Granulomatosis With Polyangiitis With And Without Antineutrophil Cytoplasmic Antibodies: A Case-Control Study.

Background: ANCA-negative GPA remains a diagnosis of exclusion. Clinical differences between patients with ANCA-negative vs ANCA-positive GPA have not been analyzed in sizable case-control studies, and the effects of ANCA-seroconversion from negative to positive are not well documented.

Methods: A single-center, sex, and age matched case-control study evaluated ANCA-negative vs ANCA-positive GPA from January 1, 1996, to December 31, 2015.

Unsupervised Exercise in Interstitial Lung Disease: A Delphi Study to Develop a Consensus Preparticipation Screening Tool for Lymphangioleiomyomatosis.

Background: Little research is available to provide practical guidance to health care providers for exercise preparticipation screening and referral of patients with interstitial lung diseases (ILDs), including lymphangioleiomyomatosis (LAM), to participate in remote, unsupervised exercise programs.

Research Question: What exercise preparticipation screening steps are essential to determine whether a patient with LAM is medically appropriate to participate in a remote, unsupervised exercise program?

Study Design And Methods: Sixteen experts in LAM and ILD participated in a two-round modified Delphi study, ranking their level of agreement for 10 statements related to unsupervised exercise training in LAM, with an a priori definition of consensus. Additionally, 60 patients with LAM completed a survey of the perceived risks and benefits of remote exercise training in LAM.

Vaping-associated constrictive bronchiolitis.

A woman in her 40s who vaped reported rapid onset of shortness of breath and cough. Pulmonary function testing showed severe obstruction and no substantial response to an inhaled bronchodilator, and chest computed tomography revealed extensive air trapping. A lung cryobiopsy was done after a comprehensive and unrevealing evaluation, which showed characteristics of airway inflammation and prominent bronchial-associated lymphoid tissue.

Pulmonary hypertension in ANCA-associated vasculitis: a retrospective analysis.

Background: Little is known about pulmonary hypertension (PH) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Objectives: The aims of this retrospective study, in which echocardiography was used for detection of PH, were to identify the potential causes of PH in AAV and to analyze the risk factors for mortality.

Methods: We performed a retrospective descriptive review of 97 patients who had AAV with PH at our institution from January 1, 1997, through December 31, 2015.

Risk Stratification in Transthyretin Cardiac Amyloidosis: The Added Value of Lung Spirometry.

Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized disease that often results in heart failure and death. Traditionally, biological staging systems are used to stratify disease severity. Reduced aerobic capacity has recently been described as useful in identifying higher risk of cardiovascular events and death.

A 79-Year-Old Man With Recurrent Respiratory and Constitutional Symptoms, Elevated Acute Phase Reactants, and Pancytopenia.

A 79-year-old man was examined because of recurrent dyspnea and constitutional symptoms that included malaise, fatigue, fevers, and arthralgias over the past 7 years. He was a nonsmoker who was a retired farmer. Elevated levels of acute phase reactants and C-reactive protein and a high erythrocyte sedimentation rate were noted often in his health records.

Pulmonary manifestations in VEXAS syndrome.

Background: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently recognized multisystem disorder caused by somatic mutations in the UBA1 gene.

Methods: A retrospective cohort study was conducted on all patients with VEXAS syndrome evaluated at our institution from June 2020 through May 2022. Medical records and chest imaging studies were reviewed.

Clinical Outcomes With and Without Plasma Exchange in the Treatment of Rapidly Progressive Interstitial Lung Disease Associated With Idiopathic Inflammatory Myopathy.

Background/objective: A subset of patients with idiopathic inflammatory myopathy (IIM) develops highly fatal, rapidly progressive interstitial lung disease (RP-ILD). Treatment strategies consist of glucocorticoid and adjunctive immunosuppressive therapies. Plasma exchange (PE) is an alternative therapy, but its benefit is unclear.

Treatment Outcomes for Rheumatoid Arthritis-Associated Interstitial Lung Disease: A Real-World, Multisite Study of the Impact of Immunosuppression on Pulmonary Function Trajectory.

