Presentation of IPF

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Presentation of IPF Key clinical features of IPF Symptoms at presentation Patients with idiopathic pulmonary fibrosis (IPF) may present with a number of different symptoms.1-3 Patients who are older than 50 years of age General risk - Incidence increases with older age, factors of typically presenting in the 60s and 70s 1 Men are at greater risk than women IPF The majority of patients have a history of cigarette smoking Listening to the lungs Lung auscultation is currently the best way to recognise IPF early.4 A key clinical feature for recognising IPF is the presence of bilateral Velcro® -like crackles that can be heard when listening to the basal section of the patient's lungs. Crackles are discontinuous, short, explosive sounds that, in IPF, resemble the sound of a strip of Velcro® slowly separating.4 Identification of crackles should prompt a pulmonary function test. For further details on what to listen for, including audio examples, see Listening to IPF Pulmonary function test IPF is a restrictive lung disease limiting lung capacity and impairing lung expansion.5 All patients with suspected interstitial lung disease (ILD) should undergo spirometry and diffusing capacity pulmonary function tests to provide an assessment of the restrictive impairment.5 The observed restrictive impairment typically manifests as reduced measures of lung capacity and volume, such as forced vital capacity, total lung capacity and residual volume.5 These measures may appear normal in patients with IPF and a concomitant obstructive disease, such as chronic bronchitis or emphysema.5 It is also possible for a patient with IPF to have normal pulmonary function at rest; however, the diffusing capacity of the lungs for carbon monoxide (DLco) at presentation is a more reliable guide of disease status than other resting lung function variables; research has shown that a DLco <40% is suggestive of advanced fibrotic disease.5 References 1. Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788–824. 2. NICE Clinical Guidelines 163: Idiopathic Pulmonary Fibrosis. June 2013. 3. NICE Clinical Pathway: Idiopathic Pulmonary Fibrosis overview. April 2015. 4. Cottin V, et al. Eur Respir J. 2012;40:519–521. 5. Wells AU, et al. Thorax. 2008;63:v1–v58..

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Restrictive Lung Diseases & Pulmonary Vascular Disease Pulmonary 2018 RESTRICTIVE LUNG DISEASES AND PULMONARY VASCULAR DISEASE Joel Thibodeaux, MD, Phone: 469-419-4535 Email: [email protected] INTRODUCTION This lecture will discuss basic concepts, etiologic factors, pathologic features, pathogenesis, and clinicopathologic findings in the different types of interstitial lung diseases. It also will touch briefly on pulmonary vascular diseases. LEARNING OBJECTIVES: • Acute respiratory distress syndrome (ARDS) (BP, pp. 460-461) o Define ARDS and list common causes o Illustrate the mechanism of lung injury in ARDS and the role of proinflammatory and anti-inflammatory mediators o Describe the morphologic features of ARDS. Understand how they evolve, and know what happens if the patient survives. • Diffuse interstitial lung disease (BP, pp. 472-474, 480-482) o List the major forms of diffuse interstitial lung diseases discussed o Be able to identify classic morphologic features of common interstitial lung diseases. o Recognize the major causes of pneumoconioses (BP, pp. 474-478) o Delineate the gross and microscopic features resulting from exposure to coal dust, silica, organic/animal dust, and asbestos. o Sarcoidosis (BP, pp. 478-480) . Define sarcoidosis . List the most common organs involved, and describe the characteristic histologic lesion . Describe the radiographic, gross, and histologic appearance of lesions in the hilar lymph nodes and lungs • Define primary pulmonary hypertension. Recognize the characteristic histologic
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Idiopathic Bronchiolocentric Interstitial Pneumonia Samuel A. Yousem, MD, Sanja Dacic, MD, PhD Department of Pathology, University of Pittsburgh Medical Center, Presbyterian University Hospital, Pittsburgh, Pennsylvania plugs of fibromyxoid connective tissue, and poorly The authors report 10 patients with a distinctive formed granulomas (1–3). This triad of morphologic idiopathic bronchiolocentric interstitial pneumonia changes allows the pathologist to direct the pulmo- having some histologic similarities to hypersensitiv- nologist to question the patient for specific inhala- ity pneumonitis. Bronchiolocentric interstitial tional exposures. We have accumulated 10 cases pneumonia has a marked predilection for women that have very similar morphologic findings to hy- (80%) and occurs in middle age (40–50 years). Chest persensitivity pneumonitis, with the exclusion of radiographs and pulmonary function tests show in- interstitial granulomas, in which extensive investi- terstitial and restrictive lung disease, while the his- gations failed to reveal a cause for the inflamma- tologic appearance is that of a centrilobular inflam- tion. The clinicopathologic features of this idio- matory process with small airway fibrosis and pathic bronchiolocentric interstitial pneumonia are inflammation that radiates into the interstitium of the basis of this report which suggests a more ag- the distal acinus in a patchy fashion. Granulomas gressive and life threatening biologic behavior for are not identified. At a mean followup of approxi- bronchiolocentric interstitial pneumonia than nor- mately 4 years in nine patients, 33% of patients mally associated with hypersensitivity pneumonitis were dead of disease and 56% had persistent or or some of the other conditions in the histologic progressive disease suggesting a more aggressive differential diagnosis. course than hypersensitivity pneumonitis and non- specific interstitial pneumonia, the two major dis- ease processes in the differential diagnosis.
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Restrictive Lung Disease
Downloaded from https://academic.oup.com/ptj/article/48/5/455/4638136 by guest on 29 September 2021 RESTRICTIVE LUNG DISEASE WARREN M. GOLD, M.D. RESTRICTIVE LUNG DISEASE is a These disorders can be divided into two pattern of abnormal lung function defined by groups: extrapulmonary and pulmonary. a decrease in lung volume (Fig. I).1,2 The In extrapulmonary restriction, an abnormal total lung capacity is decreased and, in severe increase in the stiffness of the chest wall (kypho restrictive defects, all of the subdivisions of the scoliosis) restricts the lung volumes, as does total lung capacity including vital capacity, respiratory muscle weakness (poliomyelitis or functional residual capacity, and residual vol muscular dystrophy). These extrapulmonary ume are decreased. In mild or moderately se causes of pulmonary restriction are treated vere restrictive defects, the residual volume may be normal or slightly increased. CLINICAL DISORDERS CAUSING TABLE 1 RESTRICTIVE LUNG DISEASE CAUSES OF RESTRICTIVE LUNG DISEASE Restrictive lung disease is not a specific clin I. Extrapulmonary restriction 3 ical entity, but only one of several patterns of A. Chest wall stiffness (kyphoscoliosis) B. Respiratory-muscle weakness (muscular dystrophy)4 abnormal lung function. It is produced by a C. Pleural disease (pneumothorax)5 number of clinical disorders (see Table 1). II. Pulmonary restriction A. Surgical resection (pneumonectomy)6.7 Dr. Gold: Director, Pulmonary Laboratory and Re B. Tumor (bronchogenic carcinoma or metastatic tumor)8 search Associate in Cardiology (Pulmonary Physiology), C. Heart disease (hypertensive, arteriosclerotic, rheu Children's Hospital Medical Center; Associate in Pedi matic, congenital)9 atrics and Tutor in Medical Science, Harvard Medical 10 11 School, Boston, Massachusetts.
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Restrictive Lung Disease in Patients with Subclinical Coronavirus Infection: Are We Bracing Ourselves for Devastating Sequelae?
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