Interstitial lung disease Flashcards by Meghan May

Three synonyms for interstitial lung disease

Infiltrative lung disease -Infiltration of cellular and non-cellular elements within alveolar septa and alveoli.
Diffuse parenchymal lung disease (DPLD)
Restrictive lung disease – Characterized by reduced total lung capacity in presence of a normal or reduced expiratory flow rate.

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Infiltration of cellular and non-cellular
elements within alveolar septa and alveoli.

Infiltrative lung disease

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Characterized by reduced total lung
capacity in presence of a normal or reduced expiratory flow rate

Restrictive lung disease

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– Fibrosing lung disorders (pneumoconioses)
– Granulomatous disorders (sarcoidosis)
– Idiopathic interstitial pneumonias (IIPs)

these are all examples of

Chronic interstitial lung disease

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“_______” – Damage to pneumocytes and
endothelial cells

Alveolitis

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Pathogenesis of ILDS: Leads to leukocytes releasing cytokines which mediate and stimulate

interstitial fibrosis

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Interstitial fibrosis –
–↓es lung _____ and _____
• ↓ed lung expansion during inspiration

compliance and elasticity

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What are our modulating factors for patholgenesis of Interstitial lung disorders?

chronicity of lung exposure, effectiveness of lung defenses, extent of injury, intactness of basement membrane, individual susceptibility

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What are clinical findings of interstitila lung disease?

what do you see on xray?

• Dry cough and dyspnea
• Late inspiratory crackles, bibasilar (Velcro
crackles)
• Cor pulmonale

-bilateral reticulonodular infiltrates

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Non-neoplastic lung diseases in response
to inhalation of mineral dusts inhaled in the
workplace.

Pneumoconioses

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include diseases induced by organic and inorganic particulate matter/chemical fumes /vapors.
• Coal dust, silica, asbestos, beryllium
• 25% cases of chronic interstitial lung disease

Pneumoconioses

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Devo of pneumoconiosis depends on:

amount of dust retained in parenchyma and airways

size/shape/buoyace of particle

solubility of particle and physiochemical reactivity

possible additional effects of other irritants

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1 – 5 μm particles will – reach bifurcation of
– < 0.5 μm – reach alveoli and are

respiratory bronchioles and alveolar ducts

phagocytosed by alveolar macrophages

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Coals workers pneumocosis, silicosis, silicatosis, asbestosis are alls forms of

fibrogenic pnemoconiosis

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Anthracotic pigment – coal mines, urban centers, tobacco smoke
Pulmonary anthracosis
Simple
Complicated

all examples of:

Coal workers pneumoconiosis = CWP

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How does anthracosis present?

asmptomatic and anthracotic pigment in interstitial compartment and lymph nodes

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Simple coal workers’ pneumoconiosis
• Fibrous opacities _____
•are seen in which lobes?
• Characterized by coal dust deposits adjacent
to _______

< 1 cm

Upper lobes and upper portions of lower lobes

respiratory bronchioles

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Complicated coal workers’ pneumoconiosis
(progressive massive fibrosis)
• Fibrous opacities ____
• _________ central necrosis
• Massive fibrosis –

> 1 cm

With or without

crippling lung disease (Black
lung disease)

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What other issues do we see with complicated coal workers pneumoconiosis?

cor pulmonale

see w/ Caplan syndrome: CWP with rheumatoid nodules in lung

NO increased incidence of TB or Cancer

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• Most common occupational disease worldwide
• Crystalline silicon dioxide (quartz)
– Foundries (metal casting), sandblasting, silica mines

Silicosis

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Pathogenesis of silicosis

• Quartz activates alveolar macrophages after engulfment → cytokine release → fibrogenesis

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Describe what you see on HE and grossly due to chronic exposure to quartz leading to silicosis

Gross we see Polarizable quartz particles and “Egg-shell” calcification in hilar lymph nodes

HE: Nodular opacities with concentric layers of collagen

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What are complications of silicosis?

cor pulmonale; association with Caplan
syndrome with increased risk for TB (silicotuberculosis) and cancer

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Two Asbestos forms
–______ (e.g. – chrysotile) – curly and flexible
–______ (e.g. – crocidolite) – straight and rigid

Serpentine

Amphibole

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deposition sites of asbestos: Source of asbestos:

– respiratory bronchioles, alveolar ducts and alveoli Sources:Insulation around pipes in old naval ship, Roofing material used over 20 years ago, Demolition of old buildings

What are ferruginous bodies?

macrophages phagocytose asbestos fibers and coat them with ferritin (iron and protein

What are our asbestos releated diseases?

Benign pleural plaques (not precursor of mesothelioma) diffuse interstitial fibrosis, bronchogenic carcinoma, mosothelioma, NO incraesed risk for TB, cor pulmonale and Caplan syndrome

What do we see on HE and gross for asbestos?

increase in collagen, much more thickened Gross you will see pleural plaques--little nobs all over

Berylliosis * Beryllium – where from? * \_\_\_\_\_\_\_ inflammation – TB and sarcoidosis (differential diagnosis) * Complications – _____ and \_\_\_\_\_

Nuclear and airspace industry Granulomatous cor pulmonale and lung cancer

• Multisystem granulomatous disease of unknown etiology • 25% cases of chronic interstitial lung disease

Sarcoidosis

Where and what population do we see infected with sarcoidosis?

highest in scandanavia, AA, F, and 70% less then 40 years old \*\*non-smokers

Sarcoidosis • Disorder of immune regulation • Unknown antigen → Interaction with \_\_\_\_\_\_ cells → cytokine release → recruitment of \_\_\_\_\_\_\_\_\_ → non-necrotizing granuloma formation

CD4 TH monocytes/histiocytes

Sarcoidosis is a diagnosis of

exclusion

What systems are involved in sarcoidosis?

