scholarly journals Up-to-Date Information on Rheumatoid Arthritis-Associated Interstitial Lung Disease

2015 ◽  
Vol 9s1 ◽  
pp. CCRPM.S23289 ◽  
Author(s):  
Takafumi Suda
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Cytotoxic Agents ◽  
Radiological Patterns

Pulmonary involvement is common in rheumatoid arthritis (RA) and affects all the components of the lung. Interstitial lung disease (ILD) is the most predominant pulmonary manifestation and has been identified as the main cause of morbidity and mortality in RA. Clinically significant RA-ILD occurs in approximately 10% of RA patients. Several risk factors, such as old age, male gender, and smoking, have been reported to date. Histologically, the proportion of the usual interstitial pneumonia (UIP) pattern is higher in RA-ILD than in ILD associated with other connective tissue diseases, and RA-ILD also shows nonspecific interstitial pneumonia and organizing pneumonia patterns. High-resolution computed tomography scans are highly predictive of the histological UIP pattern with a specificity of 96%-100%. Acute exacerbation, which is the acute deterioration of the respiratory status characterized by newly developed bilateral infiltrates with unknown etiologies, has been reported in RA-ILD. Although acute exacerbation of RA-ILD has high mortality, similar to that of idiopathic pulmonary fibrosis, its incidence is lower in RA-ILD than in idiopathic pulmonary fibrosis. A consensus treatment has not yet been established. Current therapeutic regimens typically include corticosteroids with or without cytotoxic agents. Recent large longitudinal studies reported that the prognosis of RA-ILD was poor with a median survival of 2.6-3.0 years. Furthermore, histological and/or radiological patterns, such as UIP or non-UIP, have significant prognostic implications. RA-ILD patients with histological or radiological UIP patterns have poorer prognoses than those with non-UIP patterns. This review assessed the characteristics of RA-ILD by overviewing recent studies in the field and focused on the clinical significance of histological and/or radiological patterns in RA-ILD.

scholarly journals SAT0098 TREATMENT OF A COHORT OF PATIENTS WITH INTERSTITIAL LUNG DISEASE AND RHEUMATOID ARTHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 982.2-982
Author(s):  
C. Aguilera Cros ◽  
M. Gomez Vargas ◽  
R. J. Gil Velez ◽  
J. A. Rodriguez Portal
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Latin American ◽  
Usual Interstitial Pneumonia ◽  
Specific Treatment ◽  
American Thoracic Society ◽  
Rapid Progression

Background:There is no specific treatment for interstitial lung disease (ILD) secondary to Rheumatoid Arthritis (RA) other than the treatment of RA without extra-articular involvement. Current regimens usually include corticosteroid therapy with or without immunosuppressants (IS), there is no consensus for the treatment.Objectives:To analyze the different treatment regimens in a cohort of patients with ILD and RA in our clinical practice.Methods:Descriptive study of 57 patients treated in our Hospital (1/1/2018 until 12/31/2019) with a diagnosis of RA (ACR 2010 criteria) and secondary ILD.The most recent American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Society (ALAT) guidelines define three HRCT (High Resolution Computed Tomography) patterns of fibrosing lung disease in the setting of idiopathic pulmonary fibrosis (IPF): definite Usual Interstitial pneumonia (UIP) (traction bronchiectasis and honeycombing), possible UIP and inconsistent with UIP. The distinction between definite UIP and possible UIP in these to the presence or absence of honeycombing. Approved by the Ethics Committee.Quantitative variables are expressed as mean (SD) and dichotomous variables as percentages (%). Statistical analysis with SPSS version 21.Results:21 men and 36 women were included, with a mean age of 69 ± 10 years (mean ± SD), history of smoking (smokers 14%, non-smokers 43%, former smokers 42%). Clinical ILD at diagnosis (dyspnea 61%, dry cough 56%, crackling 70%, acropachy 7%). 84% were positive rheumatoid factor and 70% positive anticitrullinated protein antibody.Diagnosis of ILD by HRCT in 100% of patients with different patterns: defined UIP 26 (45%), probable UIP 2 (3%) and not UIP 29 (50%). The diagnosis of ILD was confirmed by biopsy in 12 patients.79% underwent (T) treatment prior to the diagnosis of ILD with glucocorticoids and disease-modifying drugs (DMARD). Among the traditional DMARDs used were: Methotrexate 68% (there were no cases of MTX pneumonitis), Leflunomide 47%, Hydroxychloroquine 26% and Sulfasalazine 21%. Biological therapy in 15 patients: Etanercept 19%, Adalimumab 5%, Infliximab 3% and Certolizumab 2%. Two patients presented an exacerbation and rapid progression of the ILD during the T with Etanercept with the final result of death.T with IS after the diagnosis of ILD in 80% of patients (Azathioprine 15, Rituximab 14, Abatacept 10, Tocilizumab 4, Sarilumab 1, Mofetil mycophenolate 1 and Cyclophosphamide 1).Two patients with defined UIP perform T with antifibrotic: 1st Nintedanib (INBUILD Trial, This article was published on September 29, 2019, at NEJM.org) 2nd Pirfenidone (initial diagnosis of IPF Idiopathic Pulmonary Fibrosis and subsequent of seropositive RA with UIP). Both improved greater than 10% in forced vital capacity (FVC) and diffusion capacity of the lung for carbon monoxide (DLCO) in the 6 months after onset of T.Conclusion:Our results, in general, agree with what is published in the literature. Prospective, multicentre and larger sample studies are necessary to better define which patients would benefit more from IS T or antifibrotic T (or if the antifibrotic should be added to the previous IS).Disclosure of Interests:None declared

