Combination therapy with TOF might have the potential to control refractory anti-MDA5 Ab+ DM-ILD.

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BackgroundRheumatoid arthritis (RA) is a systemic inflammatory disease that affects joints and various organs including the lung. The pulmonary involvement is critical for prognosis of the patients and decision of the treatment. Moreover, the pulmonary involvement showed various abnormalities such as interstitial pneumonia (ILD) and airway disease (AD). Importantly, a pulmonary abnormality coexists with other ones in RA patients. There have been large numbers of studies on the prevalence of pulmonary abnormalities and clinical features of patients with these lesions. However, it remains to be elucidated what existence pattern of pulmonary abnormalities RA patients have.ObjectivesTo reveal the existence pattern of the pulmonary abnormalities in RA patients using cluster analysis, and to clarify the clinical features of patients with multiple pulmonary abnormalities.MethodsSubjects were consecutive 208 RA patients who were treated with bDMARDs as the first one from Feb. 2004 to Sep. 2015 in our department and received HRCT scan before and after the therapy. Pulmonary abnormalities were classified into 4 categories (ILD, nodular lesions, AD and other) and 20 lesions such as ground-glass opacity, reticular pattern, bronchiolitis and bronchiectasis and were examined their existence and distribution. Cluster analysis was conducted according to the existence of the lesions by Ward method. Clinical features were analyzed through reviewing medical records.ResultsSubjects were 208 RA cases (M/F; 64/144, mean age 59.2 year-old, disease duration 13.1 years). Pulmonary lesions were found in 146 (70.2%) of RA patients before treatment Imaging findings were 81of ILD (39%),45 of nodular lesion (21.6%) and 115 of AD (55%). Cluster analysis showed 6 clustered (Fig.), 1; no pulmonary lesions, 2; AD without bronchoectasia, 3; AD with bronchoectasia, 4; AD with curved linear opacities, 5; AD with nodular lesions, and 6; reticular pattern with AD.AD was common abnormalities and coexisted with other pulmonary lesions in RA. AD was found in 79%, 78% and 71% of patients with pulmonary abnormalities, ILD and nodular lesions, respectively. AD alone, AD with ILD, and AD with nodular pattern were found in 16.3%, 8.6% and 28.9%, respectively, while patients without pulmonary lesions were 29.8% in RA. AD was frequently associated with ILD and nodule compare to non-AD.No differences were found in gender, smoking history, disease duration and disease activity between patients with and without AD. New emergence or exacerbation of pulmonary abnormalities developed in AD patients compared to those without pulmonary abnormalities or AD. No significant differences were found in clinical features, among AD alone, AD with ILD and AD with nodules.ConclusionsPulmonary abnormalities were found in 70% in RA. AD was found in 55% of RA patients and coexisted with other pulmonary lesions such as ILD and nodular lesions. Patients with AD frequently showed newly emerging or worsening pulmonary lesions, regardless of the coexistence of other pulmonary lesions. Th...

BackgroundDermatomyositis (DM)/ polymyoitis (PM) are systemic diseases characterized by muscle inflammation, which shows varieties of clinical symptoms and signs. We have experienced cases of DM/PM with generalized edema as reported previously by others (1). Moreover, we found that there were many myositis patients who lost their body weight (BW) after starting of high dose glucocorticoid (GC) therapy. Thus, we hypothesized that hidden generalized edema is a characteristic clinical feature of myositis.ObjectivesTo determine whether generalized edema is a hidden clinical feature of myositis. If so, what myositis patients have the feature.MethodsThe study was a retrospective observation study. The subjects were consecutive 67 of DM/PM and 53 of SLE patients who diseases for the first time, admitted our department from April 2007 to September 2016 and received immunosuppressive therapy including over 30mg/day GC. The patients were excluded who had cardiogenic or nephrogenic edema or whose BW data was not available. To detect hidden generalized edema caused by inflammation, we examined the change in BW within 2 weeks after starting immunosuppressive therapy. The clinical features of DM/PM patients with/without BW change were examined through reviewing medical record.ResultsThe included subjects were DM/PM 66 patients (M/F; 18/48 with a mean age of 59.4 y.) and SLE 40 patients (M/F;14/26 with a mean age of 49.8 y.). The body weight of DM/PM and SLE were 56.4±14.0 and 54.7±10.9 kg, respectively.Decrease in BW within 2weeks after starting the therapy were 3.02±2.99kg of DM/PM and 0.85±2.87 kg of SLE, which was larger in DM/PM compared to SLE (Fig A). The numbers of patients who lost BW more than 2kg within the 2 weeks were 42 in DM/PM (64%) and 14 in SLE (35%).Serum albumin levels were slightly decreased by 0.18 g/ dl (0.06 to 0.30; 95% CI) in DM/PM, while no significant change was detected in SLE.In myositis, change in BW was similar among DM, amyopathic DM (ADM) and PM (Fig.B). Moreover, no differences were found in the change of BW between patients with and without male sex, malignancy, interstitial pneumonia, anti-ARS Ab and anti-MDA5Ab. Additionally, between patients with and without BW loss more than 2kg, no differences were found in age, serum TP, Alb and CRP levels before and after treatment and prognosis.ConclusionsDM/PM patients lose BW by immunosuppressive therapy including GC, which indicates the existence of hidden generalized edema that might be a characteristic clinical feature in inflammatory myopathy.References Tu J1, McLean-Tooke A, Junckerstorff R. Increasing recognition of dermatomyositis with subcutaneous edema - is this a poorer prognostic marker? Dermatol Online J. 2014;20:21244. Disclosure of InterestNone declared