Ryu, Jay H

BackgroundSpontaneous pneumomediastinum (SP) is an uncommon disorder but has been described in cases with connective tissue diseases (CTDs), most commonly dermatomyositis and polymyositis. We aimed to explore this relationship by analyzing the characteristics of CTD patients with SP.MethodsUsing a computer-assisted search, we identified 25 patients with CTD and SP evaluated between January 1997 and December 2016 at our institution. Clinicoradiologic characteristics, treatment, and outcomes were extracted and chest computed tomography studies analyzed.ResultsWe identified 25 patients with CTD and SP. Median (range) age at SP occurrence was 55 (18–82) years, and 60% of the patients were women. Smoking history was present in 37%. Spontaneous pneumomediastinum was symptomatic in 56% of patients. Eighteen patients (72%) had a known CTD diagnosis, and 20 patients (80%) manifested radiologic evidence of interstitial lung disease. Spontaneous pneumomediastinum diagnosis was achieved with chest radiography in 20% of cases and chest computed tomography in the other cases. Spontaneous pneumomediastinum was managed with expectant observation alone in 22 cases (88%). Four patients (16%) had concomitant pneumothorax, 1 of whom required chest tube drainage. There were no deaths attributable to SP during the median (range) follow-up of 13 (0–174) months. Cumulative survival was 52% at 1 year and 40% at 2 years.ConclusionsSpontaneous pneumomediastinum is an uncommon manifestation of CTD and usually occurs in the presence of interstitial lung disease. Although SP seems to be associated with a relatively benign short-term course, occurrence of SP in CTD patients may be a poor prognostic factor.

ObjectiveConstrictive bronchiolitis (CB) is a poorly understood pulmonary manifestation of primary Sjögren syndrome (pSS). We aimed to clarify the presenting clinicoradiologic features and clinical course of pSS-associated CB through a retrospective cohort study.MethodsWe retrospectively identified 11 patients with pSS and CB (defined by mosaic pattern with air trapping on computed tomography) encountered at our institution over 9 years from 2007 to 2015. Presenting clinical features, laboratory results, radiologic findings, and clinical course were analyzed.ResultsOur patients were mostly women (91%), with a median age 53 years (range, 31–76 years) at the time of pulmonary symptom (dyspnea or cough) onset. Most (64%) were nonsmokers. Average interval between diagnosis of pSS and the onset of pulmonary symptoms was 4.4 years; in 4 patients (36%), CB was the presenting manifestation of pSS. Chest radiographs were normal (67%) or demonstrated hyperinflation (33%). Pulmonary function testing demonstrated airflow obstruction in 7 patients (63%), and 2 patients (18%) had an isolated reduction in diffusing capacity, whereas the remaining 2 patients manifested mixed physiology or normal results. Surgical lung biopsy was performed in 2 patients and confirmed the diagnosis of CB in both. Treatment was variable among our patients included glucocorticoids, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide. Among 6 patients with follow-up computed tomography and pulmonary function, the results remained stable or worsened.ConclusionsConstrictive bronchiolitis is a rare extraglandular manifestation and can sometimes be the presenting manifestation of pSS. This pulmonary manifestation is associated with an indolent clinical course and does not respond well to immunosuppressive therapy.

ObjectiveIgG4‐related disease (IgG4‐RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4‐RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation.MethodsThe RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require urgent treatment or that worsen despite treatment. After a training exercise, investigators reviewed 12 written IgG4‐RD vignettes based on real patients. Investigators calculated both an RI score as well as a physician's global assessment (PhGA) score for each vignette. In a longitudinal assessment, 3 investigators used the RI in 15 patients with newly active disease who were followed up over serial visits after treatment. We assessed interrater and intrarater reliability, precision, validity, and responsiveness.ResultsThe 26 physician investigators included representatives from 6 specialties and 9 countries. The interrater and intrarater reliability of the RI was strong (0.89 and 0.69, respectively). Correlations (construct validity) between the RI and PhGA were high (Spearman's r = 0.9, P < 0.0001). The RI was sensitive to change (discriminant validity). Following treatment, there was significant improvement in the RI score (mean change 10.5 [95% confidence interval (95% CI) 5.4–12], P < 0.001), which correlated with the change in the PhGA. Urgent disease and damage were captured effectively.DiscussionIn this international, multispecialty study, we observed that the RI is a valid and reliable disease activity assessment tool that can be used to measure response to therapy.

