Pulmonary involvement in antisynthetase syndrome

Gasparotto, Michela; Gatto, Mariele; Saccon, Francesca; Ghirardello, Anna; Iaccarino, Luca; Doria, Andrea

doi:10.1097/bor.0000000000000663

Cited by 44 publications

(47 citation statements)

References 80 publications

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“…NSIP has been reported as the most common pattern on HRCT in patients with ILD secondary to autoimmune etiology, in particular in patients with antisynthetase syndrome, followed by OP. Less common is the occurrence of usual interstitial pneumonia [8][9][10]. In our case series, only half of the patients presented with cellular NSIP and OP patterns.…”

Section: Discussionmentioning

confidence: 53%

“…Less commonly, usual interstitial pneumonia (UIP) and acute interstitial pneumonia (AIP) have been reported. Nevertheless, the evidence is currently based on retrospective studies or case reports [8][9][10]. In our clinical practice, we often encountered patients finally diagnosed with ASS who had HRCT findings reported to be less frequent in this syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study

et al. 2020

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Purpose Antisynthetase syndrome (ASS) is a rare systemic autoimmune condition associated to the presence of antiaminoacyl-tRNA synthetase antibodies. Interstitial lung disease (ILD) is the most prevalent manifestation of ASS and is a major determinant of morbidity and mortality. The aim of this study was to describe the radiological characteristics of patients with ASS-associated-ILD in our institution. Materials and methods Medical records from 2014 to 2020 were retrospectively reviewed and patients with a diagnosis of ASS and evidence of ILD on HRCT were included. HRCT images were reviewed by two thoracic radiologists in consensus. Five HRCT patterns were defined: cellular non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), mixed NSIP/OP pattern, acute interstitial pneumonia (AIP) pattern and fibrotic pattern. Descriptive statistics was calculated for all variables. Results Twenty-two patients with ASS who met inclusion criteria were included. The disease presented with the typical triad of ASS in 45% of patients, 55% had ILD only at the onset. Cellular NSIP was present in 27% of patients, OP in 23%, mixed NSIP/OP in 9%, AIP in 18% and a fibrotic pattern in 23%. Conclusion HRCT findings in ASS-associated ILD are often non-specific; nevertheless, it is important to consider this diagnosis, especially in patients presenting with acute onset of symptoms.

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Section: Discussionmentioning

confidence: 53%

Section: Introductionmentioning

confidence: 99%

Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study

et al. 2020

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“…Mechanic's hand is major sign of rheumatic diseases. Previous research suggested that pulmonary arterial hypertension has a low prevalence in ASS-IP compared with other CTDs, but it independently affects prognosis and survival [21].…”

Section: Discussionmentioning

confidence: 96%

Quantitative High-resolution Computed tomography Analysis of Antisynthetase Syndrome with Interstitial Pneumonia

Peng

et al. 2021

Preprint

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Background Non-specific interstitial pneumonia (NSIP) combined with organizing pneumonia (OP) pattern has been confirmed in pathology or radiology. This study is to analyze the correlation between high-resolution computed tomography (HRCT) quantitative indexes (QI) and pulmonary function test parameters (PFTs), and compare differences of HRCT patterns in Antisynthetase Syndrome with interstitial pneumonia (ASS-IP).Methods Data of ASS-IP patients admitted to respiratory department of Ping Jin hospital from January 2014 to December 2019 were retrospectively reviewed.Results 21 ASS-IP patients were enrolled in this study and 3 patients were NSIP, 9 patients were OP and 8 patients were NSIP overlap OP (NSIP/OP) pattern. (1) Volume, Weight percentages of the extracted whole lung volume with attenuation values (V%, W %) and mean lung attenuation (MLA) of non-aerated area is 5.69%, 16.43% and -11.76 Hounsfield unit (Hu). Total MLA of attenuation values(MLAtotal) is -678.97 Hu.(2)FVC and MLA of poorly aerated（r=0.58,P=0.048）, FEV1 and MLA（r=0.79,P=0.001）of poorly aerated, FEV1/FVC（r=0.23,P=0.01） and MLA of normally lung aerated, have linear correlation .(3) DLCO (P=0.049), volume of poorly aerated (Vfibrosis%,P =0.03), and weight of hyper inflated (Whyper%,P=0.02) percentages, and MLAtotal (P=0.01) have significant statistic differences between patients with NSIP/OP or OP patterns, but the therapy improvement time(P=0.41) had no difference. Conclusions QI of HRCT has a good correlation with PFTs and partly reference value to pathological classification of ASS-IP. NSIP/OP may be a common pattern in ASS-IP, fibrosis severity of which is more severe than OP.