Background: Rheumatoid arthritis (RA)-associated interstitial lung disease (ILD) is common in patients with RA and leads to significant morbidity and mortality. No randomized, placebo-controlled data are available that support the role of immunosuppression to treat RA-associated ILD, despite being widely used in clinical practice.

Research Question: How does immunosuppression impact pulmonary function trajectory in a multisite retrospective cohort of patients with RA-associated ILD?

Study Design And Methods: Patients with RA who started treatment for ILD with mycophenolate, azathioprine, or rituximab were identified retrospectively from five ILD centers.

Idiopathic Chronic Eosinophilic Pneumonia Evolving to Pulmonary Fibrosis: A Retrospective Analysis.

Background: Patients with idiopathic chronic eosinophilic pneumonia (ICEP) may have pulmonary fibrosis.

Objectives: To investigate the predictors of pulmonary fibrosis in ICEP, to describe the timeline of pulmonary fibrosis after ICEP diagnosis, and to detail the radiologic pattern of fibrosis.

Methods: A retrospective computer-assisted search was performed to identify patients with ICEP seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1997, through September 1, 2019.

Excipient lung disease secondary to intravenous heroin use.

We present a case of a woman who had progressive shortness of breath and wheezing with a mild restrictive pulmonary function pattern. She was initially diagnosed with eosinophilic granulomatosis with polyangiitis on the basis of peripheral eosinophilia, bronchoalveolar lavage eosinophilia (47%) and surgical lung biopsy findings. Six months following her diagnosis, the patient returned because of persistent symptoms, and a second review of the lung biopsy revealed thrombotic lesions in the pulmonary vessels with polarisable foreign body materials, associated giant cell reactions and numerous eosinophil infiltrates, consistent with intravenous drug abuse.

Challenges in the Diagnosis and Management of Fibrotic Hypersensitivity Pneumonitis: A Practical Review of Current Approaches.

Recent advances in fibrotic hypersensitivity pneumonitis include improved diagnostic guidance, systematic assessments of immunosuppressive therapy, and the recent availability of antifibrotic therapy (nintedanib) for those with progressive disease. A standardized approach to diagnosis may lead to better inclusion criteria for future therapeutic protocols and delineation of disease or treatment response predictors for real-world management. This review will highlight current diagnostic and treatment challenges and remaining knowledge gaps or areas of uncertainty, with a practical overview of supporting evidence and its clinical implications.

Antigen identification and avoidance on outcomes in fibrotic hypersensitivity pneumonitis.

Background: Suspected causative antigens may be unidentified in 30-50% of patients with fibrotic hypersensitivity pneumonitis (f-HP). It is unclear whether antigen identification and avoidance in this setting offer any additional clinical benefit. We hypothesised that antigen identification and avoidance may improve the clinical course of patients with fibrotic disease.

Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.

Introduction: Amyloid transthyretin amyloidosis (ATTR) is characterized by deposition of a misfolded conformation of the transport protein TTR, most commonly in cardiac and nerve tissue, causing clinical disease. Pulmonary amyloidosis, or deposition of ATTR in lung tissue, is a poorly characterized manifestation of this disease. We present the clinical course, imaging characteristics, pathology results, and outcomes of a patient cohort diagnosed with pulmonary ATTR.

Association of COVID-19 antigenicity with the development of antineutrophilic cytoplasmic antibody vasculitis.

Inflammatory processes, such as an infection or drug reaction, can cause antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Although quite rare, AAV may occur with SARS-coronavirus disease 2019 (COVID-19) antigenic exposure, either from infection or immunization. We present two cases of AAV: one that developed after COVID-19 infection presenting as diffuse alveolar haemorrhage and another that developed shortly after vaccination, presenting as granulomatous pulmonary nodules.

Airway Amyloidosis: A Retrospective Analysis of 43 Patients.

Background: Amyloidosis can involve any compartment in the thorax. We aimed to explore the clinical and radiologic presentation, treatment, and clinical course of airway amyloidosis.

