scholarly journals Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

2021 ◽  
Vol 10 (12) ◽  
pp. 2548
Author(s):  
Marco Sebastiani ◽  
Fabrizio Luppi ◽  
Gianluca Sambataro ◽  
Diego Castillo Villegas ◽  
Stefania Cerri ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Rheumatic Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Clinical History ◽  
High Resolution Computed Tomography ◽  
Function Testing ◽  
And Diffusion

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

scholarly journals Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis

2020 ◽  
Author(s):  
Tamara Vojinovic ◽  
Ilaria Cavazzana ◽  
Paolo Ceruti ◽  
Micaela Fredi ◽  
Denise Modina ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Lung Function Test ◽  
High Resolution Computed Tomography ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511

Abatacept in interstitial lung disease associated with rheumatoid arthritis: national multicenter study of 263 patients

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3906-3916 ◽  
Author(s):  
Carlos Fernández-Díaz ◽  
Santos Castañeda ◽  
Rafael B Melero-González ◽  
Francisco Ortiz-Sanjuán ◽  
Antonio Juan-Mas ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Standard Dose ◽  
Usual Interstitial Pneumonia ◽  
Point Change ◽  
Lung Capacity ◽  
Sparing Effect ◽  
Severe Degree

Abstract Objective To assess the efficacy of abatacept (ABA) in RA patients with interstitial lung disease (ILD) (RA-ILD). Methods This was an observational, multicentre study of RA-ILD patients treated with at least one dose of ABA. ILD was diagnosed by high-resolution CT (HRCT). We analysed the following variables at baseline (ABA initiation), 12 months and at the end of the follow-up: Modified Medical Research Council (MMRC) scale (1-point change), forced vital capacity (FVC) or diffusion lung capacity for carbon monoxide (DLCO) (improvement or worsening ≥10%), HRCT, DAS on 28 joints evaluated using the ESR (DAS28ESR) and CS-sparing effect. Results We studied 263 RA-ILD patients [150 women/113 men; mean (s.d.) age 64.6 (10) years]. At baseline, they had a median duration of ILD of 1 (interquartile range 0.25–3.44) years, moderate or severe degree of dyspnoea (MMRC grade 2, 3 or 4) (40.3%), FVC (% of the predicted) mean (s.d.) 85.9 (21.8)%, DLCO (% of the predicted) 65.7 (18.3) and DAS28ESR 4.5 (1.5). The ILD patterns were: usual interstitial pneumonia (UIP) (40.3%), non-specific interstitial pneumonia (NSIP) (31.9%) and others (27.8%). ABA was prescribed at standard dose, i.v. (25.5%) or s.c. (74.5%). After a median follow-up of 12 (6–36) months the following variables did not show worsening: dyspnoea (MMRC) (91.9%); FVC (87.7%); DLCO (90.6%); and chest HRCT (76.6%). A significant improvement of DAS28ESR from 4.5 (1.5) to 3.1 (1.3) at the end of follow-up (P < 0.001) and a CS-sparing effect from a median 7.5 (5–10) to 5 (2.5–7.5) mg/day at the end of follow-up (P < 0.001) was also observed. ABA was withdrawn in 62 (23.6%) patients due to adverse events (n = 30), articular inefficacy (n = 27), ILD worsening (n = 3) and other causes (n = 2). Conclusion ABA may be an effective and safe treatment for patients with RA-ILD.

scholarly journals Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease

2015 ◽  
Vol 47 (2) ◽  
pp. 588-596 ◽  
Author(s):  
Joshua J. Solomon ◽  
Jonathan H. Chung ◽  
Gregory P. Cosgrove ◽  
M. Kristen Demoruelle ◽  
Evans R. Fernandez-Perez ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Predictors Of Mortality ◽  
Risk Of Death ◽  
Increased Risk ◽  
Over Time

Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a “definite” or “possible” usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.

