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Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients

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Abstract

The strongest predictive factor for the development of interstitial lung disease (ILD) in myositis (IIM) patients is the presence of different antisynthetase antibodies. The aim of this study was to compare the clinical characteristics, radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients. Mean disease duration was 46.6 (range 4–198) months. All patients fulfilled the classification criteria for IIM. All patients underwent chest radiography, pulmonary function tests and HRCT at he time of diagnosis and 6 months after the immunosuppressive therapy. Routine laboratory tests, RF, ANA, anti-ENA, anti-SS-A, anti-histidyl-transfer RNA antibody (Jo-1) measurements were performed in all patients. ILD was found to be present in 70.4% of ASS patients. The anti-SS-A negative ASS group had a more frequent association with alveolitis and responded well to immunosuppressive therapy (p < 0.05). HRCT scan showed more fibrosis in the SS-A positive group. 15.8% of patients died due to pulmonary or cardiac complications. In conclusion, coexistence of anti-SS-A and anti-Jo-1 antibody may be a good predictor for a more coarse and severe ILD in IIM patients who require a more aggressive approach in therapy.

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Abbreviations

ACR:

American College of Rheumatology

ANA:

Antinuclear antibodies

ASS:

Antisynthetase syndrome

BOOP:

Bronchiolitis obliterans and organizing pneumonia

CK:

Creatinin kinase

CRP:

C-Reactive protein

DAD:

Diffuse alveolar damage

DLCO:

Carbon monoxide diffusion capacity

ENA:

Extractable nuclear antigen

FEV1%:

Forced expiratory volume in 1 s

FVC:

Functional vital capacity

HRCT:

High-resolution computer tomography

IF:

Immunofluorescence

IIM:

Idiopathic inflammatory myopathy

ILD:

Interstitial lung disease

IPF:

Idiopathic pulmonary fibrosis

Jo-1:

Histidyl-transfer RNA synthetase

LDH:

Lactate-dehydrogenase

NSIP:

Non-specific interstitial pneumonia

MSA:

Myositis-specific antibody

OM:

Overlap myositis

PFT:

Pulmonary function tests

RA:

Rheumatoid arthritis

RF:

Rheumatoid factor

RNP:

Ribonucleoprotein

SD:

Standard deviation

SSc:

Scleroderma

Sm:

Smith antigen

tRNA:

Transfer ribonucleic acid

UIP:

Usual interstitial pneumonia

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Acknowledgment

This work was supported by grants from the National Research Foundation (OTKA 255989).

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No conflict of interest has been declared by the authors.

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Correspondence to Andrea Váncsa.

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Váncsa, A., Csípő, I., Németh, J. et al. Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients. Rheumatol Int 29, 989–994 (2009). https://doi.org/10.1007/s00296-009-0884-9

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