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Medicina
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Pulmonary Involvement Correlated with Rheumatic Diseases
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Pulmonary Involvement Correlated with Rheumatic Diseases

A special issue of Medicina (ISSN 1648-9144).

Deadline for manuscript submissions: closed (31 January 2023) | Viewed by 8017

Special Issue Editors

Dr. Elisa Baratella

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Guest Editor
Department of Radiology, Cattinara Hospital, University of Trieste, Strada di Fiume 447, 34128 Trieste, Italy
Interests: interstitial lung diseases; IPF; occupational lung diseases; sarcoidosis
Dr. Barbara Ruaro

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Co-Guest Editor
Pulmonology Unit, Department of Medical Surgical and Health Sciences, University Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy
Interests: interstitial lung disease; idiopathic pulmonary fibrosis; rheumatic diseases; pulmonary arterial hypertension (PAH)
Special Issues, Collections and Topics in MDPI journals
Dr. Cristina Marrocchio

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Co-Guest Editor
Department of Radiology, Cattinara Hospital, University of Trieste, 34127 Trieste, Italy
Interests: interstitial lung diseases; high-resolution CT; lung cancer; thoracic interventional procedures; lung nodules

Special Issue Information

Dear Colleagues,

Despite improvements in the care of patients suffering from rheumatic diseases, interstitial lung diseases (ILD) and cardiovascular involvement remain major determinants of morbidity and mortality in this population.  Therefore, the early detection of these complications and the precise assessment of their severity are crucial for the appropriate management of rheumatic patients.

In this context, high-resolution computed tomography (HRCT) plays a major role in diagnosing rheumatic disease-related ILD, identifying the pattern of lung involvement, and assessing the improvement, stability, or progression of the pulmonary alterations during follow-up. Moreover, chest CT scans can identify direct and indirect signs of cardiovascular involvement, including prognostically significant diseases, such as pulmonary hypertension.

Therefore, clinicians and radiologists involved in the care of rheumatic patients must be aware of the possible pulmonary and cardiovascular complications that may occur, their diagnostic assessment, and their therapeutic options.

This Special Issue aims to provide a thorough overview of the pulmonary and cardiovascular involvement secondary to rheumatic diseases in order to improve the management and care of these patients.

Dr. Elisa Baratella
Dr. Barbara Ruaro
Dr. Cristina Marrocchio
Guest Editors

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Keywords

  • interstitial lung diseases
  • chest CT
  • rheumatic diseases
  • pulmonary function tests (PFT)
  • vascular involvement
  • sarcoidosis
  • ultrasound

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Published Papers (2 papers)

