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A 62-year-old woman came to our hospital with worsening cough and dyspnea over the preceding week, during which time she had been treated with azithromycin and prednisone for suspected pneumonia. She had no fever, chills, or sweats, but her cough had become productive of clear to blood-tinged phlegm during the interval. Medical history was significant for insulin-dependent diabetes mellitus and OSA. She had quit smoking 44 years earlier and had no history of lung disease. She was a bank teller residing in southeastern Minnesota and described no relevant inhalational or environmental exposures, drug use, aspiration, or travels preceding her illness. [ABSTRACT FROM AUTHOR]

Background: Increased risk of spontaneous pneumothorax has been described in patients with Marfan syndrome and has been attributed, in part, to the presence of apical blebs and bullae. Objectives: We assess the risk of pneumothorax and its relationship to the presence of apical blebs and bullae in patients with Marfan syndrome in the era of CT imaging. Methods: A retrospective cohort study was performed of all patients 13 years or older with Marfan syndrome evaluated at the Mayo Clinic, Rochester, Minn., USA, from 1998 through 2008. One hundred and sixty-six patients met the current diagnostic criteria for Marfan syndrome and had chest imaging studies available for review. Results: The median age was 40 years (range 14-71); 37% had a smoking history. Eight of 166 patients (4.8%) had experienced 1 or more episodes of spontaneous pneumothorax, and 2 of these 8 patients had 2 or more episodes. Apical blebs or bullae were identified on radiologic imaging in 16 patients (9.6%). Four of 16 (25%) patients with apical blebs or bullae had a history of spontaneous pneumothorax compared to 4 of 150 patients (2.7%) without blebs or bullae (p = 0.003). Conclusions: The frequency of blebs is relatively low in patients with Marfan syndrome but the risk of pneumothorax is significantly higher in those with radiologically detectable blebs or bullae. Chest CT scanning to identify blebs and bullae may allow risk stratification for pneumothorax in patients with Marfan syndrome. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The article presents a study which evaluates the trends in the incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis (IPF) in the community. Subjects of the population-based study involves 47 adult IPF patients from Olmsted County, Minnesota from 1997 to 2005. Results showed that the incidence of IPF decreased during the study period, and thirty seven patients have experienced a total of 53 respiratory exacerbations in which 32 have died.

Reports on a 59-year-old woman with long-standing seropositive rheumatoid arthritis presented to the Mayo Clinic in Rochester, Minnesota, with symptoms of dyspnea on exertion that had worsened gradually. Manifestations of the condition; Evidence of chronic symmetrical synovial inflammation in the metacarpophalangeal found in the patient's extremities.

OBJECTIVE: To assess the current spectrum of causes and clinical features associated with hypersensitivity pneumonitis (HP). PATIENTS AND METHODS: We studied consecutive patients with HP diagnosed at the Mayo Clinic In Rochester, Minn, from January 1, 1997, through December 31, 2002. Diagnostic criteria for HP included the following: (1) presence of respiratory symptoms, (2) radiologic evidence of diffuse lung disease, (3) known exposure or a positive serologic test result to an Inciting antigen, and (4) no other identifiable cause for the lung disease. If there was no identifiable Inciting antigen, 1 of the following 2 criteria was required: (1) lung biopsy specimen that demonstrated features of HP or (2) bronchoalveolar lavage lymphocytosis and high-resolution computed tomographic evidence of ground-glass opacities or centrilobular nodules bilaterally. RESULTS: The mean ± SD age of the 85 study patients was 53±14 years; 53 patients (62%) were women. Only 2 patients (2%) were current smokers. Chronic (⩾4 months) respiratory symptoms were present in 66 patients (78%). Histopathologlc confirmation was obtained in 64 patients (75%). The cause was identified in 64 patients (75%), and the most common causes were avian antigens (34%) and Mycobacterlum avium complex in hot tub water (21%). Farmer's lung disease accounted for 11% of cases, and an additional 9% were related to household mold exposure. The Inciting antigen was not identifiable in 25% of patients. CONCLUSION: Most patients with HP seen at this tertiary care referral center in the Midwest region of the United States had chronic HP, and the most common causes were exposure to birds and exposure to hot tubs. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To reassess the clinical and radiological features of chronic nitrofurantoin-induced lung disease and eventual clinical outcome. PATIENTS AND METHODS: We retrospectively reviewed the medical records of 18 patients with chronic nitrofurantoin-induced lung disease who were seen at the Mayo Clinic in Rochester, Minn. from January 1, 1997, to December 31, 2002. RESULTS: The median age of the 18 patients was 72 years (range, 47–90 years) at the time of diagnosis; 17 (94%) were women. Onset of symptoms occurred after a median interval of 23 months (range, 10–144 months) following the initiation of nitrofurantoin therapy for the prevention of recurrent urinary tract infections. All patients presented with persistent dyspnea and cough associated with lung infiltrates detected on chest radiography. Ten computed tomograms were available for review and revealed bilateral areas of ground-glass opacities in all cases and showed sub-pleural irregular linear opacities and patchy consolidation in some cases. Nitrofurantoin therapy was discontinued in all patients, and most improved subsequently; 9 patients received corticosteroid therapy. CONCLUSIONS: Chronic nitrofurantoin-induced lung disease is seen predominantly in older women who present with respiratory symptoms after a year or more of nitrofurantoin therapy. Associated radiological features are relatively nonspecific but usually include bilateral areas of ground-glass opacities on computed tomography of the chest. Cessation of nitrofurantoin therapy leads to improvement and suffices in the management of some patients, although corticosteroid therapy may be helpful in those more severely affected. [ABSTRACT FROM AUTHOR]

