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New Insights in Lung Involvement Secondary to Autoimmune Diseases
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New Insights in Lung Involvement Secondary to Autoimmune Diseases

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

Deadline for manuscript submissions: closed (20 August 2024) | Viewed by 6925

Special Issue Editors

Dr. Gianluca Sambataro

E-Mail Website
Guest Editor
Regional Referral Centre for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy
Interests: interstitial pneumonia with autoimmune features; IPAF; interstitial lung diseases associated with systemic autoimmune diseases
Dr. Giovanni Zanframundo

E-Mail Website
Guest Editor
Department of Internal Medicine and Therapeutics, Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
Interests: idiopathic inflammatory myopathies; antisynthetase syndrome
Dr. Domenico Sambataro

E-Mail Website
Guest Editor
Artroreuma srl, Reumatology Outpatient Clinic Accredited with Italian National Health System, Corso San Vito 53, 95030 Mascalucia, Italy
Interests: connective tissues diseases; interstitial lung disease associated to connective tissue diseases; disease activity; nailfold capillaroscopy

Special Issue Information

Dear Colleagues,

Respiratory failure is among the main causes of mortality in many Systemic Autoimmune Diseases (SAD), including Rheumatoid Arthritis (RA), Vasculitides and Connective Tissue Diseases.

Lung involvement in the context of SAD typically results in Interstitial Lung Disease (ILD), however obstructive conditions due to small airways involvement are not uncommon.

ILD may represent the first manifestation of SAD, dominating the clinical picture compared to more nuanced non-respiratory signs and symptoms. In these cases, proper classification of the underlying SAD becomes a true challenge and may be subjected to variations during follow-up (e.g., Interstitial Pneumonia with autoimmune features progressing to a specific SAD).

Diagnosis and management of SAD-related lung involvement often requires a multidisciplinary approach involving pulmonologists and rheumatologists, which is not always feasible in routine clinical practice.

This Special Issue aims to collect the most recent findings regarding diagnosis and management of patients affected by SAD with lung involvement. We believe that this focused approach will significantly contribute to current knowledge on this topic. Another purpose of this Special Issue is to stimulate the scientific debate and a closer collaboration between pulmonologists and rheumatologists aimed at a mutual sharing of competences.

In this Special Issue, original research articles, brief reports of original data and reviews are welcome. Research areas may include (but should not be limited to) the following:

  • Diagnostic approach to SAD patients with lung involvement;
  • Imaging techniques for assessment of SAD-related lung involvement, including application of artificial intelligence to HRCT analysis;
  • Risk factors for lung involvement in SAD patients;
  • Prognosis of SAD with lung involvement;
  • Pathogenic mechanisms underlying lung involvement in SAD;
  • Multidisciplinary evaluation of SAD patients with lung involvement;
  • Therapeutic strategies for SAD-related lung involvement.

We look forward to receiving your contributions.

Dr. Gianluca Sambataro
Dr. Giovanni Zanframundo
Dr. Domenico Sambataro
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

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

  • interstitial lung disease
  • interstitial pneumonia with autoimmune features
  • antisynthetase syndrome
  • rheumatoid arthritis
  • Sjogren’s syndrome
  • systemic sclerosis
  • idiopathic inflammatory myopathy

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Published Papers (5 papers)

