The Pulmonary Histopathology of Anti-KS Transfer RNA Synthetase Syndrome

Schneider, Frank; Aggarwal, Rohit; Bi, David; Gibson, Kevin F.; Oddis, Chester V.; Yousem, Samuel A.

doi:10.5858/arpa.2013-0667-oa

Cited by 22 publications

(8 citation statements)

References 20 publications

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“…Antibodies against cN1A were described in IBM, PM/DM, Ssc, SLE, and Sjögren's syndrome (range 0-37%). However, associations with ILD have not been identified [26,27,[30][31][32]43,45]. We demonstrated novel data regarding the presence of cN1A antibodies in ILDs, including ASS, HP, and IPF.…”

Section: Discussionmentioning

confidence: 83%

Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease

Moll

Platenburg

Platteel

et al. 2020

JCM

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Connective tissue diseases (CTDs) are an important secondary cause of interstitial lung disease (ILD). If a CTD is suspected, clinicians are recommended to perform autoantibody testing, including for myositis autoantibodies. In this study, the prevalence and clinical associations of novel myositis autoantibodies in ILD are presented. A total of 1194 patients with ILD and 116 healthy subjects were tested for antibodies specific for Ks, Ha, Zoα, and cN1A with a line-blot assay on serum available at the time of diagnosis. Autoantibodies were demonstrated in 63 (5.3%) patients and one (0.9%) healthy control (p = 0.035). Autoantibodies were found more frequently in females (p = 0.042) and patients without a histological and/or radiological usual interstitial pneumonia (UIP; p = 0.010) and a trend towards CTD-ILDs (8.4%) was seen compared with other ILDs (4.9%; p = 0.090). The prevalence of antibodies specific for Ks, Ha, Zoα, and cN1A was, respectively, 1.3%, 2.0%, 1.4%, and 0.9% in ILD. Anti-Ha and Anti-Ks were observed in males with unclassifiable idiopathic interstitial pneumonia (unclassifiable IIP), hypersensitivity pneumonitis (HP), and various CTD-ILDs, whereas anti-cN1A was seen in females with antisynthetase syndrome (ASS), HP, and idiopathic pulmonary fibrosis (IPF). Anti-Zoα was associated with CTD-ILD (OR 2.5; 95%CI 1.11–5.61; p = 0.027). In conclusion, a relatively high prevalence of previously unknown myositis autoantibodies was found in a large cohort of various ILDs. Our results contribute to the awareness that circulating autoantibodies can be found in ILDs with or without established CTD. Whether these antibodies have to be added to the standard set of autoantibodies analysed in conventional myositis blot assays for diagnostic purposes in clinical ILD care requires further study.

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Section: Discussionmentioning

confidence: 83%

Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease

Moll

Platenburg

Platteel

et al. 2020

JCM

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“…In order to bypass the gnosologic conundrum it is tempting to consider the presence of these highly specific antibodies as definitory of an underlying CTD in subjects with ILD, even in the absence of other manifestations. This would be of particular interest in those patients with ARS antibodies who present with a pulmonary-dominant disease and an UIP-like radiographic pattern, as has been described ( 29 ). This situation can be visualized in 1 patient from our IPAF cohort with anti-Jo1 who underwent a diagnostic surgical lung biopsy.…”

Section: Discussionmentioning

confidence: 97%

Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy

Romero-Bueno

Rodríguez-Nieto²,

Miras³

et al. 2023

Front. Med.

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ObjectivesTo assess performance of interstitial pneumonia (IP) with autoimmune features (IPAF) criteria in clinical practice and describe the utility of additional workup in identifying patients with underlying connective tissue diseases (CTD).MethodsWe set a retrospective study of our patients with autoimmune IP, who were allocated to CTD-IP, IPAF or undifferentiated autoimmune IP (uAIP) subgroups according to the updated classification criteria. Presence of the process-related variables comprising IPAF defining domains was scrutinized in all patients, and, when available, the results of nailfold videocapillaroscopy (NVC) were recorded.ResultsThirty nine out of 118 patients, accounting for 71% of former undifferentiated cases, fulfilled IPAF criteria. Arthritis and Raynaud’s phenomenon were prevalent in this subgroup. While systemic sclerosis-specific autoantibodies were restricted to CTD-IP patients, anti-tRNA synthetase antibodies were also present in IPAF. In contrast, rheumatoid factor, anti-Ro antibodies and ANA nucleolar patterns could be found in all subgroups. Usual interstitial pneumonia (UIP) / possible UIP were the most frequently observed radiographic patterns Therefore, the presence of thoracic multicompartimental findings as also performance of open lung biopsies were useful in characterizing as IPAF those UIP cases lacking a clinical domain. Interestingly, we could observe NVC abnormalities in 54% of IPAF and 36% of uAIP tested patients, even though many of them did not report Raynaud’s phenomenon.ConclusionBesides application of IPAF criteria, distribution of IPAF defining variables along with NVC exams help identify more homogeneous phenotypic subgroups of autoimmune IP of potential relevance beyond clinical diagnosis.

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“…Schneider et al 10 Four showed bibasilar reticular infiltrates with traction bronchiectasis and subpleural honeycomb change; one showed bibasilar patchy ground glass infiltrates with subpleural sparing.…”

Section: Nsipmentioning

confidence: 98%

“…4 Anti-KS autoantibodies presumably target asparaginyl-tRNA synthetase, and are found in part of a group of ASS seen in a few patients with IIMs. [5][6][7][8][9][10][11][12] Because of their rarity, there is very limited information available on the clinical presentation patterns and evolution of disease associated with anti-KS associated ASS or anti-KS syndrome. The aim of the present study was to review the published literature on anti-KS syndrome and summarize clinical features and prognosis.…”

Section: Introductionmentioning

confidence: 99%

Interstitial lung disease is a major characteristic of anti-KS associated ant-synthetase syndrome

et al. 2020

Therapeutic Advances in Chronic Disease

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Background: Anti-KS autoantibodies are rare myositis-specific autoantibodies that have been described to target asparaginyl-transfer RNA synthetase. Methods: Here, we review the published literature on critical issues concerning the detection of anti-KS antibodies and the clinical features associated with their presence. Results: Seven articles are reviewed, in all of which immunoprecipitation was employed for the detection of anti-KS antibodies. A total of 47 patients were included; the ratio of females to males was 1.9:1. In total, 46 (98%) of these patients had interstitial lung disease (ILD), which was the sole manifestation in half (50%) of them. Pulmonary pathology revealed 7 (27%) with usual interstitial pneumonia, and 16 (62%) with non-specific pneumonia. Arthritis was present in about one-quarter (26%) of patients, and the incidence of Raynaud’s phenomenon and mechanic’s hands was 19% and 32%, respectively. However, manifestations of myositis were rare (9%). In addition, three (11%) patients had malignant tumors. Most patients responded to glucocorticoid therapy. Conclusions: Identifying anti-KS in patients with ILD may be useful for treatment, but reliable practical detection is needed. Furthermore, clinicians need to be aware of the possible presence of anti-KS antibodies in patients with ILD, either isolated or in combination with myositis.

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The Pulmonary Histopathology of Anti-KS Transfer RNA Synthetase Syndrome

Cited by 22 publications

References 20 publications

Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease

Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease

Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy

Interstitial lung disease is a major characteristic of anti-KS associated ant-synthetase syndrome

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