AB0528 CHARACTERIZATION OF ANTI-MPO POSITIVE INTERSTITIAL LUNG DISEASE. CLINICAL-SEROLOGIC AND RADIOLOGIC FEATURES AND SURVIVAL

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1561-1562
Author(s):  
G. Cassone ◽  
G. Dei ◽  
G. Sambataro ◽  
A. Manfredi ◽  
S. Cerri ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Pulmonary Involvement ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Anca Associated Vasculitis ◽  
Radiologic Features

Background:Prevalence of anti-neutrophil cytoplasmic antibody (ANCA) in patient with idiopathic pulmonary fibrosis (IPF) ranges from 1 to 35%, mainly anti-MPO. The presence of ANCA positivity seems to be a poorer prognostic factor in patient with IPF, and some of these patients will develop clinical vasculitis (7-23%).Unfortunately, the majority of the available studies on this topic are retrospective and the real natural history of the disease remains poorly understood.Objectives:Aim of the study was to investigate the clinical, serological and radiologic features of patients with interstitial lung disease (ILD) and positivity for anti-MPO, and to evaluate the survival of this population compared with IPF patients.Methods:We retrospectively analysed 30 patients with ILD and anti-MPO antibodies, without diagnosis of vasculitis, from 3 different rheumatology-pulmonology Italian Center.For each patient, clinical, radiologic and serological data were evaluated. Treatments were also collected, both immunosuppressants or antifibrotic agents.Finally, survival of ILD-MPO patients and of 90 unselected idiopathic pulmonary fibrosis (IPF) patients was compared.Results:Thirty patients were enrolled in the study (see table for the characteristics of the patients).Fibrosing pneumonia was described in 73.3% of patients (usual interstitial pneumonia [UIP] in 19 patients), and 10 patients (33.3%) received antifibrotic drugs, all with UIP pattern. Of interest, 7 patients were treated with immunosuppressants (azathioprine, cyclophosphamide, mycophenolate mofetil), independently by the ILD pattern and 21 (70%) low dosage of steroids.After a median period of 23.5 months (range 11-111), 7 patients developed an ANCA associated vasculitis, while other 3 developed other rheumatic diseases.Finally, when compared with IPF, ILD-MPO patients had a better survival (81.2%±0.9 vs 54.7±0.7 for ILD-MPO and IPF, respectively; p=0.045)Conclusion:ILD positive for anti-MPO antibodies are still a not definite condition. We need larger population to identify possible markers for the evolution in an ANCA associated vasculitis, to define the prognosis of disease and the better therapeutic approach.References: :[1]Mohammad AJ, et al. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017;44:1458-67Table.Serological, clinical and radiological features of anti-MPO + interstitial lung diseaseNumber30Males/female15/15Median age (years + IQR)68 (17)Median follow-up (months + IQR)39.5 (61)Smoke36.70%ILD pattern Usual interstitial pneumonia63.30% Nonspecific interstitial pneumonia16.70% Hipersensitivity pneumonia10% Other fibrosing pneumonia10%Median FVC (% + IQR)83 (23)Median DLCO (% + IQR)53 (28)Clinical features Raynaud’s phenomenon7.70% Sicca syndrome0 Arthralgias20% Arthritis3.40%Serology Antinuclear antibodies30.80% Anti-extractable nuclear antibodies (ENA)8% Anti-SSA4% Rheumatoid factor21.40%Therapy Immusuppressants23.30% Anti-fibrotic drugs33.30%Disclosure of Interests:None declared