Methods: A computer-assisted search was performed to identify patients who had biopsy-proven airway amyloidosis and were evaluated at Mayo Clinic in Rochester, MN, from January 1, 1997 through December 31, 2019.

Improvement of constrictive bronchiolitis (bronchiolitis obliterans) after rituximab therapy in 2 patients with primary sjögren syndrome.

Constrictive bronchiolitis is one of the manifestations of small-airway involvement in primary Sjögren syndrome (SS) and is associated with fixed airflow obstruction despite treatment with bronchodilators, macrolides, corticosteroids, and corticosteroid-sparing agents. Reports have shown a beneficial effect of rituximab on interstitial lung disease associated with SS, but the effect of rituximab on constrictive bronchiolitis is unknown. Herein, we present 2 cases of patients with constrictive bronchiolitis associated with SS who experienced symptomatic improvement and stabilization of pulmonary function testing (PFT) after rituximab therapy.

Pleuritis and Pericarditis in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis.

Background: Pleural and pericardial involvements are well recognized in eosinophilic granulomatosis with polyangiitis (EGPA) but considered rare manifestations of the other forms of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV).

Research Question: What are the frequency and clinical characteristics of pleuritis and pericarditis in AAV?

Study Design: and Methods: Using an institutional database of 1,830 patients with AAV, we analyzed clinical notes and diagnosis codes for key words related to pleuritis and pericarditis. Chart review to confirm these findings was performed.

Pulmonary Low Attenuation Areas on CT in ANCA-associated Vasculitis: A quantitative and semi-quantitative analysis correlated with pulmonary function testing for obstructive airway disease.

Objective: A subset of ANCA-associated vasculitis (AAV) patients are known to manifest obstructive airway disease. Using low attenuation areas (LAA) in the lung on HRCT as an imaging marker for obstructive airway disease, we analyze HRCT studies in AAV patients compared to a matched non-AAV group using visual semi-quantitative and automated quantitative analysis for presence and severity of LAA. Furthermore, HRCT and pulmonary function testing are compared to assess agreement between tests for airway obstruction.

Host Factors and Outcomes in Hospitalizations for Pneumocystis Jirovecii Pneumonia in the United States.

Objective: To assess host factors in pneumocystis jirovecii pneumonia (PCP)-related hospitalizations and compare outcomes between HIV and non-HIV patients.

Methods: Using the National Inpatient Sample database, we identified 3384 hospitalizations with PCP (International Classification of Diseases, Ninth Revision, Clinical Modification code: 136.3) as the primary discharge diagnosis from 2005 to 2014.

Idiopathic pulmonary fibrosis and gastroesophageal reflux disease: A population-based, case-control study.

Background: It is unknown whether gastroesophageal reflux disease (GERD) is a risk factor or consequence of idiopathic pulmonary fibrosis (IPF). This study aimed to determine whether patients with IPF were more likely to have GERD compared with age- and sex-matched controls who either had 1) interstitial lung disease (ILD) other than IPF or 2) no diagnosed lung disease (population control).

Methods: We used the medical records-linkage system of the Rochester Epidemiology Project (REP) to identify patients with IPF who resided in Olmsted County, Minnesota, from January 1, 1997, through June 30, 2017.

A 48-Year-Old Woman With Chronic Cough, Dyspnea, and Bronchiectasis.

A 48-year-old woman sought a second opinion for dyspnea and chronic productive cough; she was a never smoker. Mild respiratory symptoms persisted since childhood and had progressively worsened over the previous decade. In addition, an unintentional 30-pound weight loss had occurred over several years.

Pulmonary nodules in patients with primary Sjögren's syndrome: Causes, clinico-radiologic features, and outcomes.

Background: Primary Sjögren's Syndrome (pSS) is characterized by an immune-mediated lymphoplasmacytic infiltration of the salivary and lacrimal glands. Pulmonary nodules are not uncommonly encountered in these patients.

Methods: We conducted a retrospective computer-assisted search for patients with pSS who were encountered at our institution between 1999 and 2018 and had histologically characterized pulmonary nodule(s)/mass (es) (PNs).