ILD - Known Origins

Categories

• inorgranic

• organic - hypersensitivity pneumonitis

• irritant gases

• medications and illicit drugs

• radiation therapy

what are the systemic diseases

• connective tissue disease

• sarcoidosis

Asbestosis def

Asbestos fibres are a mixture of fibrous minerals composed of hydrous silicates of magnesium, sodium and iron in various proportions (typically 0.5 um in diameter)

Common uses for asbestos

• buidling materials (floor tiles,insulation,roofing materials)

• automobile materials (undercoating, brake lining, clutch casinig)

• flame retardant materials (stage curtains, uniforms for firefighters)

Prolonged inhaltion of asbestos fiber can cause

lung cancer, asbestosis, mesothelioma

asbestos is hazardous when the fibers are

distrubued and become airborne

patient with asbestosis have considerable exposure for many years before presentation of disease of how many years

> 20 years

pathophysiology

inhaled asbestos fibres are deposited at alveolar duct bifurcations → alveolar macrophages release cytokines → laying down of collagen → fibrosis

what is it calld when calcification of fibrotic tissue called

fibrocalcific pleural plaque

Radiographic abnormalities

• pleural changes

  • plaque

  • fibrosis

  • effusions - benign asbestos pleural effusion

  • atelectatsis

  • mesothelioma

• bilteral, lower zone reticulonodulardular infiltrates

• parenchymal scarring

• lung cancer

parenchymal fibrosis from asbestos exposure

• multi-lobe → involvement is most common

• lower lobe → most commonly affected

• pleural calcification → pleural plaques or rounded atelectasis

Diagnosis

transbronchial biopsy (TBB) or surgical biopsy

clinical presentation

• nonspecific

  • Slow progressive dyspnea on exertion

  • crackeles O/A

  • PFT: restrictive imparient with decrease DLCO

  • non productive cough

Treatment and prognosis

• no proven treatment for asbestosis

• management is supportive in nature

• severe impairment is 30 - 40 years after exposure

Malignancy Mesothelioma

rare, agressive form of cancer that develops in the lining of the lungs or abdomen

what is it called when mesothelioma reaches the adomen and lining of the lungs

Peritoneal and pleural mesothelioma

what is mesothelioma causes by

asbesotos mesothiloma has no cure and has a poor prognosis 1

what is the percentage of suriving asbestos prognosis

10% 5- years

Coal’s Worker’s Pneunoconiosis (CWP) → Organix exposure

• pulmonary deposition and accumulation of large amounts of coal dust

• coal miners lung, black lung

disease prevalance varies in different areas of the country and from

mine to mine bc coal content varies by region

two types of CWP

1. simple CWP

2. complicated CWP

characterized by

multiple small nodular opacities on CXR that occur at the site which cal dust aggregated

where is simple CWP develop around

first and second generation respiratory bronchioles and casue adjacent alveoli retract (Focal emphysema)

focal emphysema

dilation of the terminal and respiratory bronchioles

Asymptomatic - simple CWP

SOB and cough do not deveclop until disease progresses to massive fibrosis

Complicated CWP

• progressive massive fibrosis

• fibrotic nodules > 1cm in diameter

where does nodules appear

peripherial regions of upper lobes and extend to the hilum

nodules composed of dense collagenous tissue with

black pigmentation

CWP pathophysiology

• coal dust that enters the of lungs can either be destroyed nor removed

• coal dust accumulates → coal macules → nodules → emphysema and fibrosis

clinical presentiaotn CWP

• simple → largely asymptomatic

• complicated CWP → cough, dyspnea. and lung function impairment

IF DISEASE ADVANCES, COR PULMONALE MAY BE FOUND

Treatment prognosis -CWP

• treatment is supportive & pallitive

  • Supp O2 & bornchodilators prn

• prognosis is poor one the pt has been determined have complicated CWP

Inorgnaic exposure → Silicosis

• person with choronic silicosis typically have had more than 20 years of silica exposure

  • more than 30 years, 12% decloped silicosis

the number of people dying with silicosis in NA has __ dramatically because of improved workplace protocols

declined

cigarette smoking adds to the lung damage caused by

silica

Silicosis pathophisology

• alveolar macrophages engulf inhaled free silica particles → nodules → coaleces to form large masses of fibrous tissue