scholarly journals SAT0035 RESPONSE TO ABATACEPT OF DIFFERENT PATTERNS OF INTERSTITIAL LUNG DISEASE IN RHEUMATOID ARTHRITIS: NATIONAL MULTICENTER STUDY OF 263 PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 947.1-948
Author(s):  
C. Fernández-Díaz ◽  
S. Castañeda ◽  
R. Melero ◽  
J. Loricera ◽  
F. Ortiz-Sanjuán ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Multicenter Study ◽  
Standard Dose ◽  
Usual Interstitial Pneumonia ◽  
Research Support ◽  
Prednisone Dose ◽  
Radiological Patterns

Background:Interstitial Lung Disease (ILD) is a severe extraarticular manifestation of rheumatoid arthritis (RA). In this line, several radiological patterns of RA-ILD have been described: i) usual interstitial pneumonia (UIP), ii) nonspecific interstitial pneumonia (NSIP), iii) obliterating bronchiolitis, iv) organized pneumonia and mixed patterns. Abatacept (ABA) could be an effective and safe option for patients with RA-ILD, although the response in the different radiological patterns is not well defined.Objectives:Our aim was to assess the response to ABA in different radiological patterns of ILD.Methods:Observational retrospective multicenter study of RA-ILD treated with ABA. ILD was diagnosed by HRCT and classified by radiological patterns in 3 different subgroups of RA-ILD: a) UIP, b) NSIP and c) “other”. ABA was used sc. or iv. at standard dose. We assessed: a) Dyspnoea (MMRC scale; significant variation ≥1); b) Respiratory function tests (significant changes ≥10% in FVC and DLCO); c) HRCT imaging; d) DAS28 e)prednisone dose.Variables were collected at months 0, 3, 6, 12 months and subsequently every 12 months until a maximum of 60 months.Results:We included 263 patients: 106 UIP, 84 NSIP and 73 others (150 women / 113 men), mean age 64.64±10 years. Total patients positive for RF or CCPA were 235 (89.4%) and 233 (88.6%), respectively. In 26 out of 263 patients, the development of ILD was closely related to the administration of sDMARDs (MTX n = 11 and LFN n = 1) or bDMARDs (ETN n = 5, ADA n = 4, CZP n = 2 and IFX n = 3). Patient characteristics are shown in table 1. Figure 1 shows the evolution of the cases with available data after a mean follow-up of 22.7±19.7 months. Mean DLCO and FVC remained stable in the 3 groups without statistically significant changes, and all the groups showed a statistically significant reduction in DAS28 and prednisone dose.Conclusion:ABA could be a good choice of treatment in patients with RA-ILD independently of the radiological pattern of ILD.Disclosure of Interests:Carlos Fernández-Díaz Speakers bureau: Brystol Meyers Squibb, Santos Castañeda: None declared, Rafael Melero: None declared, J. Loricera: None declared, Francisco Ortiz-Sanjuán: None declared, A. Juan-Mas: None declared, Carmen Carrasco-Cubero Speakers bureau: Janssen, MSD, AbbVie, Novartis, Bristol Myers Squibb, and Celgene, S, Rodriguéz-Muguruza: None declared, S. Rodrigez -Garcia: None declared, R. Castellanos-Moreira: None declared, RAQUEL ALMODOVAR Speakers bureau: Abbvie, Celgene, Janssen, Lilly, Novartis, Pfizer., CLARA AGUILERA CROS: None declared, Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Sergi Ordoñez: None declared, Susana Romero-Yuste: None declared, C. Ojeda-Garcia: None declared, Manuel Moreno: None declared, Gemma Bonilla: None declared, I. Hernández-Rodriguez: None declared, Mireia Lopez Corbeto: None declared, José Luis Andréu Sánchez: None declared, Trinidad Pérez Sandoval: None declared, Alejandra López Robles: None declared, Patricia Carreira Grant/research support from: Actelion, Roche, MSD, Consultant of: GlaxoSmithKline, VivaCell Biotechnology, Emerald Health Pharmaceuticals, Boehringer Ingelheim, Roche, Speakers bureau: Actelion, GlaxoSmithKline, Roche, Natalia Mena-Vázquez: None declared, C. Peralta-Ginés: None declared, ANA URRUTICOECHEA-ARANA: None declared, Luis Marcelino Arboleya Rodríguez: None declared, J. Narváez: None declared, DESEADA PALMA SANCHEZ: None declared, Olga Maiz-Alonso: None declared, J. Fernández-Leroy: None declared, I. Cabezas-Rodriguez: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, A. Ruibal-Escribano: None declared, JR De Dios-Jiménez Aberásturi: None declared, Paloma Vela-Casasempere: None declared, C. González-Montagut Gómez: None declared, J M Blanco: None declared, Noelia Alvarez-Rivas: None declared, N. Del-Val: None declared, M. Rodíguez-Gómez: None declared, Eva Salgado-Pérez: None declared, Carlos Fernández-López: None declared, E.C. Cervantes Pérez: None declared, A. Devicente-DelMas: None declared, Blanca Garcia-Magallon Consultant of: MSD, Speakers bureau: Pfizer, Amgen, Celgene, MSD, Cristina Hidalgo: None declared, Sabela Fernández: None declared, R. López-Sánchez: None declared, Edilia García-Fernández: None declared, S. Castro: None declared, P. Morales-Garrido: None declared, Andrea García-Valle: None declared, Rosa Expósito: None declared, L. Exposito-Perez: None declared, Lorena Pérez Albaladejo: None declared, Ángel García-Aparicio: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Diagnostic Approach to Advanced Fibrotic Interstitial Lung Disease: Bringing Together Clinical, Radiologic, and Histologic Clues

