Gotway, Michael B.

AbstractInterstitial lung disorders are a group of respiratory diseases characterized by interstitial compartment infiltration, varying degrees of infiltration, and fibrosis, with or without small airway involvement. Although some are idiopathic (e.g., idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias, and sarcoidosis), the great majority have an underlying etiology, such as systemic autoimmune rheumatic disease (SARD, also called Connective Tissue Diseases or CTD), inhalational exposure to organic matter, medications, and rarely, genetic disorders. This review focuses on diagnostic approaches in interstitial lung diseases associated with SARDs. To make an accurate diagnosis, a multidisciplinary, personalized approach is required, with input from various specialties, including pulmonary, rheumatology, radiology, and pathology, to reach a consensus. In a minority of patients, a definitive diagnosis cannot be established. Their clinical presentations and prognosis can be variable even within subsets of SARDs.

The use of lymphoid interstitial pneumonia (LIP) as a diagnostic term has changed considerably since its introduction. Utilizing a multi-institutional collection of 201 cases from the last 20 years that demonstrate features associated with the LIP rubric, we compared cases meeting strict histologic criteria of LIP per American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus (“pathologic LIP”; n=62) with cystic cases fulfilling radiologic ATS/ERS criteria (“radiologic LIP”; n=33) and with other diffuse benign lymphoid proliferations. “Pathologic LIP” was associated with immune dysregulation including autoimmune disorders and immune deficiency, whereas “radiologic LIP” was only seen with autoimmune disorders. No case of idiopathic LIP was found. On histology, “pathologic LIP” represented a subgroup of 70% (62/88) of cases with the distinctive pattern of diffuse expansile lymphoid infiltrates. In contrast, “radiologic LIP” demonstrated a broad spectrum of inflammatory patterns, airway-centered inflammation being most common (52%; 17/33). Only 5 cases with radiologic cysts also met consensus ATS/ERS criteria for “pathologic LIP.” Overall, broad overlap was observed with the remaining study cases that failed to meet consensus criteria for “radiologic LIP” and/or “pathologic LIP.” These data raise concerns about the practical use of the term LIP as currently defined. What radiologists and pathologist encounter as LIP differs remarkably, but neither “radiologic LIP” nor “pathologic LIP” present with sufficiently distinct findings to delineate such cases from other patterns of diffuse benign lymphoid proliferations. As a result of this study, we believe LIP should be abandoned as a pathologic and radiologic diagnosis.

Background: Pectus excavatum (PE) deformity involves posterior depression of the sternum and adjacent costal cartilages. The relationship of CT/MRI chest cardiac compression indices used as indications for surgical repair of PE with echocardiographic findings and with improvement in cardiac chamber compression and function post PE repair is unknown. Methods: We evaluated right atrial (RA) size, tricuspid annulus (TA size, right ventricular (RV) outflow tract size as well as RV 2D strain on intra-operative trans-esophageal echocardiography (TEE) immediately pre and post surgical PE repair. Inspiratory and expiratory Haller Index (HI) and cardiac compression indices (CCI) were measured on pre op CT/MR scans (Figure). Offline measurement of chamber dimensions on TEE images was performed and Speckle tracking strain measurements were made using Syngo US Workplace software (Siemens). P<0.05 was considered significant. Results: 60 patients, 73% male, 27% female, age 33.6±10.3 yrs underwent PE repair following CT/MRI from 2010-2014 (Figure). There was a strong correlation between expiratory CT/MRI chest AP diameter and pre-op RA size (panel A). Pre op RA and TA compression on TEE inversely correlated with pre op RV global longitudinal strain rate(RVGLSR) (panels B & C). CCI predicted improvement in RA size post PE repair (panels D & E). In addition pre op RA size on TEE correlated with improvement in RVGLSR post PE repair surgery (panel F). Conclusion: In patients with PE deformity, pre surgical CCI correlated with right-sided cardiac chamber compression on pre op TEE. Severity of chest and cardiac compression also predicted magnitude of improvement in right-sided chamber size and RV longitudinal deformation post PE repair. Our findings provide insight regarding the beneficial effects of PE surgery on right heart chamber compression and improvement in RV function and the important role of intraoperative TEE for patients undergoing PE repair surgery.

Abstract Approximately 5 to 15% of patients with pulmonary coccidioidomycosis subsequently develop pulmonary cavities. These cavities may resolve spontaneously over a number of years; however, some cavities never close, and a small proportion causes complications such as hemorrhage, pneumothorax or empyema. The impact of azole antifungal treatment on coccidioidal cavities has not been studied. Because azoles are a common treatment for symptomatic pulmonary coccidioidomycosis, we aimed to assess the impact of azole therapy on cavity closure. From January 1, 2004, through December 31, 2014, we retrospectively identified 313 patients with cavitary coccidioidomycosis and excluded 42 who had the cavity removed surgically, leaving 271 data sets available for study. Of the 271 patients, 221 (81.5%) received azole therapy during 5-year follow-up; 50 patients did not receive antifungal treatment. Among the 271 patients, cavities closed in 38 (14.0%). Statistical modeling showed that cavities were more likely to close in patients in the treated group than in the nontreated group (hazard ratio, 2.14 [95% CI: 1.45–5.66]). Cavities were less likely to close in active smokers than nonsmokers (11/41 [26.8%] vs 97/182 [53.3%]; P = 0.002) or in persons with than without diabetes (27/74 [36.5%] vs 81/149 [54.4%]; P = 0.01).We did not find an association between cavity size and closure. Our findings provide rationale for further study of treatment protocols in this subset of patients with coccidioidomycosis. Lay Summary Coccidioidomycosis, known as valley fever, is a fungal infection that infrequently causes cavities to form in the lungs, which potentially results in long-term lung symptoms. We learned that cavities closed more often in persons who received antifungal drugs, but most cavities never closed completely.