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Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in...
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Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (30 June 2021) | Viewed by 38803

Special Issue Editors

Prof. Dr. Marco Sebastiani

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Guest Editor
Rheumatology Unit, Azienda Ospedaliera Guglielmo da Saliceto di Piacenza, University of Parma, 43126 Parma, Italy
Interests: interstitial lung disease; rheumatoid arthritis; nailfold capillaroscopy; systemic vasculitis; connctive tissue diseases
Special Issues, Collections and Topics in MDPI journals
Dr. Andreina Manfredi

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Guest Editor
Rheumatology Unit, Azienda Ospedaliera Universitaria Policlinico of Modena, 41121 Modena, Italy
Interests: interstitial lung disease in rheumatic disorders; diagnosis and treatment of interstitial lung disease; connective tissue diseases; nailfold capillaroscopy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Interstitial lung disease (ILD) is one of the most common and serious pulmonary complications of connective tissue diseases (CTD) and rheumatoid arthritis (RA), with variable clinical impact and severity, often resulting in significant morbidity and mortality. The prevalence reported for ILD is variable according to different classification modalities among case series and specific diagnoses, with a higher frequency in patients with systemic sclerosis and idiopathic inflammatory myopathies. The real incidence of RA-ILD is unknown, but prevalence rates of 7.7% for symptomatic RA-ILD, 67% for radiologic RA-ILD, and 80% for biopsy-identified RA-ILD have been reported. About 10% of RA patients develop a clinically significant ILD that is responsible for 10%–20% of mortality, with a mean survival of 5–8 years. Due to the lack of randomized controlled trials and recommendations, the therapeutic choice for CTD-ILD and RA-ILD is currently very challenging. To further complicate this scenario, both conventional and biologic disease-modifying anti-rheumatic drugs (DMARDs) have been implicated in the development of drug-related pulmonary toxicity, with conflicting data. Due to the absence of evidence for RA-ILD treatment efficacy and potential adverse effects, the decision to treat should be based on the balance between treatment benefits and the burden of the disease in each single patient.

Dr. Marco Sebastiani
Dr. Andreina Manfredi
Guest Editors

Manuscript Submission Information

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Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • lung fibrosis
  • interstitial lung disease
  • rheumatoid arthritis
  • connective tissue diseases
  • vasculitis
  • biologic and conventional DMARDs
  • anti-fibrotic agents

Published Papers (8 papers)

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Editorial

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5 pages, 206 KiB  
Editorial
Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders: Still a Long Journey
by Marco Sebastiani, Caterina Vacchi, Giulia Cassone and Andreina Manfredi
J. Clin. Med. 2022, 11(2), 410; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm11020410 - 14 Jan 2022
Cited by 4 | Viewed by 1457
Abstract
Interstitial lung disease (ILD) is one of the most frequent pulmonary complications of autoimmune rheumatic diseases (ARDs), and it is mainly associated with connective tissue diseases (CTDs) and rheumatoid arthritis (RA) [...] Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)

