Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome.

Interstitial lung disease (ILD) is a common extra-muscular manifestation of antisynthetase (AS) syndrome. ILD prevalence is higher with anti-Jo-1 antibody positivity. Data on long-term outcomes in these patients are lacking. Over 15 years, we identified subjects with anti-Jo-1 positive AS syndrome and ILD. Demographics, pulmonary function testing (PFT), high-resolution computed tomography (HRCT), histopathology, and long-term survival were analyzed. We identified 103 subjects (mean age 49.2 years, female predominance [70%]). The predominant myopathy was polymyositis (64%) followed by dermatomyositis (24%). In approximately half of studied subjects, AS syndrome and ILD were diagnosed within 6 months of each other. The majority had restriction on PFTs (98%). Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern (52%), followed by NSIP overlapping with organizing pneumonia (OP) (22%). Thirty-nine subjects had biopsy data. Ten-year survival was 68%. Multivariable analysis adjusted for age at ILD diagnosis, gender, FVC and DLCO, revealed that male gender (HR = 2.60, p = 0.04) and DLCO at presentation (HR = 0.94, p = 0.05) significantly predicted mortality. We present a large cohort of anti-Jo-1 positive AS syndrome with ILD and note good overall survival.

Zamora AC ，Hoskote SS ，Abascal-Bolado B ，White D ，Cox CW ，Ryu JH ，Moua T ... -  《-》

Clinical characteristics of patients with anti-EJ antisynthetase syndrome associated interstitial lung disease and literature review.

Interstitial lung disease (ILD) is an extra-muscular manifestation of antisynthetase syndrome (ASS). The aim of this study is to analyze the clinical characteristics of anti-EJ associated ILD in a large cohort of patients. Retrospective cohort study of patients with anti-EJ associated ILD. All available data of clinical and laboratory characteristics, pulmonary function tests, laboratory parameters, high resolution computed tomography (HRCT) and treatment were collected and analyzed from medical records. We identified 51 subjects. Average age at diagnosis was 55.6 years. Thirty-two of 51 patients were female. Concurrent autoantibodies against Ro52 were seen in 92.2% patients studied. HRCT patterns were mainly non-specific interstitial pneumonia (NSIP). The predominant myositis subset was amyopathic dermatomyositis (ADM) (41.2%) followed by dermatomyositis and polymyositis. Thirty-four patients improved on corticosteroids alone or in combination with immunosuppressive drugs as treatment and ten patients were stabilized. However, eleven patients (21.6%) initially improved during 12.0 ± 4.4 months, then progressively recurred despite steroid treatment (mean prednisone dose 11.6 ± 3.5 mg). The recurrence group included a significantly higher proportion of patients with NSIP pattern (p < 0.05). In the literature review the most common manifestations of anti-EJ ASS were ILD (89.3%) and myositis (58.9%). ILD are common features of the anti-EJ ASS. Patients with anti-EJ ILD often had an onset of ILD with lower lung-predominant opacities and NSIP. Although the disease responded well to the initial combination therapy of corticosteroid and immunosuppressant, recurrence was frequent. NSIP pattern was significantly more frequent in the recurrence group.

Liu Y ，Liu X ，Xie M ，Chen Z ，He J ，Wang Z ，Dai J ，Cai H ... -  《-》

Clinical features of anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China.

Zhan X ，Yan W ，Wang Y ，Li Q ，Shi X ，Gao Y ，Ye Q ... -  《-》

Long-term pulmonary outcomes and mortality in idiopathic inflammatory myopathies associated with interstitial lung disease.

Cobo-Ibáñez T ，López-Longo FJ ，Joven B ，Carreira PE ，Muñoz-Fernández S ，Maldonado-Romero V ，Larena-Grijalba C ，Cubas IL ，Muriel ET ，Mateos CB ，de la Peña Lefebvre PG ，Gomez-Gomez A ，Nogal LB ，Pérez A ，Almodovar R ，Lojo L ，Ruiz-Gutiérrez L ，López-Robledillo JC ，García de Yébenes MJ ，Nuño-Nuño L ... -  《-》

Interstitial lung disease in anti-Jo-1 patients with antisynthetase syndrome.

To assess the outcome of interstitial lung disease (ILD) in anti-Jo-1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti-Jo-1 patients with and without ILD. Ninety-one anti-Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans. Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti-Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels. Our findings confirm that ILD is a frequent complication in anti-Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti-Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.

Marie I ，Josse S ，Hatron PY ，Dominique S ，Hachulla E ，Janvresse A ，Cherin P ，Mouthon L ，Vittecoq O ，Menard JF ，Jouen F ... -  《-》