scholarly journals A Cross-Sectional Study of Radiological Profile of Interstitial Lung Disease Using High Resolution Computed Tomography of Patients Attending Medical College at Ahmedabad

2021 ◽  
Vol 8 (17) ◽  
pp. 1127-1132
Author(s):  
Nikunj C. Desai ◽  
Nilesh P. Parkar ◽  
Asutosh N. Dave
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Cross Sectional Study ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Acute Interstitial Pneumonia

BACKGROUND Interstitial lung disease (ILD) is an unpredictable diffuse parenchymal lung disease, which involves interstitium of lung (tissue around the alveoli of the lungs). High resolution computed tomography (HRCT) is one of the confirmatory, easily accessible methodology for the conclusion and follow up assessment of interstitial lung disease. We wanted to study the normal HRCT patterns found with interstitial lung disease and contrastingly different HRCT designs and clinical information in differential determination of pulmonary fibrosis. We also wanted to study the different patterns of interstitial lung disease on high resolution computed tomography and thereby provide accurate diagnosis and management to the patients. METHODS The study was a hospital based prospective, cross sectional study. In the present study, total fifty patients referred from Department of Medicine and Department of Pulmonary Medicine of GCS Medical College having suspicion of interstitial lung disease were studied from April 2019 to September 2019. All patients underwent HRCT thorax on 16 slice Siemens computerised tomography (CT) scan machine in recumbent position utilising usual HRCT protocol. Lung abnormalities were noticed and classified for explicit diagnosis of interstitial lung pathologies. RESULTS Most of the patients (N = 25) were found to be in the age group of 50 - 80 years (17 female & 8 male). Progressive dyspnoea (N = 47; 94 %) was the most common chief complaint. The most common form of interstitial lung disease was usual interstitial pneumonia (UIN) (N = 18; 36 %) in our study. Acute interstitial pneumonia (AIP) (N = 7; 14 %) and non-specific interstitial pneumonia, NSIP (N = 7; 14 %) were the next common interstitial lung diseases. CONCLUSIONS The most well-known interstitial lung disease seen in our examination was usual interstitial pneumonia. Cases of interstitial lung disease are on the rise. Interstitial lung disease should be ruled out in all patients with progressive dyspnoea, particularly when there are no obvious or known causes of dyspnoea. Clinical and laboratory findings, pulmonary function tests, history of exposure along with HRCT workup is indispensable for the identification or exclusion of interstitial lung disease. HRCT is also useful for the follow-up study. KEYWORDS Acute Interstitial Pneumonia (AIP), High Resolution Computed Tomography (HRCT), Interstitial Lung Disease (ILD), Nonspecific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP)

scholarly journals Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yun Jae Kim ◽  
Jooae Choe ◽  
Ho Jeong Kim ◽  
Jin Woo Song
Keyword(s):  
Prognostic Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Course ◽  
Usual Interstitial Pneumonia ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
High Resolution Computed Tomography

AbstractInterstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolution computed tomography (HRCT) images of 62 patients with primary SJS-ILD were retrospectively analyzed (biopsy-proven cases, n = 16). The mean patient age was 59.8 years; 83.9% of the patients were females, and 38.7% showed a usual interstitial pneumonia (UIP) pattern on HRCT. The median follow-up period was 61.5 months. During follow-up, 15 patients (24.2%) died, 7 (11.3%) experienced acute exacerbation (AE), and 27 (43.5%) progressed. The 1-, 3- and 5-year survival rates were 93.5%, 85.8%, and 81.1%, respectively. Age (hazard ratio [HR]: 1.158, P = 0.003), C-reactive protein (CRP) level (HR: 1.212, P = 0.045), FVC (HR: 0.902, P = 0.005), and a UIP pattern on HRCT (HR: 4.580, P = 0.029) were significant prognostic factors in multivariable Cox analysis. In conclusion, death, AE, and ILD progression occurred in 25%, 10%, and 50% of the patients with SJS-ILD, respectively. Older age, higher CRP level, lower FVC, and a UIP pattern on HRCT indicated poor prognosis.

