Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality. @ERSpublications In rheumatoid-arthritis associated interstitial lung disease, physiology, and not HRCT pattern, predicts mortality

Rationale: Early physical therapy (PT) interventions may benefit patients with acute respiratory failure by preventing or attenuating neuromuscular weakness. However, the optimal dosage of these interventions is currently unknown.Objectives: To determine whether an intensive PT program significantly improves long-term physical functional performance compared with a standard-of-care PT program.Methods: Patients who required mechanical ventilation for at least 4 days were eligible. Enrolled patients were randomized to receive PT for up to 4 weeks delivered in an intensive or standard-of-care manner. Physical functional performance was assessed at 1, 3, and 6 months in survivors who were not currently in an acute or long-term care facility. The primary outcome was the Continuous Scale Physical Functional Performance Test short form (CS-PFP-10) score at 1 month. Measurements and Main Results:A total of 120 patients were enrolled from five hospitals. Patients in the intensive PT group received 12.4 6 6.5 sessions for a total of 408 6 261 minutes compared with only 6.1 6 3.8 sessions for 86 6 63 minutes in the standard-of-care group (P , 0.001 for both analyses). Physical function assessments were available for 86% of patients at 1 month, for 76% at 3 months, and for 60% at 6 months. In both groups, physical function was reduced yet significantly improved over time between 1, 3, and 6 months. When we compared the two interventions, we found no differences in the total CS-PFP-10 scores at all three time points (P = 0.73, 0.29, and 0.43, respectively) or in the total CS-PFP-10 score trajectory (P = 0.71).Conclusions: An intensive PT program did not improve long-term physical functional performance compared with a standard-of-care program.Clinical trial registered with www.clinicaltrials.gov (NCT01058421).

Objective Small series suggest mycophenolate mofetil (MMF) is well tolerated and may be an effective therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). We examined the tolerability and longitudinal changes in pulmonary physiology in a large and diverse cohort of patients with CTD-ILD treated with MMF. Methods We identified consecutive patients evaluated at our center between January 2008 and January 2011 and prescribed MMF for CTD-ILD. We assessed safety and tolerability of MMF and used longitudinal data analyses to examine changes in pulmonary physiology over time, before and after initiation of MMF. Results We identified 125 subjects treated with MMF for a median 897 days. MMF was discontinued in 13 subjects. MMF was associated with significant improvements in estimated percentage of predicted forced vital capacity (FVC%) from MMF initiation to 52, 104, and 156 weeks (4.9% ± 1.9%, p = 0.01; 6.1% ± 1.8%, p = 0.0008; and 7.3% ± 2.6%, p = 0.004, respectively); and in estimated percentage predicted diffusing capacity (DLCO%) from MMF initiation to 52 and 104 weeks (6.3% ± 2.8%, p = 0.02; 7.1% ± 2.8%, p = 0.01). In the subgroup without usual interstitial pneumonia (UIP)-pattern injury, MMF significantly improved FVC% and DLCO%, and in the subgroup with UIP-pattern injury, MMF was associated with stability in FVC% and DLCO%. Conclusion In a large diverse cohort of CTD-ILD, MMF was well tolerated and had a low rate of discontinuation. Treatment with MMF was associated with either stable or improved pulmonary physiology over a median 2.5 years of followup. MMF appears to be a promising therapy for the spectrum of CTD-ILD.

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Objectives To describe the clinical features of patients who presented with “idiopathic” interstitial pneumonia but who were ultimately diagnosed with anti-synthetase syndrome based on clinical features and positive anti-PL-7 or -PL-12 antibodies. Methods Over a 24 month period, in our interstitial lung disease (ILD) program, we evaluated 37 patients who presented with clinical features suggestive of anti-synthetase (AS) syndrome, negative anti-JO-1 antibodies, and who were assessed for other anti-tRNA synthetase (anti-tRS) antibodies. All data were abstracted from the medical record. Results Nine (24%) were confirmed to have non-anti-Jo-1 positive AS syndrome based on clinical features and the presence of other anti-tRS antibodies (seven with anti-PL-7 and two with anti-PL-12 antibodies). Five were women; seven were Caucasian. All nine presented with dyspnea as the initial symptom and with ILD as the first manifestation. Elevated CPK was identified in three patients (median 75, range 22–925), but only two had muscle weakness. Pulmonary physiology revealed restriction (forced vital capacity 60% of predicted) and impaired gas transfer (diffusing capacity for carbon monoxide 40% of predicted). All had similar findings on thoracic HRCT scans, with extreme basilar predominance of abnormalities and patterns suggestive of non-specific interstitial pneumonia and organizing pneumonia. Immunomodulatory therapies were used to treat the ILD—responses were variable, but some subjects clearly improved. Conclusion Anti-PL-7 and PL-12 antibodies may be more common among patients presenting with “idiopathic” interstitial pneumonia than formerly considered and should be checked in patients with features of AS syndrome despite a negative anti-nuclear or anti-JO-1 antibodies. Further research is needed to advance understanding of anti-PL-7 or anti PL-12 positive AS syndrome, including its prognosis, optimal approaches to therapy, and to determine how its course differs from other forms of ILD.

In patients with fibrotic lung disease and an acute respiratory decline, a detailed diagnostic evaluation revealed a potential infectious aetiology in up to one-third of cases. However, there was no association between this finding and outcomes in these patients. One-year survival was dismal in patients who suffered an acute respiratory decompensation.

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