Objective: to describe presenting features of pulmonary infarction that may simulate those of lung cancer. Patients and methods: We reviewed the medical records of 43 patients with pulmonary infarction diagnosed by surgical lung biopsy at the Mayo Clinic in Rochester, Minn, from January 1, 1996, to December 31, 2002. Of 16 patients presenting with an undiagnosed solitary pulmonary nodule or mass, 6 had features suggestive of lung cancer on additional imaging, including abnormalities on contrast-enhancement computed tomography (CT), position emission tomography (PET), or nonsurgical lung biopsy before surgical resection. We examined the presenting symptoms, epidemiological, clinical, and radiological features, and clinical course of these 6 patients. Results: All 6 patients, ranging in age from 41 to 85 years, had a history of smoking and underlying cardiopulmonary disease. In 5 of the 6 patients, CT showed a nodule in the subpleural region of the lung. Three patients had abnormalities on contrast-enhancement CT, 2 had abnormalities on PET, and 1 had abnormal cytologic findings on a transthoracic needle biopsy of the lung; all these studies showed abnormalities suggestive of lung cancer. Surgical resection of the nodule or mass revealed pulmonary infarction associated with organizing thrombi in all 6 patients. Conclusions: Pulmonary infarctions can closely mimic the clinicoradiological characteristics of lung cancer, an association not reported previously. Furthermore, cytologic changes that occur in pulmonary infarctions may produce malignant-appearing cells on needle biopsy of the lung. The possibility of pulmonary infarction should be considered in the differential diagnosis of a solitary lung nodule or mass located in the subpleural region, even in the absence of clinically recognized venous thromboembolism. [ABSTRACT FROM AUTHOR]

Primary biliary cholangitis (PBC) is a progressive autoimmune disease of the liver causing destruction of intrahepatic bile ducts, associated with lymphocytic and granulomatous inflammation. PBC has been associated with many extrahepatic manifestations including interstitial lung disease. However, comprehensive pulmonary histopathology in PBC has not been well documented. Sixteen PBC patients who underwent lung biopsies were identified from surgical pathology files in three institutions. Histopathologic review was performed. Patient age ranged 41 to 79 years (median 55 years) and 15 patients (94%) were women. Specimens consisted of lobectomy (n = 1), surgical biopsies (n = 12), transthoracic needle biopsy (n = 1) and transbronchial biopsy (n = 2). Fifteen of 16 (94%) cases showed lymphocytic inflammation, mainly localized to peribronchiolar stroma and alveolar septa. Thirteen (81%) cases revealed non-necrotizing granulomas, most of which were poorly formed, reminiscent of those seen in liver biopsies from PBC patients. Six cases also showed eosinophilic infiltrates. Organizing pneumonia was seen in 7 cases. Four cases showed diffuse interstitial fibrosis with nonspecific interstitial pneumonia and usual interstitial pneumonia patterns. One patient underwent lobectomy for a mass lesion and was diagnosed with light chain deposition disease with underlying κ-restricted extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. In summary, PBC-associated histopathologic changes in the lung include lymphocytic inflammation predominantly around small airways and non-necrotizing granulomas in multiple compartments of lung tissue, which parallel PBC-associated histopathology in liver biopsies, often associated with other common patterns of diffuse lung disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: The gender, age, and lung physiology (GAP) model, interstitial lung diseases-GAP (ILD-GAP) model, and the smoking history, age, and diffusion capacity of the lung (SADL) model were compared using a systemic sclerosis-ILD (SSc-ILD) cohort to evaluate which best determined prognosis., Methods: The models were applied to a cohort of 179 patients with SSc seen at a tertiary care center within 1 year of ILD diagnosis. Demographics, clinical characteristics, and mortality were recorded. The performance of the models was assessed using standardized mortality ratios (SMR) of observed versus predicted outcomes for calibration and concordance (c)-statistics for discrimination., Results: SSc-ILD patients with usual interstitial pneumonia (31, 17%) had a higher mortality than those with non-specific interstitial pneumonia (147, 83%) (hazard ratio 2.27; 95%CI 1.03-4.97). All 3 models had comparable discrimination (c = 0.72, 0.72, and 0.71, respectively). Regarding calibration, the ILD-GAP model underestimated mortality (SMR 1.50; 95%CI 1.05-2.14). Calibration was acceptable for SADL (SMR 1.00; 95%CI 0.70-1.44) and GAP (SMR 0.90; 95%CI 0.63-1.29). The SADL model underestimated mortality in Stage I ILD., Conclusions: The ILD-GAP model underestimated mortality, and the SADL model underestimated mortality in certain subgroups. However, the GAP model performed well in this cohort, providing the best prognostic information for SSc-ILD.

