Association of anti-aminoacyl-transfer RNA synthetase antibody and anti-melanoma differentiation-associated gene 5 antibody with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease

Context The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti–asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. Objective To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. Design We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. Results The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Conclusions Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS–antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

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Cutaneous manifestations of dermatomyositis characterized by myositis-specific autoantibodies

F1000Research ◽

10.12688/f1000research.20646.1 ◽

2019 ◽

Vol 8 ◽

pp. 1951 ◽

Cited By ~ 2

Author(s):

Naoko Okiyama ◽

Manabu Fujimoto

Keyword(s):

Interstitial Lung Disease ◽

Clinical Symptoms ◽

Inflammatory Myopathy ◽

High Specificity ◽

Transfer Rna ◽

Recent Analysis ◽

Cutaneous Manifestations ◽

Different Types ◽

Specific Autoantibody ◽

Aminoacyl Transfer

Dermatomyositis (DM) is an inflammatory myopathy with characteristic skin manifestations, the pathologies of which are considered autoimmune diseases. DM is a heterogeneous disorder with various phenotypes, including myositis, dermatitis, and interstitial lung disease (ILD). Recently identified myositis-specific autoantibodies have been associated with distinct clinical features. For example, anti-melanoma differentiation-associated protein 5 antibodies have a high specificity for clinically amyopathic DM presenting rapidly progressive ILD. Furthermore, anti-transcriptional intermediary factor 1γ antibodies found in patients with juvenile and adult DM are closely correlated with malignancies, especially in elderly patients. Finally, patients with anti-aminoacyl-transfer RNA synthetase antibodies share characteristic clinical symptoms, including myositis, ILD, arthritis/arthralgia, Raynaud’s phenomenon, and fever; thus, the term “anti-synthetase syndrome” is also used. With a focus on the characteristic cutaneous manifestations in each subgroup classified according to myositis-specific autoantibodies, we introduce the findings of previous reports, including our recent analysis indicating that skin eruptions can be histopathologically classified into myositis-specific autoantibody-associated subgroups and used to determine the systemic pathologies of the different types of antibody-associated DM.

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

Therapeutic Advances in Chronic Disease ◽

10.1177/2040622320968417 ◽

2020 ◽

Vol 11 ◽

pp. 204062232096841

Author(s):

Yongpeng Ge ◽

Sizhao Li ◽

Shanshan Li ◽

Linrong He ◽

Xin Lu ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Clinical Features ◽

Malignant Tumors ◽

Usual Interstitial Pneumonia ◽

Transfer Rna ◽

Critical Issues ◽

Pulmonary Pathology ◽

Mechanic’S Hands

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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