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9 pages, 254 KiB  
Article
MUC5B rs35705950 Promoter Variant Is Associated with Usual Interstitial Pneumonia in Patients with Antisynthetase Syndrome
by Daphne Rivero-Gallegos, Mayra Mejía, Karol J. Nava-Quiroz, Espiridión Ramos-Martínez, Heidegger N. Mateos-Toledo, Héctor Isaac Rocha-González, Juan Carlos Huerta-Cruz, Gloria Pérez-Rubio, Ingrid Fricke-Galindo, Jorge Rojas-Serrano and Ramcés Falfán-Valencia
J. Clin. Med. 2024, 13(20), 6159; https://doi.org/10.3390/jcm13206159 - 16 Oct 2024
Viewed by 707
Abstract
Background: The presence of the rs35705950 variant in the MUC5B gene promoter is a critical genetic risk factor in idiopathic pulmonary fibrosis (IPF). It has been associated with usual interstitial pneumonia (UIP) in several interstitial lung diseases (ILDs). In antisynthetase syndrome (ASSD), [...] Read more.
Background: The presence of the rs35705950 variant in the MUC5B gene promoter is a critical genetic risk factor in idiopathic pulmonary fibrosis (IPF). It has been associated with usual interstitial pneumonia (UIP) in several interstitial lung diseases (ILDs). In antisynthetase syndrome (ASSD), most high-resolution computed tomography (HRCT) patterns are inflammatory, but up to 13% have UIP, leading to a worse prognosis. Methods: This single-center study included 60 patients with ASSD-ILD. We investigated whether carrying the MUC5B rs35705950 promoter variant was associated with UIP. To estimate the strength of the association between the genotype of the MUC5B rs35705950 promoter variant and the fibrotic pattern we used the odds ratio (cOR), and to assess the effect of confounding variables (age, evolution time, and sex), we performed a logistic regression to obtained the adjusted odds ratio (aOR). Results: The GT genotype of the MUC5B rs35705950 promoter variant is associated with up to a 4-fold increased risk of UIP (cOR 5.0, 95% CI 1.13–22.10), and the effect was even maintained after adjusting for potentially confounding variables such as sex, age, and time to progression (aOR 5.2, 95% CI 1.04–25.89). Conclusions: our study supports the role of MUC5B rs35705950 in ASSD-ILD with UIP. It reinforces that this polymorphism in our population could have a similar genetic basis to that already described in other ILDs that present predominantly fibrotic patterns. Full article
(This article belongs to the Special Issue New Insights in Lung Involvement Secondary to Autoimmune Diseases)
13 pages, 806 KiB  
Article
Myositis-Associated Interstitial Lung Disease: The Experience of a Tertiary Center
by Bianca Paulo Correia, Raquel Campanilho-Marques, Eduardo Dourado, Mariana Silva, Augusto Silva, Filipa Costa, Matilde Bandeira, Ana Teresa Melo, Sofia C. Barreira and João E. Fonseca
J. Clin. Med. 2024, 13(20), 6085; https://doi.org/10.3390/jcm13206085 - 12 Oct 2024
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Abstract
Background: Interstitial lung disease (ILD) is a common extra-muscular manifestation of idiopathic inflammatory myopathies (IIMs), often associated with a poorer prognosis and increased mortality risk. Methods: This retrospective study aimed to characterize lung involvement and treatment response in an IIM cohort [...] Read more.
Background: Interstitial lung disease (ILD) is a common extra-muscular manifestation of idiopathic inflammatory myopathies (IIMs), often associated with a poorer prognosis and increased mortality risk. Methods: This retrospective study aimed to characterize lung involvement and treatment response in an IIM cohort at a Portuguese tertiary center, followed between June 2016 and March 2024. We analyzed data from high-resolution computed tomography (HRCT) scans and pulmonary function tests (PFTs) to assess associations with autoantibody profiles and treatment regimens. Results: A total of 198 patients were included, with 69 (34.8%) exhibiting ILD. Antisynthetase syndrome (ASyS) and dermatomyositis were the most common diagnoses among IIM-ILD patients, with ASyS being significantly more frequent in this group than in non-ILD patients (p < 0.001). Anti-Jo1 and anti-MDA-5 antibodies were more frequent in ILD patients (p < 0.001 and p = 0.021), while anti-Mi2 antibodies were less common (p = 0.002). Non-specific interstitial pneumonia (NSIP) was the most common radiological pattern (69.5%). IIM-ILD patients presented with significantly lower forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO) compared to non-ILD patients (p < 0.001 for all values). Longitudinal analysis showed improved DLCO (p = 0.022) and stable or improved FVC (p = 0.097), especially with intravenous immunoglobulin (IVIg) and azathioprine (AZA). Combination therapies including IVIg with mycophenolate mofetil (MMF) or rituximab (RTX) also improved DLCO and FVC. Most ILD patients (89.6%) had stable HRCT patterns over time. Conclusions: Our findings highlight the potential for stabilizing or even improving lung function in IIM-ILD with appropriate immunosuppressive therapy, particularly with regimens incorporating IVIg and AZA, and combination therapies. Full article
(This article belongs to the Special Issue New Insights in Lung Involvement Secondary to Autoimmune Diseases)
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14 pages, 1989 KiB  
Article
The Pattern and Progression of “Usual” Interstitial Pneumonia with Autoimmune Features: Comparison with Patients with Classic Interstitial Pneumonia with Autoimmune Features and Idiopathic Pulmonary Fibrosis
by Alessandro Libra, Michele Colaci, Lucia Spicuzza, Giuliana Luca, Sefora Fischetti, Giorgio Pashalidis, Chiara Alfia Ferrara, Giuseppe Ielo, Domenico Sambataro, Giuliana La Rosa, Federica Libra, Stefano Palmucci, Carlo Vancheri and Gianluca Sambataro
J. Clin. Med. 2024, 13(2), 369; https://doi.org/10.3390/jcm13020369 - 10 Jan 2024
Viewed by 1430
Abstract
Background: We proposed the term “UIPAF” to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called “Interstitial Pneumonia with Autoimmune Features” (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of [...] Read more.