2016 ◽  
Vol 141 (7) ◽  
pp. 901-915 ◽  
Author(s):  
Brandon T. Larsen ◽  
Maxwell L. Smith ◽  
Brett M. Elicker ◽  
Jessica M. Fernandez ◽  
Guillermo A. Arbo-Oze de Morvil ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Data Sources ◽  
Advanced Fibrosis ◽  
Diagnostic Dilemma ◽  
End Stage

Context.— Idiopathic pulmonary fibrosis (IPF) is a distinctive clinicopathologic entity and the most common form of progressive diffuse lung scarring in older adults. Idiopathic pulmonary fibrosis manifests histopathologically as the usual interstitial pneumonia pattern. The usual interstitial pneumonia pattern is distinguished by geographically and temporally heterogeneous fibrosis that is peripherally accentuated, often with honeycombing and traction bronchiectasis. Idiopathic pulmonary fibrosis is not the only disease that leads to end-stage lung fibrosis, however, and several other entities may also cause advanced fibrosis. Surgical lung biopsies often present a diagnostic dilemma when they show clear evidence of advanced fibrosis, but the clinical, imaging, and/or histopathologic subcharacteristics suggest something other than IPF. Objective.— To address this dilemma, we review several other fibrotic lung diseases, including connective tissue disease–associated interstitial lung disease, chronic hypersensitivity pneumonitis, advanced pulmonary Langerhans cell histiocytosis, end-stage pulmonary sarcoidosis, Erdheim-Chester disease, Hermansky-Pudlak syndrome, and others, detailing their clinical, radiologic, and histopathologic attributes and emphasizing similarities to and differences from IPF. Data Sources.— Data sources comprised published peer-reviewed literature and personal experience of the authors. Conclusions.— Often, clues in the lung biopsy may offer the first suggestion of a fibrotic lung disease other than IPF, and accurate classification is important for prognosis, treatment, and the development of future therapies.

scholarly journals SAT0040 RISK FACTORS FOR DEVELOPING AND MORTALITY FOR ACUTE EXACERBATION OF RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 950.2-951
Author(s):  
S. Izuka ◽  
H. Yamashita ◽  
Y. Takahashi ◽  
H. Kaneko
Keyword(s):  
Rheumatoid Arthritis ◽  
Risk Factors ◽  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Vascular Diseases ◽  
Collagen Vascular Diseases ◽  
Survivor Group

