results:Patients with CTD-ILD+ exhibited a significantly lower TAng frequency than CTD-ILD- patients (p<0.001). Similar results were obtained when patients with CTD-ILD+ were compared with HC (p=0.004) although no difference was observed between CTD-ILD+ and IPF. In addition, a significant increase of TAng frequency was shown in patients with CTD-ILD- in relation to IPF patients (p<0.001), while no difference was observed between CTD-ILD- and HC.Conclusion:Our results reveal a decrease of TAng frequency related to vascular damage in CTD-ILD+. Furthermore, we disclose that the presence of ILD is associated with lower TAng frequency.References:[1]Expert Rev Clin Immunol 2018;14(1):69-82.[2]Circulation 2007;116(15):1671-82.[3]Ann Rheum Dis 2015 74(5):921-7.[4]PLoS One 2017;12(8):e0183102.Acknowledgements:Personal funds, VP-C: PREVAL18/01 (IDIVAL); SR-M: RD16/0012/0009 (ISCIII-ERDF); LL-G: INNVAL20/06 (IDIVAL); RP-F: START PROJECT (FOREUM); RL-M: Miguel Servet type I CP16/00033 (ISCIII-ESF).Disclosure of Interests:Verónica Pulito-Cueto: None declared, Sara Remuzgo Martinez: None declared, Fernanda Genre: None declared, Belén Atienza-Mateo: None declared, Victor Manuel Mora-Cuesta: None declared, David Iturbe-Fernández: None declared, Leticia Lera-Gómez: None declared, Raquel Pérez-Fernández: None declared, Pilar Alonso Lecue: None declared, Javier Rodriguez Carrio: None declared, Diana Prieto-Peña: None declared, Virginia Portilla: None declared, Ricardo Blanco Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Grant/research support from: Abbvie, MSD and Roche, Alfonso Corrales: None declared, Jose Manuel Cifrián-Martínez: None declared, Raquel López-Mejías: None declared, Miguel A González-Gay Speakers bureau: Pfizer, Abbvie, MSD, Grant/research support from: Pfizer, Abbvie, MSD

scholarly journals Management of Myositis-Associated Interstitial Lung Disease

Medicina ◽  
2021 ◽  
Vol 57 (4) ◽  
pp. 347
Author(s):  
Tomoyuki Fujisawa
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Current Knowledge ◽  
Strong Predictor ◽  
Calcineurin Inhibitors ◽  
Pulmonary Involvement ◽  
Trna Synthetase ◽  
Evidence Based Guidelines ◽  
Substantial Heterogeneity

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.

Sign in / Sign up

Export Citation Format

Share Document