• upper lobe predominant abnormalities characterized by multiple small nodular opacities in central lung tissue

• small nodules are asymptomiac

• nodules may coalesce into large midlung zone mase

progressive massive fibrosis is

modules may coalesce into large midlung zone mases

two types of silicosis

simple

complicated

what is simple silicosis

• pattern of small and round nodules scattered thorughout the lung

• no songle nodule is >9 mm diameter

• typically asymptomatic

what is complicated silicosis

• nodules that caolesce and form large masses of fibrous tissues; upper lobes and perihilar region

• severe-cases - fibrotic regions may undergo necrosis and cavitate

silicosis clinical presentation

• exertional dyspnea

• varible sputum production

• many patients have mixed obstructive/restrictive PFTs with decrease DLCO

treatment and prognosis →.silicosis

• patient with very advance silicosis and without other significant comorbid conditions, lung transplantation should be consdired

• at the time of disgnosis is somewhat predictive of prognosis but the rate of progression varies

• patients have higher increase risk of lung cancer & active TB

inorganic exposure → berylliosis

an extremely light metal found in certain plastics, ceramics, rocket fuel & x-ray tubes

chronic beryllium lung disease (CBD)

development of granulomas and diffuse interstitial inflammatory reaction

Berylliosis pathophysiology

• acute inhalation of beryllium fumes or particles can cause a toxic or allergic pneumonitis sometimes accompanied by rhinitis, pharyngitis, tracheobronchitis

• more complex form of berylliosis → development of granulomas and diffuse interstitial inflammatory reaction

clinical presentation - berylliosis

inspiratory crackles O/A

berylliosis treamtment and prognosis

• corticosteroid

• oxygen supplementation and in certain advance cases lung transplantation

Ogranic exposure - hypersensitivity pneumonitis (HP)

cell mediated immune response of the lung cause by repeated inhalation of variety of offending agents or antigens

what antigens cause HP

• bacterial

• fungi

• animal protein

• chemicals

HP - patients chronically exposed to low level of inhaled antiagents may develop

subtle interstitial inflammatory reactions in the lung that doesn’t result in noticeable symptoms for months to years

HP prevelance and incidence of HP appear to vary considerably depending upon

• intensity of exposure to inciting anitgens

• season

• geographical conditions

• proximity to certain industries

only small proportion of exposed individuals develop clinically significant HP

Clinical presentation HP → acute

few days of SOB, chest pain, fever chills, cough (may productive)

Chronic HP

pulmonary interstitial inflammatory reaction → no noticeable symptoms for months to years

• eventually present with severe impairing disease

• restrictive pattern observed in acute or subacute disease

treatment and prognosis HP

• strict antigen avoidance

• immunosuppression with corticosteroids in symptomatic or physiologically impairing disease

therapeutic exposures - medication & illicit drugs

• chemotherapeutic agents

• antibiotics

• immunosuppressive agents

• antiarrhythmic drugs

• illicit drugs

• misc agents

Risks for the drugs for causing ILD __ related to cumulative dosage

directly

diagnosis is confirmed by

open lung biospy

ILD s found without any infectious ogranism or nown exposure, what do we suspect

drug induced interstitial process must be suspected

therapeutic exposure pathophysiology

unknown

clinical presentation

• physical findings of drug-induced lung disease are nonpsecific ( breath sounds decreased)

treatment and prognosis

• avoidance of further exposure

• corticosteroid

• prognosis is variable and depends on the specfic drug

complications of drug-induced pulmonary toxicity include

fibrosis, resp failure, pulmonary embolism, pulmonary HTN, PTX

radiation therapy

presenting within 6 months of radiation therpay generally have ground glass abnormalities thought to respresnt acute inflammation

radiation therapy 2 categories

acute pneumonitic phase

late fibrotic

acute pneumonitic phase

develop 2-3 months after exposure

late fibrotic phase

may develop immediately after the acute pneuminotic phase, without an acute pneumonitic period or after a symptom free latent period

fibrosis generally develops 6 - 12 months after radiation exposure; Pleural effusiom assocaited with this phase

prcire cause od radiation induce lung disease is

not known

diagnosis similar to drug-induced lung disease

irritant gases

inhalation of irritant gasses may cause chemical pneumonitis; in severe cases can cause ILD

effects of irritant gases depends on the extebt and duration of eposure and specfic agent