Background: We proposed the term “UIPAF” to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called “Interstitial Pneumonia with Autoimmune Features” (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively. Methods: The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team. Results: We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF. Conclusions: UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF. Full article
(This article belongs to the Special Issue New Insights in Lung Involvement Secondary to Autoimmune Diseases)
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12 pages, 576 KiB  
Article
Clinical Characteristics of Anti-Synthetase Syndrome and Variables Associated with Interstitial Lung Disease and Mortality: A Retrospective Cohort Study
by Tulaton Sodsri, Tananchai Petnak and Pintip Ngamjanyaporn
J. Clin. Med. 2023, 12(21), 6849; https://doi.org/10.3390/jcm12216849 - 30 Oct 2023
Cited by 6 | Viewed by 1956
Abstract
Anti-synthetase syndrome (ASS) is a rare autoimmune disease. Since the knowledge of ASS remains limited, we conducted the retrospective study aiming to describe clinical characteristics and identify variables associated with interstitial lung disease (ILD) and mortality among patients with ASS. Patients diagnosed with [...] Read more.
Anti-synthetase syndrome (ASS) is a rare autoimmune disease. Since the knowledge of ASS remains limited, we conducted the retrospective study aiming to describe clinical characteristics and identify variables associated with interstitial lung disease (ILD) and mortality among patients with ASS. Patients diagnosed with ASS from January 2013 to October 2022 were included. Patient demographics, clinical manifestations, myositis auto-antibody profiles, HRCT findings, and laboratory tests were collected. Variables associated with mortality risk and ILD were evaluated using the Cox proportional hazards model and the logistic regression model, respectively. A total of 82 patients with ASS were included. Clinical manifestations included arthritis (57%), Raynaud’s phenomenon (32%), mechanic’s hands (29%), fever (26%), and myositis (17%). The myositis auto-antibody profiles included anti-PL-7 (29%), anti-Jo-1 (27%), anti-EJ (17%), anti-PL-12 (16%), and anti-OJ (11%). ILD was observed in 64 patients (78%). Among patients with ILD, 21 initially presented with ILD before developing other ASS clinical manifestations, 29 simultaneously presented with ILD and other symptoms, and 14 had isolated ILD throughout follow-up. Overall, 6 patients presented with rapid-progressive ILD. With a median follow-up time of 2.5 years, mortality was observed in 10 patients (12.2%). Factors associated with mortality included increased lymphocyte counts (adjusted HR, 0.74; 95% CI, 0.61–0.91; p < 0.01), isolated ILD (adjusted HR, 9.59; 95% CI, 1.52–60.61; p = 0.02) and the presence of anti-Ro52 antibodies (adjusted HR, 0.14; 95% CI, 0.02–0.93; p = 0.04). Factors associated with ILD included age (adjusted OR, 1.10; 95% CI, 1.03–1.18; p = 0.01), presence of anti-Ro52 antibodies (adjusted OR, 17.92; 95% CI, 2.13–138.68; p = 0.01), and presence of arthritis (adjusted OR, 0.09; 95% CI, 0.01–0.75; p = 0.03). Our study demonstrated a favorable overall mortality rate among ASS patients. Full article
(This article belongs to the Special Issue New Insights in Lung Involvement Secondary to Autoimmune Diseases)
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8 pages, 252 KiB  
Brief Report
Predictors of Interstitial Lung Disease in Mixed Connective Tissue Disease
by Manuel Silvério-António, Joana Martins-Martinho, Ana Teresa Melo, Francisca Guimarães, Eduardo Dourado, Daniela Oliveira, Jorge Lopes, André Saraiva, Ana Gago, Margarida Correia, Ana L. Fernandes, Sara Dinis, Rafaela Teixeira, Susana P. Silva, Carlos Costa, Tiago Beirão, Carolina Furtado, Pedro Abreu, Carmo Afonso and Nikita Khmelinskii
J. Clin. Med. 2023, 12(23), 7481; https://doi.org/10.3390/jcm12237481 - 3 Dec 2023
Viewed by 1414
Abstract
Interstitial lung disease (ILD) frequently complicates mixed connective tissue disease (MCTD) and contributes to increased mortality. We aimed to identify predictors of ILD in MCTD patients. This is a nationwide, multicentre, retrospective study including patients with an adult-onset MCTD clinical diagnosis who met [...] Read more.
Interstitial lung disease (ILD) frequently complicates mixed connective tissue disease (MCTD) and contributes to increased mortality. We aimed to identify predictors of ILD in MCTD patients. This is a nationwide, multicentre, retrospective study including patients with an adult-onset MCTD clinical diagnosis who met Sharp’s, Kasukawa, Alarcón-Segovia, or Kahn’s diagnostic criteria and had available chest high-resolution computed tomography (HRCT) data. Univariate and multivariate analyses were conducted. We included 57 MCTD patients, with 27 (47.4%) having ILD. Among ILD patients, 48.1% were asymptomatic, 80.0% exhibited a restrictive pattern on pulmonary function tests, and 81.5% had nonspecific interstitial pneumonia on chest HRCT. Gastroesophageal involvement (40.7% vs. 16.7%, p = 0.043) and lymphadenopathy at disease onset (22.2% vs. 3.3%, p = 0.045) were associated with ILD. Binary logistic regression identified lymphadenopathy at disease onset (OR 19.65, 95% CI: 1.91–201.75, p = 0.012) and older age at diagnosis (OR 1.06/year, 95% CI: 1.00–1.12, p = 0.046) as independent ILD predictors, regardless of gender and gastroesophageal involvement. This study is the first to assess a Portuguese MCTD cohort. As previously reported, it confirmed the link between gastroesophageal involvement and ILD in MCTD patients. Additionally, it established that lymphadenopathy at disease onset and older age at diagnosis independently predict ILD in MCTD patients. Full article
(This article belongs to the Special Issue New Insights in Lung Involvement Secondary to Autoimmune Diseases)
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