Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: A single centre study and review of the literature

Francesca Ingegnoli, Chiara Lubatti, Anna Ingegnoli, Patrizia Boracchi, Silvana Zeni, Pier Luigi Meroni

Research output: Contribution to journalArticle

Abstract

Interstitial lung disease (ILD) is a frequent complication of inflammatory myopathies with high rates of morbidity and mortality. Antibodies against aminoacyl-tRNA-synthetases are the strongest predictive factors in ILD. In this study, we reviewed the literature and we retrospectively analysed high-resolution computed tomography (HRCT) findings in a cohort of 131 consecutive subjects: 75 with polymyositis (PM), 43 with dermatomyositis (DM), one with amyophatic PM, two with paraneoplastic syndromes, and 10 with overlapping syndromes. The inclusion criteria were PM/DM, anti-Jo1 antibody positivity, and HRCT-assessed ILD. The effect of 12. months' treatment with cyclophosphamide (CYC) or cyclosporin A (CsA) plus steroids was assessed by comparing baseline and follow-up HRCT scans for evidence of stability, improvement or worsening. Fifteen patients (11.5%) had ILD and were Jo-1 positive. They were all women with PM, and had a mean age of 47.33. years and a median duration of symptoms of 7.26. months. At baseline, HRCT showed ground-glass attenuations in eight cases, septal thickening in seven, and honeycombing in four. Twelve months after diagnosis, ILD had worsened in nine patients (60%; exact confidence interval [ECI] 32-84) and was stable in four (two patients were lost to follow-up). Seven of the 15 patients were treated with CsA, and 12-month HRCT revealed a worsening in ILD in five cases (71%; ECI 0.29-0.96); ILD also worsened (ECI 16-84) in four of the eight patients treated with CYC pulses (50%). The evolution of the HRCT findings was not significantly different between the two groups. Our findings confirm that ILD is a common early manifestation in patients with Jo1-positive PM. Over twelve months, HRCT showed worsening ILD in most of our patients, with no difference in the HRCT changes between those treated with CYC or CsA.

Original languageEnglish
Pages (from-to)335-340
Number of pages6
JournalAutoimmunity Reviews
Volume11
Issue number5
DOIs
Publication statusPublished - Mar 2012

Fingerprint

Polymyositis
Interstitial Lung Diseases
Anti-Idiotypic Antibodies
Tomography
Cyclophosphamide
Cyclosporine
Dermatomyositis
Confidence Intervals
Paraneoplastic Syndromes
Amino Acyl-tRNA Synthetases
Myositis
Lost to Follow-Up
Glass
Steroids
Morbidity
Mortality

Keywords

  • High-resolution computed tomography
  • Interstitial lung disease
  • Polymyositis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients : A single centre study and review of the literature. / Ingegnoli, Francesca; Lubatti, Chiara; Ingegnoli, Anna; Boracchi, Patrizia; Zeni, Silvana; Meroni, Pier Luigi.

In: Autoimmunity Reviews, Vol. 11, No. 5, 03.2012, p. 335-340.

Research output: Contribution to journalArticle

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abstract = "Interstitial lung disease (ILD) is a frequent complication of inflammatory myopathies with high rates of morbidity and mortality. Antibodies against aminoacyl-tRNA-synthetases are the strongest predictive factors in ILD. In this study, we reviewed the literature and we retrospectively analysed high-resolution computed tomography (HRCT) findings in a cohort of 131 consecutive subjects: 75 with polymyositis (PM), 43 with dermatomyositis (DM), one with amyophatic PM, two with paraneoplastic syndromes, and 10 with overlapping syndromes. The inclusion criteria were PM/DM, anti-Jo1 antibody positivity, and HRCT-assessed ILD. The effect of 12. months' treatment with cyclophosphamide (CYC) or cyclosporin A (CsA) plus steroids was assessed by comparing baseline and follow-up HRCT scans for evidence of stability, improvement or worsening. Fifteen patients (11.5{\%}) had ILD and were Jo-1 positive. They were all women with PM, and had a mean age of 47.33. years and a median duration of symptoms of 7.26. months. At baseline, HRCT showed ground-glass attenuations in eight cases, septal thickening in seven, and honeycombing in four. Twelve months after diagnosis, ILD had worsened in nine patients (60{\%}; exact confidence interval [ECI] 32-84) and was stable in four (two patients were lost to follow-up). Seven of the 15 patients were treated with CsA, and 12-month HRCT revealed a worsening in ILD in five cases (71{\%}; ECI 0.29-0.96); ILD also worsened (ECI 16-84) in four of the eight patients treated with CYC pulses (50{\%}). The evolution of the HRCT findings was not significantly different between the two groups. Our findings confirm that ILD is a common early manifestation in patients with Jo1-positive PM. Over twelve months, HRCT showed worsening ILD in most of our patients, with no difference in the HRCT changes between those treated with CYC or CsA.",
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