Background:Among collagen vascular diseases, rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is the most commonly associated with ILD with acute exacerbation (AE) [1]. One study reported that ILD diagnosis at an older age, the usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography, and methotrexate (MTX) use were associated with AE in patients with RA-ILD [2]. However, because these studies included few patients, the risk factors and prognosis of AE in patients with RA-ILD remain unclear. Therefore, this study examined the characteristics of RA-ILD patients with AE, and the variables associated with mortality due to AE of RA-ILD.Objectives:To investigate the risk factors for AE and mortality of RA-ILD.Methods:We retrospectively collected the clinical data of 165 RA-ILD patients admitted to our hospital between July 2010 and October 2019. We compared clinical characteristics between patients who developed AE (AE group) and those who did not (non-AE group), and identified the variables significantly associated with AE occurrence. We also compared the admission characteristics of those who survived (survivor group) and those who died (non-survivor group) after admission for AE. AE was defined using previously proposed criteria [3], which were modified slightly for application to RA-ILD.Results:The mean patient age was 73.6 ± 9.7 years and 97 (71.9%) patients were female. Thirty (22.2%) patients developed AE, of whom thirteen (43.3%) died (mean follow-up, 64.9 months). In univariate analyses UIP pattern and MTX were not associated with AE. However, in multivariate analyses, UIP pattern was associated with AE (OR 2.68, 95% CI 1.10–6.52,p=0.03). Median age (70vs. 80 years,p=0.003), non-use of MTX (70.6%vs. 23.1%,p=0.025), and C reactive protein level (median 9.38vs. 18.12 mg/dL,p=0.02) on admission were significantly higher in patients who died of AE. In the Cox proportional hazard model, UIP pattern (HR 4.67, 95% CI 1.02–21.5,p=0.048) and non-use of MTX (HR 0.16, 95% CI 0.04–0.72,p=0.016) were associated with death.Conclusion:Our data suggest that the UIP pattern is related to AE, and non-use of MTX and UIP pattern are related to death due to AE of RA-ILD.References:[1] Suda T, Kaida Y, Nakamura Y et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases.Respir Med2009;103:846-53.[2] Hozumi H, Nakamura Y, Johkoh T et al. Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study.BMJ Open2013;3:e003132.[3] Collard HR, Moore BB, Flaherty KR et al. Idiopathic pulmonary fibrosis clinical research network investigators. Acute exacerbations of idiopathic pulmonary fibrosis.Am J Respir Crit Care Med2007;176:636-43.Disclosure of Interests:None declared

scholarly journals Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Regina Celia Carlos Tibana ◽  
Maria Raquel Soares ◽  
Karin Mueller Storrer ◽  
Gustavo de Souza Portes Meirelles ◽  
Katia Hidemi Nishiyama ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Clinical Diagnosis ◽  
Lung Biopsy ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Surgical Lung Biopsy

Abstract Background Usual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable usual interstitial pneumonia pattern on HRCT who were subjected to surgical lung biopsy. Methods HRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded. Results A total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%) followed by idiopathic pulmonary fibrosis (24.0%), interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%) and familial interstitial lung disease (10.0%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and fibroblastic foci on surgical lung biopsy had a high risk of death. Conclusions The most common disease associated with a probable usual interstitial pneumonia pattern on HRCT is fibrotic hypersensitivity pneumonitis followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequencies of usual interstitial pneumonia and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indications for surgical lung biopsy.

scholarly journals Mortality rate in rheumatoid arthritis-related interstitial lung disease: the role of radiographic patterns

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Maria A. Nieto ◽  
Maria J. Rodriguez-Nieto ◽  
Olga Sanchez-Pernaute ◽  
Fredeswinda Romero-Bueno ◽  
Leticia Leon ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Mortality Rate ◽  
General Population ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Multiple Regression Model ◽  
Multicentric Study

Abstract Background To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients and to evaluate the role of radiographic patterns in mortality. Methods A longitudinal multicentric study was conducted in RA-ILD patients from 2005 to 2015 and followed-up until October 2018 in Madrid. Patients were included in the Neumologia-Reumatología y Enfermedades Autoinmunes Registry, from diagnosis of ILD. The main outcome was all-cause mortality. The radiographic pattern at baseline [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), or others] was the independent variable. Covariables included sociodemographic and clinical data. Survival techniques were used to estimate MR, expressed per 1000 persons-year with their 95% confidence intervals [CI]. Cox multiple regression model was run to examine the influence of radiographic patterns on survival. SMR [CI] was calculated comparing MR obtained with MR expected in the general population of Madrid by indirect age-gender standardization. Results 47 patients were included with a follow-up 242 patients-year. There were 16 (34%) deaths, and most frequent causes were acute ILD exacerbation and pneumonia. MR was 64.3 [39.4–104.9], and 50% of the patients died at 8.3 years from ILD diagnosis. After adjusting for confounders, (UIP compared to NSIP was associated with higher mortality risk. The overall SMR was 2.57 [1.4–4.17]. Women of 60–75 years of age were the group with the highest SMR. Conclusions RA-ILD is associated with an excess of mortality compared to general population. Our results support that UIP increases the risk of mortality in RA-ILD, regardless other factors.

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