Restrictive Lung Diseases Pathology Study Guide

Restrictive Lung Disease Pathology Video

Restrictive Lung Diseases

Restrictive lung diseases are characterized by restricted filling of the lung where the FVC is more decreased than FEV₁ resulting in a FEV₁/FVC ratio of above 70%.

Restrictive lung diseases are most commonly caused by interstitial diseases of the lung and chest wall abnormalities like obesity.

Different Examples of Cryobiopsy Showing UIP pattern.A) A low-magnification image showing dense scarring obliterating the alveolar architecture and abruptly alternating with relatively normal lung (patchy fibrosis). Some fibroblastic foci are visible even at this magnification for their pale-gray color. B) Fibroblastic focus better visualized at higher magnification. C) An area of honeycombing. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases. Casoni GL, Tomassetti S, Cavazza A, Colby TV, Dubini A, Ryu JH, Carretta E, Tantalocco P, Piciucchi S, Ravaglia C, Gurioli C, Romagnoli M, Gurioli C, Chilosi M, Poletti V - PloS one (2014). Not Altered. CC.
(A) Smoking-related interstitial fibrosis involving subpleural lung parenchyma (pleural surface is at the left) with centrilobular accentuation (arrow); (B) thickening of the alveolar septa by dense, eosinophilic, paucicellular collagen bundles. Pulmonary adenocarcinoma mutation profile in smokers with smoking-related interstitial fibrosis. Primiani A, Dias-Santagata D, Iafrate AJ, Kradin RL - International journal of chronic obstructive pulmonary disease (2014). Not Altered. CC.
Restrictive Lung Diseases Pathology. Low magnification micrograph of emphysema. H&E stain. The left of image shows severe emphysema (large empty spaces). The lung tissue on the right of the image has relative preservation of the alveoli. The top of the image is very near the pleural surface. Nephron. Not altered. CC BY-SA 3.0
The surgical specimen of a mass-like opacity in the left lower lobe. Small, loose-formed, non-necrotizing granulomas are seen in the bronchiole and alveolar duct (left) and in the lymph tract just below the pleura (right); no granulomatous vasculitis is seen. The findings are consistent with hypersensitivity pneumonitis with poor drainage of a large amount of granulomas (Elastica van Gieson stain, left ×200, right ×100). Chronic granulomatous disease with pulmonary mass-like opacities secondary to hypersensitivity pneumonitis: a case report. Katsuya Y, Hojo M, Kawai S, Kawai T, Onodera M, Sugiyama H - Journal of medical case reports (2014). Not Altered. CC.
Panels a and b are different plates from the lung biopsy showing pulmonary alveolar proteinosis pattern characterized by type II pneumocytes hyperplasia, interstitial widening, and fine granular proteinosis material admixed with foamy macrophages. Fatal respiratory disease due to a homozygous intronic ABCA3 mutation: a case report. Journal of Medical Case Reports. Not Altered. CC.

Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is characterized by the scarring or fibrosis of lung interstitium.

Although the etiology is unknown, idiopathic pulmonary fibrosis (IPF) is likely related to cyclical lung injury as injured pneumocytes induce fibrosis.

Causes of idiopathic pulmonary fibrosis (IPF) include:

Cyclical lung injury
Bleomycin
Amiodarone
Radiation therapy

Clinical features of idiopathic pulmonary fibrosis (IPF) include:

Cough
Progressive dyspnea and cough

Fibrosis on lung CT shows subpleural patches, which progress to diffuse fibrosis and eventually forms a ‘honeycomb’ pattern which is an indicator of end-stage pulmonary fibrosis.

The recommended treatment of idiopathic pulmonary fibrosis involves lung transplantation.