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“…RPILD typically occurs in association with anti-MDA5 antibodies, whereas ILD with insidious onset, a pattern of non-specific interstitial pneumonia (NSIP) and adequate response to immunosuppression is found in the context of anti-aminoacyl-tRNA-synthetase antibodies, most frequently anti-Jo-1 (histidyl-tRNA synthetase) (Sato et al, 2009;Koreeda et al, 2010;Chen et al, 2013;Gasparotto et al, 2019). Intriguingly, the epitopes targeted by these autoantibodies reside in two unrelated cytoplasmic proteins: MDA5 represents a helicase that binds double-stranded RNA and functions as an antiviral pattern recognition receptor (Reikine et al, 2014), whereas histidyl-tRNA-synthetase generates histidyl-tRNA, which incorporates histidine into polypeptide chains (Freist et al, 1999).…”

Section: Introductionmentioning

confidence: 99%

Iacta Alea Est: The Inexorable Advance of Tofacitinib in the Treatment of Dermatomyositis-Associated Rapidly Progressive Interstitial Lung Disease. A Case Report

et al. 2020

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Rapidly progressive interstitial lung disease is typically associated with clinically amyopathic dermatomyositis and the anti-melanoma differentiation associated gene 5 antibody, a condition with high mortality and resistance to classic immunosuppression. Recent reports have described the efficacy of the Janus kinase inhibitor tofacitinib in the treatment of rapidly progressive interstitial lung disease in anti-melanoma differentiation associated gene 5 antibody-positive clinically amyopathic dermatomyositis. It is uncertain, however, whether tofacitinib alters the course of rapidly progressive interstitial lung disease in other variants of dermatomyositis that are unrelated to the anti-melanoma differentiation associated gene 5 antibody and whether the early addition of the anti-fibrotic tyrosine kinase inhibitor nintedanib interferes with the development of fibrosis. To answer these questions, we present and discuss the case of an elderly woman who presented with a flare of dermatomyositis sine myositis. Based upon the detection of anti-Jo-1 antibodies and the absence of anti-melanoma differentiation associated gene 5 antibodies, anti-synthetase syndrome was diagnosed. While the cutaneous manifestations quickly resolved with prednisone, azathioprine and tacrolimus, the respiratory function paradoxically and rapidly deteriorated, and invoked the use of tofacitinib. Markedly raised ferritin levels and a severe numerical deficiency of circulating natural killer cells paralleled the acute lung inflammation, which was reflected by 18F-fluorodeoxyglucose hypermetabolism on positron emission tomography/CT. Tofacitinib lead to a prompt clinical recovery, with a reduction in oxygen requirement, correction of hyperferritinemia, reversal of the natural killer cell deficiency, and a decrease in 18F-fluorodeoxyglucose uptake in the affected lung segments. Subsequently, nintedanib was added at a point in time when inflammation subsided. Apart from cytomegalovirus reactivation no adverse events occurred. In conclusion, tofacitinib reversed the pronounced inflammatory component of anti-Jo-1 antibody-positive, anti-melanoma differentiation associated gene 5 antibody-negative rapidly progressive interstitial lung disease, confirming that Janus kinase signaling pathways are critically involved in the pathogenesis of rapidly progressive interstitial lung disease, apparently independently of the targeted autoantigen. Although some improvement in pulmonary function was observed, it seems premature to conclusively judge on reversibility or prevention of pulmonary fibrosis by pairing both kinase inhibitors for which an extended follow-up and ideally, prospective and controlled studies are needed.

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Pulmonary involvement in antisynthetase syndrome

Cited by 44 publications

References 80 publications

Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study

Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study

Quantitative High-resolution Computed tomography Analysis of Antisynthetase Syndrome with Interstitial Pneumonia

Iacta Alea Est: The Inexorable Advance of Tofacitinib in the Treatment of Dermatomyositis-Associated Rapidly Progressive Interstitial Lung Disease. A Case Report

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