Objective: To characterize the epidemiology of sarcoidosis from 1946 through 2013., Patients and Methods: An inception cohort of patients with incident sarcoidosis from January 1, 1976, through December 31, 2013, in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Inclusion required physician diagnosis supported by histopathologic confirmation, radiologic features of intrathoracic sarcoidosis, and a compatible clinical presentation. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality. The data were augmented with a previously identified cohort of Olmsted County residents diagnosed as having sarcoidosis in 1946-1975. Incidence rates were age and sex adjusted to the 2010 US white population., Results: A total of 448 incident cases of sarcoidosis were identified (mean age, 44.2 years; 52% women). The annual incidence of sarcoidosis was 10.0 per 100,000 population. The incidence of sarcoidosis increased in women from 1950 to 1960, but otherwise there were no significant calendar year trends. However, the peak age at incidence for women shifted from 40 to 59 years in 1950 to 50 to 69 years in 2010. Similarly, the peak age at incidence for men shifted from 30 to 49 years in 1950 to 40 to 59 years in 2010. Ninety-seven percent of patients had intrathoracic involvement, but only 43% had respiratory symptoms. The overall mortality of patients with sarcoidosis was not different from that of the general population (standardized mortality ratio=0.90; 95% CI, 0.74-1.08)., Conclusion: Sarcoidosis occurred in approximately 10 persons per 100,000 per year. Most of the patients had intrathoracic involvement, although less than half had respiratory symptoms. Overall mortality was not different from that of the general population., (Copyright © 2016 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Objective: Pulmonary disease represents an important extraarticular manifestation of rheumatoid arthritis (RA). While the association of RA and interstitial lung disease is widely acknowledged, obstructive lung disease (OLD) in RA is less well understood. We therefore aimed to assess the incidence, risk factors, and mortality of OLD in patients with RA., Methods: We examined a population-based incident cohort of patients with RA and a comparison cohort of individuals without RA. OLD was defined using a strict composite criterion. Cox proportional hazards models were used to compare OLD incidence between the RA and comparator cohorts to investigate risk factors and to explore the impact of OLD on patient survival., Results: A total of 594 patients with RA and 596 subjects without RA were followed for a mean of 16.3 and 19.4 years, respectively. The lifetime risk of developing OLD was 9.6% for RA patients and 6.2% for subjects without RA (hazard ratio [HR] 1.54, 95% confidence interval [95% CI] 1.01-2.34). The risk of developing OLD was higher among male patients, among current or former smokers, and for individuals with more severe RA. Survival of RA patients diagnosed with OLD was worse compared to those without OLD (HR 2.09, 95% CI 1.47-2.97)., Conclusion: Patients with RA are at higher risk of developing OLD, which is significantly associated with premature mortality. Effective diagnostic and therapeutic strategies to detect and manage OLD in patients with RA may help to improve survival in these patients., (Copyright © 2013 by the American College of Rheumatology.)

Background: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the influence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD., Methods: Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identified the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its influence on outcome., Results: During the 30-year interval, we identified 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death., Conclusions: The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome.

