Ryu, Jay H

ObjectiveInterstitial lung disease (ILD) is associated with substantial morbidity in rheumatoid arthritis (RA), but very little is known about its long‐term progression. This study was undertaken to investigate the progression of pulmonary disease using a large single‐center cohort of patients with RA‐associated ILD.MethodsRecords of all patients with RA‐associated ILD seen at Mayo Clinic between 1998 and 2014, with at least 4 weeks follow‐up and at least 1 pulmonary function test, were identified and manually screened for study inclusion. Progression was defined as a diffusing capacity for carbon monoxide (DLco) <40% predicted (or patients whose illness was too advanced to undergo screening) or a forced vital capacity (FVC) <50% predicted. Time to progression was analyzed using the Kaplan‐Meier method.ResultsOf the 167 patients included in the study, 81 (49%) were female, with a mean ± SD age of 67 ± 10 years at diagnosis of ILD. Median follow‐up time from diagnosis of ILD was 3.3 years (range 0.01–14.8). One‐third of the patients required supplemental oxygen, 40% developed DLco <40% predicted, and 22% developed FVC <50% predicted within 5 years after ILD diagnosis. Usual interstitial pneumonia (UIP) versus nonspecific interstitial pneumonia (NSIP) was a risk factor for DLco progression (hazard ratio 3.29 [95% confidence interval 1.28–8.41]). Lower DLco and FVC at baseline increased the risk for progression to DLco <40% predicted and FVC <50% predicted, and higher rates of change in the first 6 months also increased the risk of progression.ConclusionProgressive loss of pulmonary function is common in RA‐associated ILD and is worse in patients with UIP than in those with NSIP. Predictors of progression in patients with RA‐associated ILD may aid clinicians in identifying patients at highest risk for progression of ILD.

ObjectiveTo investigate clinical and pathologic aspects of IgG4‐related disease (IgG4‐RD) in non‐Asian populations.MethodsWe conducted a retrospective review of the medical records of patients with IgG4‐RD who presented to an academic medical center from January 1994 to September 2012.ResultsAmong 166 patients identified, the median age at diagnosis was 61 years (interquartile range [IQR] 49–70 years), 75% were male, and 80% were white. The median number of organs involved was 2 (IQR 2–3). When organs were grouped according to anatomic system, the hepatopancreaticobiliary system was most commonly involved (77%). The median highest serum IgG4 level during the clinical course was 215 mg/dl (IQR 122–466). Forty‐three patients (26%) had a normal serum IgG4 level. Seventy‐nine pathology specimens were available for immunostaining. The median number of IgG4+ cells was 37 (IQR 25–82) per high‐power field, with an IgG4+:IgG+ ratio of 0.50 (IQR 0.32–0.68). Among 151 patients who received medical therapy, 72 (48%) received steroid‐sparing agents because of relapse, recurrence, or corticosteroid intolerance. Of the 66 patients who were newly diagnosed, started on corticosteroid monotherapy as the initial treatment, and followed up at our institution, 30 (45%) experienced relapse or recurrence despite an initial favorable response. The number of organs involved had a significant impact on time to relapse or recurrence, with a hazard ratio of 1.48 (95% confidence interval [95% CI] 1.12–1.93) (P < 0.01) in multivariate analysis. The standardized incidence ratio of malignancy was 4.5 (95% CI 1.5–7.5).Conclusion. IgG4‐RD is a multisystem disorder that commonly affects older men and has a propensity for relapse, recurrence, and malignancy.

Abstract Rationale Surgical lung biopsy is often required for a confident multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF). Alternative, less-invasive biopsy methods, such as bronchoscopic lung cryobiopsy (BLC), are highly desirable. Objectives To address the impact of BLC on diagnostic confidence in the multidisciplinary diagnosis of IPF. Methods In this cross-sectional study we selected 117 patients with fibrotic interstitial lung disease without a typical usual interstitial pneumonia pattern on high-resolution computed tomography. All cases underwent lung biopsies: 58 were BLC, and 59 were surgical lung biopsy (SLB). Two clinicians, two radiologists, and two pathologists sequentially reviewed clinical-radiologic findings and biopsy results, recording at each step in the process their diagnostic impressions and confidence levels. Measurements and Main Results We observed a major increase in diagnostic confidence after the addition of BLC, similar to SLB (from 29 to 63%, P = 0.0003 and from 30 to 65%, P = 0.0016 of high confidence IPF diagnosis, in the BLC group and SLB group, respectively). The overall interobserver agreement in IPF diagnosis was similar for both approaches (BLC overall kappa, 0.96; SLB overall kappa, 0.93). IPF was the most frequent diagnosis (50 and 39% in the BLC and SLB group, respectively; P = 0.23). After the addition of histopathologic information, 17% of cases in the BLC group and 19% of cases in the SLB group, mostly idiopathic nonspecific interstitial pneumonia and hypersensitivity pneumonitis, were reclassified as IPF. Conclusions BLC is a new biopsy method that has a meaningful impact on diagnostic confidence in the multidisciplinary diagnosis of interstitial lung disease and may prove useful in the diagnosis of IPF. This study provides a robust rationale for future studies investigating the diagnostic accuracy of BLC compared with SLB.

OBJECTIVE: Aspiration can cause a variety of pulmonary syndromes, some of which are not well recognized. The objective of this study was to assess the demographic, clinical, radiological, and histopathological correlates of diffuse aspiration bronchiolitis (DAB), a bronchiolocentric disorder caused by recurrent aspiration. METHODS: This was a retrospective study of 20 consecutive patients with DAB seen at the Mayo Clinic in Rochester, Minnesota, between January 1, 1998 and June 30, 2014. RESULTS: The median age of the patients was 56.5 years (range, 22-76 years), and the male/female ratio was 2.3:1.0. In 18 patients, the diagnosis of DAB was based on the results of a lung biopsy; in the 2 remaining patients, it was based on clinical and radiological features, together with documented aspiration observed in a videofluoroscopic swallow study. In 19 patients (95%), we identified predisposing factors for aspiration, including gastroesophageal reflux disease (GERD), drug abuse, and dysphagia. Common presenting features included cough, sputum production, dyspnea, and fever. Twelve patients (60%) had a history of recurrent pneumonia. In all of the patients, chest CT revealed bilateral pulmonary infiltrates consisting of micronodules and tree-in-bud opacities. In the majority of patients, interventions aimed at preventing recurrent aspiration (e.g., anti-GERD therapies) led to improvement in the symptoms of DAB. CONCLUSIONS: Young to middle-aged subjects with recognizable predisposing factors for aspiration and who report a history of recurrent pneumonia are at increased risk for DAB. Although DAB is not well recognized, certain chest CT features are characteristic of the disorder.