• chlorine

• ammonia

• ozone

• nitrogen dioxide

• phosgene

irritant respiratory damage is related to

concentration of the gas and solubility

mroe water soluble gassess dissolve in the upper airway and immediately cause muscous membrane irritation

connective tissue disease

Well- known complication of connective disease

most common implacted disoders in connective tissue disease

• scleroderma

• rheumatoid arthritis

• systemic lupus erythematosus

severity of pulmonary and non-pulmonary manifestations do

not always correlate

what are the most common symptoms of connective tissue disease

• dypsnea and cough, crackles and wheezing O/A

PFT resutls in connect tissue disease

restrictive with decrease DLCO

CT findings

variable 0 most commonly see in. NSIP and orgnaizing pneumonia features

• NSIP - GGO on HRCT

• organizing pneumonia - patchy consolidated lungs with air bronchograms

  • both in NSIP and OP can resolve with aggressive immunosuppression

• Fibrotic injury - manifests as UIP

  • reticular fibrotic opacities and honeycomb cystic changes on HRCT

scleroderma

relative rare disease characterized by chronic hardening & thicking of the skin caused by new collagen formation

• scleroderma of the lung appears in the form of ILD & fibrosis

scleroderma is one collegan vascular disorder that has pulmonary involvement in severe way which causes

significant scarring of the lung parenchyma

pulmonary complication include

• diffuse interstitial fibrosis

• severe pulmonary HTN

• aspiration pneumonitis (secondary to esophageal involvement)

on set sage in scleroderma

30 to 50 , female to male ratio 3:1

scleroderma pathophysiology

microvascular injury characterized by structural and functional endothelial cell abnormalities → diffuse interstitial fibrosis, severe pulmonary HTN, disease & aspiration pneumonitis

clinical presentation - Scleroderma

restrictive pattern consisting of decrease lung volume & diffusion capacity

Treatment and progonosis - Scleroderma

7-% 5 year survival

mycophenolate (immunosuppressive) Treatment of choice

connective tissue diease - Rheumatoid Arthritis

primarily an inflammation joint disease but it may involve the lungs

RA - pulmonary manifestations include

Pleurisy

interstitial pneumonitis

necrbiootic modules with or without cavities

Caplan’s syndrome

pulmonary HTN secondary vasculitis

pleurisy

with or without effusion (typically unilateral, R more common)

interstitial pneumonitis

• alveolar wall fibrosis, interstitial and intraalveolar cell infltrations. lymphoid nodules

necrobiotic nodules with or without cavities

gradual degeneration and swellng of lung tissues

Caplan’s syndrome

progressive pulmonary fobrosis

characterized by rounded densities in lung periphery;cavity and calcification

Systemic Lupus Erythematosus (SLE)

multisystem disorder that mostly involves the joints & skin

pulmonary involvment occurs in about __ of cases in SLE

50 - 70%

pulmonary manifestations are characterized by

• pleurisy with or without effusions

  • exudative effusion, high protein concentration; bilateral

• atelectasis

• diffuse infiltrates and pneumonitis

• Diffuse ILD

• uremic pulmonary edema

• diaphragmatic dysfunction - myopathy affecting the diaphragm

• infections

Sarcoidosis

idopathetic multisystem inflammatory disorder that can invole many systems

area effects by sarcoidosis

eyes lungs spleen liver skin mucous membranes salivary glands

sarcoidosis characterized by formation of

granuloma

manifestation of sarcoidosis include

asymptomatic hilar adenopathy: enlargement of mediastinal lymph nodes

less common: parenchymal opacites in midlung zone

what is one clinical hall mark of scarcoidosis

increase of all 3 immunoglobulins (IgM, IgG and IgA) and elevation of ACE

sarcoidosis symptoms

cough

chest pain

dyspnea

wheezing

PFT: normal, restrictive, obstructive or mixed - all with decrease DLCO

CXR sarcoidosis

hilar lymph mode enlargement bilaterially

diffuse reticulogranular pattern

Treatment sarcoidosis

corticosteroid

sarcoidosis prognosis

• majority is temp condition, recover with little to no long term effects

• 10-15% is chronic conditions