Research

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19 pages, 1444 KiB  
Article
Rituximab in the Treatment of Interstitial Lung Disease Associated with Autoimmune Diseases: Experience from a Single Referral Center and Literature Review
by Belén Atienza-Mateo, Sara Remuzgo-Martínez, Diana Prieto-Peña, Víctor Manuel Mora Cuesta, David Iturbe-Fernández, Javier Llorca, Lara Sánchez-Bilbao, Alfonso Corrales, Gerardo Blanco Rodríguez, José Javier Gómez-Román, José Manuel Cifrián and Miguel Ángel González-Gay
J. Clin. Med. 2020, 9(10), 3070; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9103070 - 23 Sep 2020
Cited by 20 | Viewed by 3287
Abstract
In the present study, we aimed to report our experience with rituximab (RTX) in the treatment of patients with ILD associated with AD (AD-ILD) at a single center. For this purpose, clinical characteristics, radiological findings, and pulmonary function tests (PFTs) of RTX-treated AD-ILD-patients [...] Read more.
In the present study, we aimed to report our experience with rituximab (RTX) in the treatment of patients with ILD associated with AD (AD-ILD) at a single center. For this purpose, clinical characteristics, radiological findings, and pulmonary function tests (PFTs) of RTX-treated AD-ILD-patients seen from May 2016 until March 2020 at a referral center for individuals with ILD were retrospectively reviewed. Additionally, an updated literature review was conducted. A total of 26 patients (mean age 58.3 ± 11.1 years at ILD diagnosis) was included. The most common ADs related to ILD were systemic sclerosis, idiopathic inflammatory myositis (including anti-synthetase syndrome) and rheumatoid arthritis. Non-specific interstitial pneumonia (n = 12) and usual interstitial pneumonia (n = 11) were the predominant radiological patterns. The sustained improvement in PFTs was observed from the start of RTX, with a statistically significant increase in DLCO from basal to one year after RTX (mean + 4.2%, p = 0.024). Overall, there were no differences when comparing PFT outcome according to the radiological pattern or the specific type of AD. In conclusion, RTX constitutes a good therapeutic option to preserve lung function in patients with AD-ILD, regardless of the radiological pattern or the underlying AD. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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11 pages, 510 KiB  
Article
Functional Progression in Patients with Interstitial Lung Disease Resulted Positive to Antisynthetase Antibodies: A Multicenter, Retrospective Analysis
by Giulia Dei, Paola Rebora, Martina Catalano, Marco Sebastiani, Paola Faverio, Maria Rosa Pozzi, Andreina Manfredi, Paolo Cameli, Francesco Salton, Carlo Salvarani, Lorenzo Cavagna, Marco Confalonieri, Elena Bargagli, Fabrizio Luppi and Alberto Pesci
J. Clin. Med. 2020, 9(9), 3033; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9093033 - 21 Sep 2020
Cited by 5 | Viewed by 2219
Abstract
Antisynthetase syndrome (ASSD) is a rare autoimmune disease characterized by serologic positivity for antisynthetase antibodies. Anti-Jo1 is the most frequent, followed by anti PL-7, anti PL-12, anti EJ, and anti OJ antibodies. The lung is the most frequently affected organ, usually manifesting with [...] Read more.
Antisynthetase syndrome (ASSD) is a rare autoimmune disease characterized by serologic positivity for antisynthetase antibodies. Anti-Jo1 is the most frequent, followed by anti PL-7, anti PL-12, anti EJ, and anti OJ antibodies. The lung is the most frequently affected organ, usually manifesting with an interstitial lung disease (ILD), which is considered the main determinant of prognosis. Some evidences suggest that non-anti-Jo-1 antibodies may be associated with more severe lung involvement and possibly with poorer outcomes, while other authors do not highlight differences between anti-Jo1 and other antisynthetase antibodies. In a multicenter, retrospective, “real life” study, we compared lung function tests (LFTs) progression in patients with ILD associated with anti-Jo1 and non-anti-Jo1 anti-synthetase antibodies to assess differences in lung function decline between these two groups. Therefore, we analyzed a population of 57 patients (56% anti-Jo1 positive), referred to the outpatient Clinic of four referral Centers in Italy (Modena, Monza, Siena, and Trieste) from 2008 to 2019, with a median follow-up of 36 months. At diagnosis, patients showed a mild ventilatory impairment and experienced an improvement of respiratory function during treatment. We did not observe statistically significant differences in LFTs at baseline or during follow-up between the two groups. Moreover, there were no differences in demographic data, respiratory symptoms onset (acute vs. chronic), extrapulmonary involvement, treatment (steroid and/or another immunosuppressant), or oxygen supplementation. Our study highlights the absence of differences in pulmonary functional progression between patients positive to anti-Jo-1 vs. non anti-Jo-1 antibodies, suggesting that the type of autoantibody detected in the framework of ASSD does not affect lung function decline. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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12 pages, 437 KiB  