scholarly journals Interstitial lung disease pathology in systemic sclerosis

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110324
Author(s):  
Kristine E. Konopka ◽  
Jeffrey L. Myers
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Restrictive Lung Disease ◽  
Diffuse Parenchymal Lung Disease ◽  
Lung Biopsies

Interstitial lung disease is a relatively frequent manifestation of systemic sclerosis with approximately one-third of patients developing clinical restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the most common cause of diffuse parenchymal lung disease in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like changes may accompany other forms of interstitial lung disease, most commonly UIP. In an appropriate clinical setting with supportive high-resolution computed tomography findings, lung biopsy is not needed to confirm the presence of interstitial lung disease and surgical lung biopsies are often reserved for atypical presentations. In this review, we discuss the histological findings that define the most common patterns of SSc-ILD and outline other findings sometimes encountered in lung biopsies obtained from systemic sclerosis patients, including pulmonary vascular changes, aspiration, chronic pleuritis, and diffuse alveolar damage.

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 OP0212 ABATACEPT IN INTERSTITIAL LUNG DISEASE ASSOCIATED WITH RHEUMATOID ARTHRITIS. NATIONAL MULTICENTER STUDY OF 263 PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 132.1-132
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 ◽  
Retention Rate ◽  
Usual Interstitial Pneumonia ◽  
Research Support ◽  
Point Change ◽  
Prednisone Dose

Background:Interstitial Lung Disease (ILD) is a severe complication of Rheumatoid Arthritis (RA). Several conventional disease-modifying anti-rheumatic drugs (cDMARDs) and biologic (b) DMARDs may induce or impaired ILD-RA. Abatacept (ABA) may be useful in ILD-RA (1).Objectives:To assess the efficacy and safety of ABA in a large series of ILD-RA for a long-term follow-up.Methods:Multicenter open-level study of ILD-RA treated with at least 1 dose of ABA. ILD was diagnosed by high-resolution computed tomography (HRTC). We study these outcomes: a) 1-point change Modied Medical Research Council (MMRC); b) forced vital capacity (FVC) and/or DLCO improvement or decline ≥10%; c) change in HRCT, d) change in DAS28. e) Prednisone dose. Values were collected at 0, 3, 6, 12 and then every 12 months.Results:We studied 263 patients (150 women/113 men) (mean age;64.6±10 years), with ILD-RA. At ABA-onset they were smokers or exsmoker (53.8%), positive APCC (88.6%), median [IQR] duration of ILD of 12 [3-41.25] months, mean DLCO (65.7±18.3) and FVC (85.9±21.8).The ILD-pattern were usual interstitial pneumonia (UIP) (40.3%), non-specific interstitial pneumonia (NSIP) (31.9%) and others (27.8%).ABA was prescribed at standard subcutaneous (125 mg/w) in 196 (74.5%) or intravenously (10 mg/kg/4 w) in 67 (25.5%); in monotherapy (n=111) or combined with cDMARDs (n=152); especially leflunomide (n=55), MTX (n=46), or antimarials (n=21).After a mean follow-up of 22.7±19.7 months most outcomes remain stable (Figure). Moreover, DAS28 improved from 4.5±1.5 to 3.1±1.3; prednisone dose reduced from a median 7.5 [5-10] to 5 mg [5-7.5] and retention rate was 76.4%. The main adverse effects were serious infections (n=28), neoplasia (n=3), serious infusion reaction (n=1) and myocardial infarction (n=1).Conclusion:ABA seems effective and relatively safe in ILD-RA.References:[1]Fernández-Díaz C et al. Semin Arthritis Rheum. 2018; 48:22-27Disclosure 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, Edilia García-Fernández: None declared, R. López-Sánchez: 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

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