Background: Limited data exist regarding the population-based epidemiology of idiopathic pulmonary fibrosis (IPF). The objective of the study was to describe the trends in the incidence, prevalence, and clinical course of IPF in the community., Methods: We conducted a population-based study of adult patients with IPF in Olmsted County, Minnesota, from 1997 to 2005. Two methods were used to identify IPF cases, as defined by the 2002 American Thoracic Society/European Respiratory Society consensus statement: (1) usual interstitial pneumonia (UIP) on a surgical lung biopsy specimen or a definite UIP pattern on a high-resolution CT image (narrow criteria) and (2) UIP on a surgical lung biopsy specimen or a definite or possible UIP pattern on CT image (broad criteria)., Results: Of 596 patients screened for the possibility of pulmonary disease or pulmonary fibrosis over 9 years of follow-up, 47 cases had IPF. Of these, 24 met the narrow criteria. The age- and sex-adjusted incidence was 8.8/100,000 and 17.4/100,000 person-years, for narrow and broad criteria, respectively. The age-adjusted incidence was higher in men than in women, and among patients aged 70-79 years. During the study period, the incidence of IPF decreased (P < .001). On December 31, 2005, the age- and sex-adjusted prevalence was 27.9/100,000 and 63/100,000 persons by narrow and broad criteria, respectively. Thirty-seven patients experienced a total of 53 respiratory exacerbations (26 IPF related, 27 non-IPF related), and 34 (72%) patients died. The primary cause of death was IPF related in 16 (47%) patients. Median survival for narrow-criteria and broad-criteria incidence cases was 3.5 and 4.4 years, respectively., Conclusions: The incidence of IPF in Olmsted County decreased over the study period. Nonprimary IPF respiratory exacerbations are as frequent as primary IPF respiratory exacerbations and an important cause of death.

Background: While patients with interstitial lung disease (ILD) may be particularly susceptible to ventilator-induced lung injury, ventilator strategies have not been studied in this group of patients., Purposes: To describe the clinical course and outcome of patients with ILD and acute respiratory failure in relation to ventilatory parameters., Methods: We retrospectively identified a cohort of ventilated patients with ILD who had been admitted to five ICUs at a single institution. We analyzed demographic data, pulmonary function test results, severity of illness, and the parameters of continuous ventilation for the initial 24 h after admission to the ICU. Primary outcomes were survival to hospital discharge and 1-year survival., Main Results: Of 94 patients with ILD, 44 (47%) survived to hospital discharge and 39 (41%) were alive at 1 year. Nonsurvivors were less likely to be postoperative, had higher severity of illness, and were ventilated at higher airway pressures and lower tidal volumes. Step changes in positive end-expiratory pressure (PEEP) of > 10 cm H(2)O were attempted in 20 patients and resulted in an increase in plateau pressure (median difference, + 16 cm H(2)O; interquartile range [IQR], 9 to 24 cm H(2)O) and a decrease in respiratory system compliance (median difference, - 0.28 mL/kg/cm H(2)O; IQR, - 0.43 to - 0.13 mL/kg/cm H(2)O). The Cox proportional hazards model revealed that high PEEP (hazard ratio, 4.72; 95% confidence interval [CI], 2.06 to 11.15), acute physiology and chronic health evaluation (APACHE) III score predicted mortality (hazard ratio 1.33; 95% CI, 1.18 to 1.50), age (hazard ratio, 1.03; 95% CI, 1 to 1.05), and low Pao(2)/fraction of inspired oxygen ratio (hazard ratio, 0.96; 95% CI, 0.92 to 0.99) to be independent determinants of survival., Conclusion: Both severity of illness and high PEEP settings are associated with the decreased survival of patients with ILD who are receiving mechanical ventilation.

Objective: Rheumatoid arthritis (RA) is associated with a variety of pleuropulmonary manifestations, some of which require surgical intervention. We investigated the spectrum of indications and results, as well as outcome associated with thoracic surgical procedures in rheumatoid patients., Methods: Identification and retrospective review of medical records of 100 patients with RA at a tertiary-referral medical center undergoing thoracic surgical procedures over a 24-year period from January 1, 1976, to December 31, 1999., Results: Sixty-four patients underwent surgical lung biopsy for localized lung lesions, 57 of which were nodules or masses; 24 lesions proved to be malignant. Surgical lung biopsy was undertaken in 40 patients for diffuse interstitial lung disease, 4 cases of which were found to be infectious. Five other patients underwent surgery for pleural disease, 3 of which proved to be benign pleuritis., Conclusion: Patients with rheumatoid disease undergo thoracic surgical procedures for a variety of indications, including benign and malignant localized lesions and pleural disease as well as diffuse parenchymal lung disease. The overall in-hospital mortality rate was low. However, the subgroup with diffuse interstitial lung disease was found to have a 20% in-hospital death rate following surgical lung biopsy.