Idiopathic Pulmonary Fibrosis. Not altered. CC
Histopathological and molecular correlates of idiopathic pulmonary fibrosis. a–c (hematoxylin and eosin) show increased magnifications of idiopathic pulmonary fibrosis in which areas of histologically normal lung is admixed with areas of active fibrosis (a, × 25), marked by serpentine glands lined with epithelia (b, × 50), which show nuclear atypia and multinucleation (c, arrow, × 400). Herpesvirus saimiri DNA was commonly detected in the nuclei of the epithelia of these serpentine glands (d, × 50) and much less evident in the admixed histologically normal lung (e, × 400; blue NBT/BCIP signal with pink counterstain). In comparison, herpesvirus saimiri DNA was not evident in cases of interstitial pneumonitis and fibrosis of known viral etiology (f, × 400 measles infection). Herpesvirus saimiri DNA was evident in the regenerating epithelia in idiopathic pulmonary fibrosis in the same cells when serial sections were probed for herpesvirus saimiri terminal repeat sequence (g, × 400) or the STP ORF 1 gene (h, × 400). Idiopathic pulmonary fibrosis is strongly associated with productive infection by herpesvirus saimiri. Folcik VA, Garofalo M, Coleman J, Donegan JJ, Rabbani E, Suster S, Nuovo A, Magro CM, Di Leva G, Nuovo GJ - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc (2013). Not Altered. CC.
Idiopathic Pulmonary Fibrosis. Figure A shows the location of the lungs and airways in the body. The inset image shows a detailed view of the lung’s airways and air sacs in cross-section. Figure B shows idiopathic pulmonary fibrosis (scarring) in the lungs. The inset image shows a detailed view of the fibrosis and how it damages the airways and air sacs. National Heart Lung and Blood Institute (NIH) - National Heart Lung and Blood Institute (NIH). Not altered. Public Domain
Idiopathic Pulmonary Fibrosis. Clubbing of the fingers in idiopathic pulmonary fibrosis - Not altered. CC BY-SA 3.0
Idiopathic Pulmonary Fibrosis. Photomicrograph of the histopathological appearances of usual interstitial pneumonia. High-power magnification (on the right) shows a focus of fibroblastic proliferation, close to an area of fibrosis within which a mild, non-specific, chronic inflammatory cell infiltrate can be observed. In the subpleural space, a typical honeycombing aspect can be recognized. IPFeditor. Not altered. CC BY-SA 3.0
Idiopathic Pulmonary Fibrosis. A chest radiograph of a patient with IPF. Note the small lung fields and peripheral pattern of reticulonodular opacification. IPFeditor. Not altered. CC BY-SA 3.0

Pneumoconiosis

Pneumoconiosis is the form of interstitial fibrosis caused by occupational exposure to fibrogenic particles like asbestos, beryllium, and silica dusts.

The alveolar macrophages in the lungs engulf these particles inducing fibrosis.

Pnemoconioses. High magnification micrograph of asbestosis of the lung, with characteristic ferruginous bodies and interstitial fibrosis. Lung biopsy. H&E stain. See also Image:Ferruginous_body.jpg - close-up of ferruginous bodies. Nephron. Not altered. CC BY-SA 3.0

Sarcoidosis

Sarcoidosis is a systemic disease characterized by growing granulomas in various body organs and is more prevalent in African American women.

Although its etiology is unknown sarcoidosis likely rises from the response of CD4+ helper T-cells to an unknown antigen.

The granulomas mostly affect the hilar lymph nodes and lungs, causing restrictive lung disease.

Histology of sarcoidosis includes naked granulomas and stellate inclusions (‘asteroid bodies’) are often seen within giant cells of the granulomas.

Other commonly affected tissues in sarcoidosis include:

Uvea (uveitis)
Skin (cutaneous nodules or erythema nodosum)
Salivary glands
Lacrimal glands

Clinical features of sarcoidosis include:

Dyspnea
Cough

Laboratory findings of sarcoidosis include:

Elevated serum ACE
Hypercalcemia

Steroids are the most preferred treatment although the condition often resolves on its own.