Abstract Acute eosinophilic pneumonia (AEP) is an uncommon acute respiratory illness of varying severity that includes presentation as acute respiratory distress syndrome with fatal outcome. AEP may be idiopathic, but identifiable causes include smoking and other inhalational exposures, medications, and infections. The pathogenesis of AEP is poorly understood but likely varies depending on the underlying cause. Airway epithelial injury, endothelial injury, and release of IL-33 are early events that subsequently promote eosinophil recruitment to the lung; eosinophilic infiltration and degranulation appear to mediate subsequent lung inflammation and associated clinical manifestations. Crucial for the diagnosis are the demonstration of pulmonary eosinophilia in the BAL fluid and the exclusion of other disease processes that can present with acute pulmonary infiltrates. Although peripheral blood eosinophilia at initial presentation may be a clue in suggesting the diagnosis of AEP, it may be absent or delayed, especially in smoking-related AEP. Optimal management of AEP depends on the recognition and elimination of the underlying cause when identifiable. The cessation of the exposure to the inciting agent (e.g., smoking), and glucocorticoids represent the mainstay of treating AEP of noninfectious origin. If AEP is recognized and treated in a timely manner, the prognosis is generally excellent, with prompt and complete clinical recovery, even in those patients manifesting acute respiratory failure.

AbstractBackground and objectiveIdiopathic pulmonary fibrosis (IPF) is increasingly diagnosed by clinical and computed tomography (CT) criteria; however, surgical lung biopsy (SLB) may still be required in patients who lack definite CT features of usual interstitial pneumonia (UIP). We reviewed a cohort of elderly patients who underwent SLB, to evaluate the benefit of SLB in diagnosing idiopathic interstitial pneumonia (IIP).MethodsWe searched the pathology records of Mayo Clinic for ambulatory patients at least 75 years old, who underwent SLB between 2000 and 2012 for indeterminate IIP. Histologic slides were reviewed and clinical data were extracted from the record.ResultsA total of 55 patients (35 male) were enrolled. Median (interquartile range) age was 77 (76–80) years. Forced vital capacity was 70 (61–76)% and diffusing capacity of the lungs for carbon monoxide was 48 (42–54)% of predicted. In total, 37 (67%) patients had IPF, including 61% of those with HRCT findings inconsistent with UIP. Thirty‐day mortality was 10% and 90‐day mortality was 15%.ConclusionThe high mortality rate of SLB complicates the risk–benefit analysis in elderly patients with IIP. The expected value of the SLB is probably highest when the HRCT features are inconsistent with UIP, due to the frequent (39%) retrieval of patterns other than UIP.

Summary Background: Pulmonary artery sarcoma (PAS) is a rare malignant neoplasm with an aggressive behavior and often difficult to distinguish from pulmonary thromboembolic disease. Aim: To assess the demographic, clinical, and radiological characteristics of PAS and clinical course. Design and Methods: We retrospectively identified and analyzed all patients with PAS seen at Mayo Clinic in Rochester, Minnesota, between January 1, 1996 and July 31, 2015. Results: Of nine patients (5 women and 4 men; median age 55 years [range, 24–74 years]), eight were diagnosed while alive with surgical (n = 6) or catheter-based endovascular biopsy (n = 2); the remaining patient was diagnosed at postmortem examination. All tumors manifested on CT as filling defect in the main, right or left pulmonary artery and were not associated with peripheral filling defects in seven patients. Seven patients were initially treated with anticoagulant therapy for presumed PE; two patients were suspected to have tumor based on constrictive or expanding effect seen on CT. Five patients died after a mean duration of 2.1 years (10 months–4.25 years) after diagnosis. Two patients are alive with recurrence and metastases of the disease 23 and 27 months after diagnosis, respectively; one remaining patient is alive and disease-free 116 months after diagnosis. Conclusions: Although PAS is associated with a poor prognosis, long-term survival is possible and can be improved by early diagnosis and prompt surgical resection. Atypical appearance on CT including central mass-like lesion without peripheral emboli and constrictive or expanding effect should raise suspicion of PAS.