Skin; nodular granulomatosis Eyes Liver; granulomatous hepatitis salivary and lacrimal glands, diabetes insipidus, bone marrow and splenic invovlement

Sarcoidosis ↑ed ________ levels as a marker of disease activity and response to steroids

angiotensin converting enzyme (ACE)

– Hypercalcemia (5% cases) – Polyclonal gammopathy – Cutaneous anergy (lack of response to common skin antigens (candida) due to consumption of CD4 THcells

Sarcoidosis

What do you see on chest xray in sarcoidosis

bilateral hilar adenopathy adn reiculonodular shadows in lungs

On HE of lung with sarcoidosis, what do you see?

interstitial granulomas has a **lymphatic flow distinct pattern** in the interstitium and see they are around vascular bundles

In this bronchiole is it surrounded by a granuloma full of ______ . Characteristic of what disease?

histocytes sarcoidosis

What kind of cell is in the picture?

Langhans giant cell, seen in sarcoidosis

Prognosis of sarcoidosis

do fairly well, you tx with steroids and pts will respond See variable responses from spontaneous remission and relapse to progressie interstitial fibrosis and cor pulmonale and death in 10-15%

– Farmers’ lung – Silo fillers’ disease – Byssinosis are all examples of:

Hypesensitivity pneumonitis

``` Inhaled antigen (known or unknown) producing granulomatous interstitial pneumonitis (extrinsic allergic alveolitis) ```

Hypersensitivity pneumonitis

Hypersensitivity pneumonitis: Type ____ hypersensitivity reaction First exposures we see _____ in serum Second exposure we see:

Type III hypersensitivity rxn First exposure: IgG antibodies in serum Second exposure: antibodies combine w/ inhaled antiG to form inmume complexes--\> inflammatory response in lung

What happens during the chonic phase of hypersensitivity pneumonitis

Granuloma fomration (Type IV hypersentivitiy response)

Causes of farmers lung; Silo fillers disease Byssinosis

farmers lung = moldy hay- thermophilic actinomycetes bacteria silo fillers = inhalation of gases from plant material (oxides of nitrogen) Byssinosis = cotton/linen/hemp and textile factory workers and monday morning blues

Interstitial and alveolar infiltrates of inflammatory cells, peri-bronchiolar accentuation, ill-defined granulomas

Interstitial lung disease: HSP

How do you diagnose Interstitial lung disease, specifically HSP?

Clinico-pathologic diagnosis – symptoms and physical findings, x-ray abnormalities, PFTs, immunologic features (Abs to suspected Ags); lung biopsy may be needed

What do we see on HE for interstitial lung disease

Granuloma, but not as well circumscribed as sarcoidosis

\_\_\_of chronic interstitial lung disease are idiopathic and usually \_\_\_\_\_\_pneumonias are idiopathic

15% usual interstitial pneumonia is idopathic

Idiopathic pulmonary fibrosis is more: male or female occurs at what age duration of symptoms

M\>F 40-70 yrs duration of syptoms 18-24 months

Clinical manifestations of idiopathc pulmonary fibrosis:

– Dyspnea; non-specific constitutional symptoms such as fever, weight loss, fatigue, arthralgias; cough

Pulmonary fibrosis • Repeated injury to the lung → alveolitis → cytokine release → \_\_\_\_\_\_\_\_\_

interstitial fibrosis

Idiopathic pulmonary fibrosis: Interstitial fibrosis → irregular dilatation of adjacent airways →

honeycomb lung (end stage interstitial fibrosis)

Honeycomb cysts are typically seen in:

Idiopathic pulmonary fibrosis

Course of idiopathic pulmonary fibrosis

progressive disease--end-stage lung; cor pulmonale

– Interstitial lung disease in 50% of patients – Wide spectrum of pulmonary changes; common manifestation – pleural effusion (unexplained pleural effusion in a young woman likely to be this).

Systemic lupus erythematosus (SLE)

Wide spectrum of pulmonary changes • Rheumatoid nodules (when associated with pneumoconiosis – Caplan syndrome) • Interstitial fibrosis • Pleural effusions

Rheumatoid arthritis (RA)

– Interstitial fibrosis with pulmonary vascular hypertrophy – Commonest cause of death

Systemic sclerosis (scleroderma)

A 66-year-old man has had increasing dyspnea for about a year. He is a smoker. He is retired from the construction business. There are some rales auscultated in both lungs on physical examination. A chest radiograph reveals bilateral diaphragmatic pleural plaques as well as diffuse interstitial lung disease. A sputum cytology shows no atypical cells. These findings are most likely to suggest prior exposure to which environmental agents?

Asbestos fibers

A 50-year-old African American woman presents with increasing shortness of breath, fever, weight loss, and night sweats for the past 4 months. She is a non-smoker. On examination, there are fine rales auscultated in all lung fields. A chest radiograph reveals hilar lymphadenopathy and a reticulonodular pattern in all lung fields. A transbronchial biopsy is performed and microscopically demonstrates numerous small pulmonary interstitial non-caseating granulomas. Which of the following is most likely?

Sarcoidosis