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Research

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16 pages, 2275 KiB  
Article
Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria
by Francesco Bozzao, Paola Tomietto, Elisa Baratella, Metka Kodric, Rossella Cifaldi, Rossana Della Porta, Ilaria Prearo, Silvia Maria Grazia Pirronello, Paola Confalonieri, Barbara Ruaro, Fabio Fischetti and Bruno Fabris
Medicina 2023, 59(4), 794; https://doi.org/10.3390/medicina59040794 - 19 Apr 2023
Cited by 1 | Viewed by 2430
Abstract
Background and Objectives: The “interstitial pneumonia with autoimmune features” (IPAF) criteria have been criticized because of the exclusion of usual interstitial pneumonia (UIP) patients with a single clinical or serological feature. To classify these patients, the term UIPAF was proposed. This study [...] Read more.
Background and Objectives: The “interstitial pneumonia with autoimmune features” (IPAF) criteria have been criticized because of the exclusion of usual interstitial pneumonia (UIP) patients with a single clinical or serological feature. To classify these patients, the term UIPAF was proposed. This study aims to describe clinical characteristics and predictive factors for progression of a cohort of interstitial lung disease (ILD) patients with at least one feature of autoimmunity, applying criteria for IPAF, specific connective tissue diseases (CTD), and a definition of UIPAF when possible. Methods: We retrospectively evaluated data on 133 consecutive patients with ILD at onset associated with at least one feature of autoimmunity, referred by pulmonologists to rheumatologists from March 2009 to March 2020. Patients received 33 (16.5–69.5) months of follow-up. Results: Among the 101 ILD patients included, 37 were diagnosed with IPAF, 53 with ILD-onset CTD, and 11 with UIPAF. IPAF patients had a lower prevalence of UIP pattern compared to CTD-ILD and UIPAF patients (10.8% vs. 32.1% vs. 100%, p < 0.01). During the follow-up, 4 IPAF (10.8%) and 2 UIPAF (18.2%) patients evolved into CTD-ILD. IPAF patients presented features not included in IPAF criteria, such as sicca syndrome (8.1%), and were more frequently affected by systemic hypertension (p < 0.01). Over one year, ILD progression (greater extent of fibrosis on HRCT and/or decline in PFTs) was less frequent in the IPAF group compared to CTD-ILD and UIPAF (32.3% vs. 58.8% vs. 72.7, p = 0.02). A UIP pattern and an IPAF predicted a faster (OR: 3.80, p = 0.01) and a slower (OR: 0.28, p = 0.02) ILD progression, respectively. Conclusions: IPAF criteria help identify patients who might develop a CTD-ILD, even though a single clinical or serological feature is respected. Future revisions of IPAF criteria should include sicca syndrome and separate UIP-pattern into a different definition (UIPAF), given its association with a different prognosis, independently from ILD classification. Full article
(This article belongs to the Special Issue Pulmonary Involvement Correlated with Rheumatic Diseases)
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Review

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21 pages, 14164 KiB  
Review
Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis
by Stefano Palmucci, Alessia Di Mari, Giovanna Cancemi, Isabella Pennisi, Letizia Antonella Mauro, Gianluca Sambataro, Domenico Sambataro, Federica Galioto, Giulia Fazio, Agata Ferlito, Fabio Pino, Antonio Basile and Carlo Vancheri
Medicina 2022, 58(12), 1757; https://doi.org/10.3390/medicina58121757 - 30 Nov 2022
Cited by 10 | Viewed by 4869
Abstract
Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions—the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different [...] Read more.
Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions—the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different immunopathological mechanisms. In polymyositis, components of the cellular immune system are involved, whereas in dermatomyositis, the pathogenesis is mainly mediated by the humoral immune response. The interstitial lung disease occurs in one-third of polymyositis and dermatomyositis patients associated with worse outcomes, showing an estimated excess mortality rate of around 40%. Lung involvement may also appear, such as a complication of muscle weakness, mainly represented by aspiration pneumonia or respiratory insufficiency. The clinical picture is characterized, in most cases, by progressive dyspnea and non-productive cough. In some cases, hemoptysis and chest pain are found. Onset can be acute, sub-acute, or chronic. Pulmonary involvement could be assessed by High Resolution Computed Tomography (HRCT), which may identify early manifestations of diseases. Moreover, Computed Tomography (CT) appearances can be highly variable depending on the positivity of myositis-specific autoantibodies. The most common pathological patterns include fibrotic and cellular nonspecific interstitial pneumonia or organizing pneumonia; major findings observed on HRCT images are represented by consolidations, ground-glass opacities, and reticulations. Other findings include honeycombing, subpleural bands, and traction bronchiectasis. In patients having Anti-ARS Abs, HRCT features may develop with consolidations, ground glass opacities (GGOs), and reticular opacities in the peripheral portions; nonspecific interstitial pneumonia or nonspecific interstitial pneumonia mixed with organizing pneumonia have been reported as the most frequently encountered patterns. In patients with anti-MDA5 Abs, mixed or unclassifiable patterns are frequently observed at imaging. HRCT is a sensitive method that allows one not only to identify disease, but also to monitor the effectiveness of treatment and detect disease progression and/or complications; however, radiological findings are not specific. Therefore, aim of this pictorial essay is to describe clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis, emphasizing the concept that gold standard for diagnosis and classification–should be based on a multidisciplinary approach. Full article
(This article belongs to the Special Issue Pulmonary Involvement Correlated with Rheumatic Diseases)
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