Article
The Spectrum of Interstitial Lung Disease Associated with Autoimmune Diseases: Data of a 3.6-Year Prospective Study from a Referral Center of Interstitial Lung Disease and Lung Transplantation †
by Belén Atienza-Mateo, Sara Remuzgo-Martínez, Víctor Manuel Mora Cuesta, David Iturbe-Fernández, Sonia Fernández-Rozas, Diana Prieto-Peña, Mónica Calderón-Goercke, Alfonso Corrales, Gerardo Blanco Rodríguez, José Javier Gómez-Román, Miguel Ángel González-Gay and José Manuel Cifrián
J. Clin. Med. 2020, 9(6), 1606; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9061606 - 26 May 2020
Cited by 22 | Viewed by 3538
Abstract
Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their risk of morbidity and mortality. However, little is known about the prevalence of AD in patients diagnosed with an ILD. In this prospective study, we determined the [...] Read more.
Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their risk of morbidity and mortality. However, little is known about the prevalence of AD in patients diagnosed with an ILD. In this prospective study, we determined the spectrum of ILD associated with AD (AD-ILD) among patients sent for assessment to a single clinic of ILD and lung transplantation from a referral center between May 2016 and December 2019. ILD diagnosis was made by pneumologists based on clinical and radiological findings and pulmonary function test abnormalities. All patients with ILD were also assessed by experienced rheumatologists. During the period of assessment, 338 patients were diagnosed with ILD. Among them, 32.8% fulfilled definitions for an AD. Most cases with AD-ILD had a diagnosis of rheumatoid arthritis (27.0%), systemic sclerosis (26.1%) or anti-synthetase syndrome (17.1%). Interestingly, 18% of the patients with AD-ILD were diagnosed as having an interstitial pneumonia with autoimmune features. Antinuclear antibodies and non-specific interstitial pneumonia were the most frequent positive autoantibodies and radiological pattern found in AD-ILD patients, respectively. In conclusion, our study indicates that a high number of ILD patients have a related AD. Consequently, close collaboration among rheumatologists and pneumologists is needed. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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10 pages, 647 KiB  
Article
Safety of Abatacept in Italian Patients with Rheumatoid Arthritis and Interstitial Lung Disease: A Multicenter Retrospective Study
by Giulia Cassone, Andreina Manfredi, Fabiola Atzeni, Vincenzo Venerito, Caterina Vacchi, Valentina Picerno, Federica Furini, Gian Luca Erre, Paola Tomietto, Anna Laura Fedele, Giovanni Della Casa, Valeria Nucera, Chiara Giannitti, Carlo Salvarani and Marco Sebastiani
J. Clin. Med. 2020, 9(1), 277; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9010277 - 19 Jan 2020
Cited by 46 | Viewed by 4526
Abstract
Background: Treatment of rheumatoid arthritis (RA)-related interstitial lung disease (ILD) is challenging, and many conventional and biologic disease-modifying anti-rheumatic drugs (DMARDs) have been associated with ILD development or progression. The aim of this multicentric retrospective study was to analyze the evolution of ILD [...] Read more.
Background: Treatment of rheumatoid arthritis (RA)-related interstitial lung disease (ILD) is challenging, and many conventional and biologic disease-modifying anti-rheumatic drugs (DMARDs) have been associated with ILD development or progression. The aim of this multicentric retrospective study was to analyze the evolution of ILD in Italian RA-ILD patients treated with abatacept (ABA). Methods: All RA-ILD patients treated with ABA for at least six months were retrospectively evaluated. Serology, previous and concurrent therapies, chest high-resolution computer tomography (HRCT), forced vital capacity (FVC), and lung diffusion of carbon monoxide (CO, DLCO) were collected. Results: Forty-four patients were included; HRCT, FVC, and DLCO were analyzed at baseline, at one year, and at the end of follow-up. A remission or a low disease activity of RA was reached in 41/44 patients. Overall, FVC and DLCO remained stable or increased in 86.1% and 91.7% of patients, respectively, while HRCT was stable or improved in 81.4% of them. Previous and concurrent treatments, in particular, methotrexate, serology, age, sex, joint and lung disease duration were not associated with the outcome at univariate analysis. Conclusion: The management of RA-ILD patients remains a critical unmet medical need. Waiting for prospective controlled studies, ABA has shown a good safety profile in our cohort of Italian RA-ILD patients. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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Review

