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.

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.

Ingegnoli F ，Lubatti C ，Ingegnoli A ，Boracchi P ，Zeni S ，Meroni PL ... -  《-》

The prognostic value of HRCT in myositis-associated interstitial lung disease.

Polymyositis and dermatomyositis-associated interstitial lung disease (PM/DM-ILD) can have variable courses. We evaluated the prognostic value of high-resolution computed tomography (HRCT) in PM/DM-ILD. The cases of 51 patients newly diagnosed with PM/DM-ILD were retrospectively reviewed. HRCT images at diagnosis were categorized into four radiological patterns based on the major findings and distributions of these abnormalities, and the disease extent on HRCT was scored. The impact of HRCT findings and other clinical parameters on day 90 and overall mortality were analyzed. Of the 51 patients (11 with polymyositis and 40 with dermatomyositis), the lower consolidation/ground-glass attenuation (GGA) pattern was observed in 21 patients (41%), lower reticulation was observed in 23 patients (45%), random GGA was observed in four patients (8%), and other patterns were observed in three patients (6%). Twenty-one patients (42%) were positive for anti-CADM-140. The lower consolidation/GGA pattern, clinically amyopathic dermatomyositis, fever (≥38.0 °C), ferritin levels >500 ng/mL, and the presence of anti-CADM-140 were significantly associated with 90-day mortality in univariate analysis. Multivariate analysis revealed that the lower consolidation/GGA pattern (odds ratio, 23.1; P = 0.02) and the presence of anti-CADM-140 (odds ratio, 14.1; P = 0.03) were independent predictors of 90-day mortality. This HRCT pattern was also associated with a higher 90-day morality rate among anti-CADM-140-positive patients. The lower consolidation/GGA pattern was also associated with overall mortality in univariate analysis, whereas only the presence of anti-CADM-140 was an independent determinant of overall mortality in multivariate analysis. HRCT patterns at diagnosis can help predict the prognosis of patients with PM/DM-ILD as well as the presence of anti-CADM-140.

Tanizawa K ，Handa T ，Nakashima R ，Kubo T ，Hosono Y ，Aihara K ，Ikezoe K ，Watanabe K ，Taguchi Y ，Hatta K ，Oga T ，Chin K ，Nagai S ，Mimori T ，Mishima M ... -  《-》

Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease.

Myositis-specific autoantibodies (MSAs) are associated with clinical phenotypes in polymyositis/dermatomyositis (PM/DM). No study has investigated the clinical features based on comprehensive MSA assessment in PM/DM-associated interstitial lung disease (ILD). We aimed to determine the practical significance of MSAs in PM/DM-ILD. Sixty consecutive PM/DM-ILD patients were retrospectively analysed. Serum MSAs were comprehensively measured using immunoprecipitation assay. Clinical features and prognosis were compared among MSA subgroups. Twenty-six (43.3%) PM/DM-ILD patients were anti-aminoacyl tRNA-synthetase antibody-positive (anti-ARS-positive), 15 (25.0%) were anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5-positive), 3 (5%) were anti-signal recognition particle antibody-positive, 1 (1.7%) was anti-transcriptional intermediary factor 1-gamma antibody-positive, and 15 (25%) were MSA-negative. There were significant differences in clinical features, including ILD form, serum ferritin and surfactant protein-D levels at ILD diagnosis, and high-resolution CT pattern among the anti-ARS-positive, anti-MDA5-positive and MSA-negative groups. The anti-MDA5-positive group showed the lowest 90-day survival rate (66.7%, anti-MDA5-positive; 100%, anti-ARS-positive; 100%, MSA-negative; P < 0.01). The anti-ARS-positive group had the highest 5-year survival rate (96%, anti-ARS-positive; 66.7%, anti-MDA5-positive; 68.3%, MSA-negative, P = 0.02). Univariate analysis revealed that anti-ARS antibody was associated with better prognosis (HR = 0.45; 95% CI, 0.18-0.89; P = 0.02), whereas anti-MDA5 antibody was associated with poorer prognosis (HR = 1.90; 95% CI, 1.02-3.39; P = 0.04). The comprehensive MSA assessment demonstrated that anti-ARS and anti-MDA5 antibodies were two major MSAs, and the clinical features differed depending on MSA status in PM/DM-ILD. Assessment of anti-ARS and anti-MDA5 antibodies is practically useful for predicting clinical course and prognosis in PM/DM-ILD patients.

Hozumi H ，Fujisawa T ，Nakashima R ，Johkoh T ，Sumikawa H ，Murakami A ，Enomoto N ，Inui N ，Nakamura Y ，Hosono Y ，Imura Y ，Mimori T ，Suda T ... -  《-》

Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease.

Cavagna L ，Caporali R ，Abdì-Alì L ，Dore R ，Meloni F ，Montecucco C ... -  《-》

Clinical characteristics of dermatomyosits/polymyositis associated interstitial lung disease according to the autoantibody.

Kishaba T ，McGill R ，Nei Y ，Ibuki S ，Momose M ，Nishiyama K ，Nagano H ，Yamashiro S ... -  《-》