Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study
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ORIGINAL ARTICLE
Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study Elisa Baratella1 · Cristina Marrocchio2 · Rossella Cifaldi3 · Mario Santagiuliana3 · Alessandro Marco Bozzato2 · Paola Crivelli4 · Barbara Ruaro3 · Francesco Salton3 · Marco Confalonieri3 · Maria Assunta Cova1 Received: 18 May 2020 / Accepted: 9 August 2020 © The Author(s) 2020
Abstract 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. Keywords Antisynthetase syndrome · HRCT · Acute interstitial pneumonia
Introduction Antisynthetase syndrome (ASS) is a rare systemic autoimmune condition classified among the idiopathic inflammatory myopathies, associated to the presence of anti-aminoacyl-transfer RNA synthetase antibodies. These antibodies are directed against enzymes that attach amino acids to * Elisa Baratella [email protected] 1
Department of Radiology, University of Trieste, Strada di Fiume 447, 34128 Trieste, Italy
2
Department of Medicine, Surgery and Health Science, University of Trieste, Strada di Fiume 447, Trieste, Italy
3
Department of Pneumology, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), Strada di Fiume 447, Trieste, Italy
4
Diagnostic Imaging 2, AOU Sassari, viale S. Pietro 43, Sassari, Italy
their cognate transfer ribonucleic acid during polypeptide synthesis. At present, eight antisynthetase antibodies have been identified, the most frequent being anti-Jo1 (histidyl), followed by anti-EJ (glycyl), anti-PL7 (threonyl) and antiPL12 (alanyl) [1]. ASS is characterized by a classical triad of Interstitial Lung Disease (ILD), myositis, and positivity to antisynthetase antibodies and other less specific symptoms such
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