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18 pages, 1462 KiB  
Review
Efficacy, Safety, and Tolerability of Treatments for Systemic Sclerosis-Related Interstitial Lung Disease: A Systematic Review and Network Meta-Analysis
by Gian Luca Erre, Marco Sebastiani, Maria Antonietta Fenu, Angelo Zinellu, Alberto Floris, Lorenzo Cavagna, Elisabetta Renzoni, Andreina Manfredi, Giuseppe Passiu, Richard John Woodman and Arduino Aleksander Mangoni
J. Clin. Med. 2020, 9(8), 2560; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9082560 - 07 Aug 2020
Cited by 16 | Viewed by 4367
Abstract
Background: There is a paucity of head-to-head comparisons of the efficacy and harms of pharmacological treatments for systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: We conducted a network meta-analysis (NMA) in order to compare the effects of different treatments with the placebo on [...] Read more.
Background: There is a paucity of head-to-head comparisons of the efficacy and harms of pharmacological treatments for systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: We conducted a network meta-analysis (NMA) in order to compare the effects of different treatments with the placebo on change in forced vital capacity (FVC), change in diffusion lung capacity for CO (DLCO), serious adverse events (SAEs), discontinuation for adverse events and mortality in SSc-ILD. Standardized mean difference (SMD) and log odds ratio were estimated using NMA with fixed effects. Results: Nine randomized clinical trials (926 participants) comparing eight interventions and the placebo for an average follow-up of one year were included. Compared to the placebo, only rituximab significantly reduced FVC decline (SMD (95% CI) = 1.00 (0.39 to 1.61)). Suitable data on FVC outcome for nintedanib were not available for the analysis. No treatments influenced DLCO. Safety and mortality were also not different across treatments and the placebo, although there were few reported events. Cyclophosphamide and pomalidomide were less tolerated than the placebo, mycophenolate, and nintedanib. Conclusion: Only rituximab significantly reduced lung function decline compared to the placebo. However, direct head-to-head comparison studies are required to confirm these findings and to better determine the safety profile of various treatments. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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35 pages, 1385 KiB  
Review
Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows
by Giulia Cassone, Andreina Manfredi, Caterina Vacchi, Fabrizio Luppi, Francesca Coppi, Carlo Salvarani and Marco Sebastiani
J. Clin. Med. 2020, 9(4), 1082; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9041082 - 10 Apr 2020
Cited by 67 | Viewed by 10559
Abstract
Rheumatoid arthritis (RA) is a chronic and systemic inflammatory disease affecting 0.5–1% of the population worldwide. Interstitial lung disease (ILD) is a serious pulmonary complication of RA and it is responsible for 10–20% of mortality, with a mean survival of 5–8 years. However, [...] Read more.
Rheumatoid arthritis (RA) is a chronic and systemic inflammatory disease affecting 0.5–1% of the population worldwide. Interstitial lung disease (ILD) is a serious pulmonary complication of RA and it is responsible for 10–20% of mortality, with a mean survival of 5–8 years. However, nowadays there are no therapeutic recommendations for the treatment of RA-ILD. Therapeutic options for RA-ILD are complicated by the possible pulmonary toxicity of many disease modifying anti-rheumatic drugs (DMARDs) and by their unclear efficacy on pulmonary disease. Therefore, joint and lung involvement should be evaluated independently of each other for treatment purposes. On the other hand, some similarities between RA-ILD and idiopathic pulmonary fibrosis and the results of the recent INBIULD trial suggest a possible future role for antifibrotic agents. From this perspective, we review the current literature describing the pulmonary effects of drugs (immunosuppressants, conventional, biological and target synthetic DMARDs and antifibrotic agents) in patients with RA and ILD. In addition, we suggest a framework for the management of RA-ILD patients and outline a research agenda to fill the gaps in knowledge about this challenging patient cohort. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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26 pages, 422 KiB  
Review
Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review
by Caterina Vacchi, Marco Sebastiani, Giulia Cassone, Stefania Cerri, Giovanni Della Casa, Carlo Salvarani and Andreina Manfredi
J. Clin. Med. 2020, 9(2), 407; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm9020407 - 03 Feb 2020
Cited by 42 | Viewed by 7630
Abstract
Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD’s natural history and due to the difficulties [...] Read more.
Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD’s natural history and due to the difficulties related to design of randomized control trials, there is a lack of prospective data about the prevalence, follow-up, and therapeutic efficacy. For these reasons, the choice of therapy for CTD-ILD is currently very challenging and still largely based on experts’ opinion. Treatment is often based on steroids and conventional immunosuppressive drugs, but the recent publication of the encouraging results of the INBUILD trial has highlighted a possible effective and safe use of antifibrotic drugs as a new therapeutic option for these subjects. Aim of this review is to summarize the available data and recent advances about therapeutic strategies for ILD in the context of various CTD, such as systemic sclerosis, idiopathic inflammatory myopathy and Sjogren syndrome, systemic lupus erythematosus, mixed connective tissue disease and undifferentiated connective tissue disease, and interstitial pneumonia with autoimmune features, focusing also on ongoing clinical trials. Full article
(This article belongs to the Special Issue Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders)
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