Sarcoidosis
Sarcoidosis. Naked granuloma. Not altered. CC.
Sarcoidosis. Pulmonary sarcoidosis with granulomas with Langhans giant cells and asteroid bodies Nephron. Not altered. CC BY-SA 3.0
Asteroid Body in Sarcoidosis. Ed Uthman. Not altered. CC BY 2.0
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Immunohistochemistry for TL1A and DR3 expression in representative patients with active (A, B) and refractory sarcoidosis and pulmonary fibrosis (C, D). A, B. TL1A and DR3 are expressed at high intensity by macrophagic, both epitheliod and multinucleated, cells and lymphocytes infiltrating the lung biopsy of the patient with active sarcoidosis. The black arrows indicate positive metaplastic epithelial cells. C, D. In the lung specimen obtained from a patient with refractory sarcoidosis and pulmonary fibrosis, pulmonary cells were mainly non reactive for TL1A and DR3; a weak immunostaining was only seen in some epitheliod cells (arrows). Note marked fibrosis around the granulomatous nodule. Original magnification 400x. TL1A/DR3 axis involvement in the inflammatory cytokine network during pulmonary sarcoidosis. Facco M, Cabrelle A, Calabrese F, Teramo A, Cinetto F, Carraro S, Martini V, Calzetti F, Tamassia N, Cassatella MA, Semenzato G, Agostini C - Clinical and molecular allergy : CMA (2015). Not Altered. CC.

Hypersensitivity Pneumonitis

Hypersensitivity pneumonitis is mainly characterized by granulomatous reaction to inhaled organic antigens like pigeon breeder’s lung.

Common signs and symptoms of hypersensitivity pneumonitis include:

Fever
Cough
Dyspnea

Symptoms of hypersensitivity pneumonitis typically occur hours after exposure.

Symptoms of hypersensitivity pneumonitis usually resolve after removal of the antigen.

Chronic exposure to the predisposing factor causing the hypersensitivity pneumonitis may result in interstitial fibrosis.

Hypersensitivity Pneumonitis. High magnification micrograph of hypersensitivity pneumonitis showing granulomatous inflammation. Trichrome stain. Nephron - Not altered. CC BY-SA 3.0
Hypersensitivity Pneumonitis. Low magnification view of the histology of chronic hypersensitivity pneumonitis. The interstitium is expanded by a chronic inflammatory infiltrate. Two multinucleated giant cells can be seen within the interstitium at left, and a plug of organizing pneumonia at bottom left. Mutleysmith - Not altered. CC BY-SA 3.0
Hypersensitivity Pneumonitis. This is a high magnification photomicrograph from a lung biopsy taken from a patient with chronic hypersensitivity pneumonitis (hematoxylin-eosin stain). It shows mild expansion of the alveolar septa (interstitium) by lymphocytes. A multinucleated giant cell, seen within the interstitium to the right of the picture halfway down, is an important clue to the correct diagnosis. Mutleysmith . Not altered. CC BY-SA 3.0
Lung staining for SA-β-galactosidase activity.(A and B) 5 μM sections of lung from of patients with IPF, (C) normal donor controls, (D) scleroderma, or (E) hypersensitivity pneumonitis were stained for SA-βgal activity. Note the blue staining epithelial cells in IPF lung (A and B, arrows) but not normal individuals, or patients with scleroderma or hypersensitivity pneumonitis. Images are representative of staining of tissue from 8 patients with IPF, 14 control subjects, 5 patients with scleroderma, and 8 patients with hypersensitivity pneumonitis. miR-34 miRNAs Regulate Cellular Senescence in Type II Alveolar Epithelial Cells of Patients with Idiopathic Pulmonary Fibrosis. Disayabutr S, Kim EK, Cha SI, Green G, Naikawadi RP, Jones KD, Golden JA, Schroeder A, Matthay MA, Kukreja J, Erle DJ, Collard HR, Wolters PJ - PloS one (2016). Not Altered. CC.