Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis
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 recen...
| Main Authors: | , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-11-01
|
| Series: | Medicina |
| Subjects: | |
| Online Access: | https://www.mdpi.com/1648-9144/58/12/1757 |
| _version_ | 1797456485209341952 |
|---|---|
| author | 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 Carlo Vancheri |
| author_facet | 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 Carlo Vancheri |
| author_sort | Stefano Palmucci |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T16:07:36Z |
| format | Article |
| id | doaj.art-d7d8fd2d5b7c49be98bd56bf8c16795d |
| institution | Directory Open Access Journal |
| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-09T16:07:36Z |
| publishDate | 2022-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-d7d8fd2d5b7c49be98bd56bf8c16795d2023-11-24T16:32:13ZengMDPI AGMedicina1010-660X1648-91442022-11-015812175710.3390/medicina58121757Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and DermatomyositisStefano Palmucci0Alessia Di Mari1Giovanna Cancemi2Isabella Pennisi3Letizia Antonella Mauro4Gianluca Sambataro5Domenico Sambataro6Federica Galioto7Giulia Fazio8Agata Ferlito9Fabio Pino10Antonio Basile11Carlo Vancheri12Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyRadiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyRegional Referral Center for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyArtroreuma S.R.L., Rheumatology Outpatient Clinic Associated with the National Health System, 95030 Mascalucia, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyRegional Referral Center for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyDepartment of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyRegional Referral Center for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital Policlinico “G. Rodolico-San Marco”, 95123 Catania, ItalyPolymyositis 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.https://www.mdpi.com/1648-9144/58/12/1757polymyositisdermatomyositislung disease interstitialmultidetector computed tomographyautoimmune diseases |
| spellingShingle | 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 Carlo Vancheri Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis Medicina polymyositis dermatomyositis lung disease interstitial multidetector computed tomography autoimmune diseases |
| title | Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis |
| title_full | Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis |
| title_fullStr | Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis |
| title_full_unstemmed | Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis |
| title_short | Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis |
| title_sort | clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis |
| topic | polymyositis dermatomyositis lung disease interstitial multidetector computed tomography autoimmune diseases |
| url | https://www.mdpi.com/1648-9144/58/12/1757 |
| work_keys_str_mv | AT stefanopalmucci clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT alessiadimari clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT giovannacancemi clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT isabellapennisi clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT letiziaantonellamauro clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT gianlucasambataro clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT domenicosambataro clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT federicagalioto clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT giuliafazio clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT agataferlito clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT fabiopino clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT antoniobasile clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis AT carlovancheri clinicalandradiologicalfeaturesofinterstitiallungdiseasesassociatedwithpolymyositisanddermatomyositis |