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

Vojinovic, T.; Cavazzana, Ilaria; Ceruti, Paolo; Fredi, Micaela; Modina, Denise; Berlendis, Marialma; Franceschini, Franco

doi:10.1007/s12016-020-08814-5

Cited by 38 publications

(35 citation statements)

References 43 publications

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“…These CTDs involve varying degrees of skeletal muscle inflammation with frequent extra-muscular organ involvement. ILD is the most common extra-muscular manifestation of IIM with NSIP being the most prevalent form 38 , while OP is also frequently seen with IIM.…”

Section: Idiopathic Inflammatory Myopathies (Iim)mentioning

confidence: 99%

Treatment Options for Interstitial Lung disease associated with Connective Tissue Disease

Ehtesham¹,

Mahmood²,

Shabbir³

et al. 2021

J Lung Health Dis

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Section: Idiopathic Inflammatory Myopathies (Iim)mentioning

confidence: 99%

Treatment Options for Interstitial Lung disease associated with Connective Tissue Disease

Ehtesham¹,

Mahmood²,

Shabbir³

et al. 2021

J Lung Health Dis

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“…Interstitial fibrosing alveolitis overlapping with manifestations of Hamman-Rich syndrome can be observed in children and adults affected with several autoimmunopathies: Rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, Sjőgren syndrome, dermatomyositis and polymyositis, ankylosing spondylitis, and mixed connective tissue disease, as well as in autoimmune vasculitides and anti-synthetase syndrome [28; 52]. Some autoantibodies, like anti-Ro52 and anti-Ku correlated with dyspnea and other symptoms in patients with interstitial lung diseases, including acute ones [52].…”

Section: Covid-19 Vs Hamman-rich Syndrome: Close Similaritiesmentioning

confidence: 99%

Old wine in new wineskins. Hamman-Rich syndrome has much in common with severe COVID-19: Do they share any link of pathogenesis or depend on the similar aetiological factor(s)?

Ivashkevich

Korovin

Petyaeva

et al. 2020

Vestnik SPbSU. Medicine

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For the first time the acute interstitial pneumonitis as a main cause of adult idiopathic respiratory distress syndrome was described in 1933–35 by the American medical scholars Louis Virgil Hamman and Arnold Rice Rich as “acute fibrosing alveolitis” later given the eponymous name Hamman-Rich syndrome. This disease targets the interstitium of the lungs and characterized by fulminant onset, acute respiratory disorder with possibility of respiratory failure, being clinically similar to respiratory distress syndrome with the pathomorphologically revealed diffuse alveolar damage. Hamman-Rich syndrome is characterized by rapid progression from symptoms of common cold such as cough, fever, chills and dyspnea to severe respiratory failure. The syndrome affects apparently healthy individuals and is not associated with any anamnestic chronic lung diseases, smoking habits, neither with patients’ age or sex. The aetiology and pathogenesis of the Hamman-Rich syndrome are not clear yet. The familiar genetic predisposition is of definite meaning, especially related to mutations of surfactant proteins and their metabolism. The possible mechanistic links of this fulminant lung disease involve: NK-cells, auto-aggressive immune activation and certain types of cytokines during their excessive systemic action, so-called cytokine storm (e.g. interleukin-18 and interleukin-2), which play the leading part in the patho-genesis of acute lung cell injury resulting in diffuse non-remitting pneumofibrosis. But all these clinical and pathogenetic features are also common for severe COVID-19. Computed tomography is used for instrumental diagnosis of Hamman-Rich syndrome and also gives the lung images quite similar to that of COVID-19, including ground glass opacity symptoms. The authors hypothesize that the same link(s) of pathogenesis underlie the severe course of COVID-19 and Hamman-Rich syndrome. It is possible that cases of Hamman-Rich syndrome were due to the circulation in populations of a less virulent, unrecognized coronavirus, which interacts with the immune system of genetically susceptible individuals in a way similar to SARS-CoV2. For sure, the identification of the characteristics of the genome of patients with Hamman-Rich syndrome will shed light on the genetic mechanisms that predispose to the severe course of COVID-19 and vice versa — the experience of severe COVID-19 treatment can be applied to the therapy of Hamman-Rich syndrome as well.

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“…Interstitial lung disease (ILD) is a frequent complication in patients with idiopathic inflammatory myopathy, showing a predominantly non-specific interstitial pneumonia (NSIP) pattern in histopathological findings [1][2][3]. A large proportion of idiopathic-inflammatorymyopathy-related ILD (IIM-ILD) cases are rapidly progressive (RP) and refractory to conventional immunosuppressive therapy, making the clinical management of IIM-ILD challenging [4].…”

Section: Introductionmentioning

confidence: 99%

The lungs were on fire: a pilot study of 18F-FDG PET/CT in idiopathic-inflammatory-myopathy-related interstitial lung disease

Liang

Liu

et al. 2021

Arthritis Res Ther

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Background Interstitial lung disease (ILD) and its rapid progression (RP) are the main contributors to unfavourable outcomes of patients with idiopathic inflammatory myopathy (IIM). This study aimed to identify the clinical value of PET/CT scans in IIM-ILD patients and to construct a predictive model for RP-ILD. Methods Adult IIM-ILD patients who were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU), from 1 January 2017 to 31 December 2020 were reviewed. PET/CT scans and other characteristics of patients who met the inclusion and exclusion criteria were collected and analysed. Results A total of 61 IIM-ILD patients were enrolled in this study. Twenty-one patients (34.4%) developed RP-ILD, and 24 patients (39.3%) died during follow-up. After false discovery rate (FDR) correction, the percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P = 0.014), bilateral lung mean standard uptake value (SUVmean, P = 0.014) and abnormal mediastinal lymph node (P = 0.045) were significantly different between the RP-ILD and non-RP-ILD groups. The subsequent univariate and multivariate logistic regression analyses verified our findings. A “DLM” model was established by including the above three values to predict RP-ILD with a cut-off value of ≥ 2 and an area under the curve (AUC) of 0.905. Higher bilateral lung SUVmean (P = 0.019) and spleen SUVmean (P = 0.011) were observed in IIM-ILD patients who died within 3 months, and a moderate correlation was recognized between the two values. Conclusions Elevated bilateral lung SUVmean, abnormal mediastinal lymph nodes and decreased DLCO% were significantly associated with RP-ILD in IIM-ILD patients. The “DLM” model was valuable in predicting RP-ILD and requires further validation.

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

Cited by 38 publications

References 43 publications

Treatment Options for Interstitial Lung disease associated with Connective Tissue Disease

Treatment Options for Interstitial Lung disease associated with Connective Tissue Disease

Old wine in new wineskins. Hamman-Rich syndrome has much in common with severe COVID-19: Do they share any link of pathogenesis or depend on the similar aetiological factor(s)?

The lungs were on fire: a pilot study of 18F-FDG PET/CT in idiopathic-inflammatory-myopathy-related interstitial lung disease

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