L6- Restrictive pulm dz, HP, adn ARDS

restrictive pulm disease

• volume issue

• reduction in inspiration of air, lungs are restricted from expanding

restrictive pulm disease- intrinsic

• parenchymal

• lung tissue itself.leading to stiffening, scarring, or inflammation

examples of intrinsic restrictive pulm disease

• idiopathic pulm fibrosis

• sarcoidosis

• pneumoconiosis

restrictive pulm disease- extrinsic

• extrapulmonary

• oobesity, chest wall def

• pleural disease

• CHF

PFTs

• decreased FEV1 (>80% of predicted normal)

• decreased FVC (<80% od predicted normal)

  • decreased more than FEV1 bc not bringing in enough air/restricted volume

• FEV1/FVC ratio normal (>0.7)

  • or increased

restrictive disease- lung volume

• decreased. TLC

• decreased FVC

restrictive disease- lugn compliance

• decreased

• cannot expand normally

restrictive disease- problem

• voluem problem: issue with full inspiration

restrictive disease- PFTs

• normal of increased FEV1/FVC

• FVC more decreased than FEV1

idiopathic pulmonary fibrosis (IPF)

• progressive extensive remodeling and scarring of the lungs due to an unknown cause

• AKA- idiopathic fibrosing interstitial pneumonia

IPF patho

• epithelial injury and senescence

• failed repair and abnormal signaling

• fibroblast activation→ excessive collagen ad extracellular matrix causing lung tissue to become thick and stiff

• self perpetuating fibrosis- increasing ECM stiffness further activated mechanotransduction pathways promoting additional fibroblast activation and matrix production

IPF- epi

demograpohics

• males >50

causes

• unknown

• amiodarone

IPF- RF

• strong association with smoking

• environment exposures: stone, metal, wood, organic dusts, and air pollutants

• small genetic component

IPF- Sx

• gradual onset

  • dyspnea on exertion

  • chronic, dry cough

  • fatigue, WL, anorexia

IPF- Physical exam findings

• diffuse, fine, dry, bibasilar inspiratory crackles

  • velcro crackles

  • classic sign

• clubbing

IPF- workup

• labs: not useful

• imaging:

  • cxr- often initial

  • HRCT chest (prefered)

    • required fro dx

• PFT:

  • restrictive pattern

  • decreased lung volume (TLC, RV)

  • normal or increased FEV1/FVC

• biopsy: may be required

IPF- CXR findings

• diffuse reticular opacities

  • lower adn peripheral lung

• decreased lung volume

IPF- HRCT findings

• reticular bibasilar and subpleural opacities with associated honeycombing

  • honeycombing: alveoli became permanently dilated with dense interstitial surrounding them

• diminished lung volume

• traction bronchiectasis

  • irregular dilation of bronchi and bronchioles caused by pulling forces from surrounding lung fibrosis

  • not caused by primary airway dz such as CF

  • found in fibrotic lung dz

IPF- dx

• compatible clinical presentation

• exclude other causes of interstitial lung disease

  • asbestos exposure, hypersensitivity pnoeumonitis, systemic sclerosis, rheumatoid arthritis

• restrictive pattern PTFs

• typical HRCT findings

  • subpleural, bibasilar predominance of reticular markings, homeycombing, traction bronchiectasis

  • when the results of HRCT are not classic for IPF surgical lung biopsy os often necessary

IPF- histopahtology

• usual interstitial pneumonia

• characterized by fibrosis and honeycomb pattern alternating with areas of normal lung parynchema patchwork pattern

• honeycombing is often subpleural and worse in lower lobes

IPF management- supportive/preventative care

REFER TO PULM

• supp O2

• prevention of pulm infections and acute exacerbation

  • immunizations

  • early outpt tx of respiratory infection

  • palliative care

IPF management- pt education

• progressive pulm disease with poor prognosis

• end of life issues and advances directives

• maen survival is 3-7 years

IPF management- medical therapy

• no medication has been found to cure IPF

• antifibrotics reduce rate of decline in lung function → do NOT effect mortality

IPF management- lung transplantation

• the median survival following lung transplantation fro IPF is estimated to be 5.2 years

sarcoidosis

• systemic inflammatory granulomatous dz of unknown etiology, characterized by the formation oof noncaseating granulomas in affected organs, MC lungs and thoracic lymph

sarcoidosis- epi

• females

• 20-40yo

• incidence highest in:

  • black americans

  • northern europeans

sarcoidosis- patho

exact etiology unknown

• exaggerated T cell response to antigens or self antigens leading to immune system activation

• immune response leads to formation of forms noncaseating granulomas

• in most pts, granulomas resolve spontaneously within 2-5yrs

• if htey persist, they can cause progressive fibrosis

sarcoidosis- clinical findings

50% are asx- incidental CXR

• constitutional sx:

  • anorexia, WL, fever, malaise

• pulm:

  • dry cough, dyspnea

  • rales

• LAD:

  • intrathoracic on imaging

• skin:

  • erythema nodosum- classic

  • lupus perino- specific to sarcoidosis

• eyes:

  • anterior uveitis

• neuro:

  • cranial nerve palsies- CN VII

• cardiac:

  • cardiomyopathies, arrythmias

• MSK/rheum

• - arthralgias, arthritis, bone lesions

sarcoidosis- clinical findings

• 5-10% present with lofgren syndrome

  • acute onset fever

  • hilar adenopathy

  • erythema nodosum or bilateral ankle inflammation

sarcoidosis workup

no single dx gold standard

• based on:

  • compatible clinical findings

  • rad findings

  • biopsy findings

  • exclusion of other potential causes

sarcoidosis- labs

• increased ACE levels

• hypercalcemia and hypercalciuria

• leukopenia

• increased ESR

sarcoidosis- PFTs

• normal in most pts

• restrictive patterns

• small % of obstructive pattern

sarcoidosis- biopsy

• demonstrating noncaseating granulomas

sarcoidosis- CXR findings

• bilateral hilar lymphadenopathy

• parenchymal involvement:

  • reticular infiltrates

  • nodules

  • cavitation

  • pleural effusion

sarcoidosis- HRCT findings

• eval abnormalities seen on CXR

• can detect parenchymal and mediastinal abnormalities that are not well delineated on CXR

sarcoidosis management- asx

• observation

• no tx recommended (spontaneous remission often occurs)

sarcoidosis management- symptomatic

• 1st line- oral steroids

  • prednisone

  • long term therapy usually required over months to years

  • decrease to min effective dose

• alternative: immunosuppressive meds

  • indication: steroids not working, steroids causing disease, pt is intolerant

  • ex. methotraxate, azathioprine

sarcoidosis prognosis

• overall is good

• best fro stage 1 or with erythema nodosum

• 20% with lung involvement suffer irreversible lung impairment

flow of occupational pulmonary disease

• small dust mineral particle os inhaled

• particle makes it to the respiratory bronchioles and alveoli

• alveolar macrophages engulf particle

• macrophage induces and intense inflam rxn

• with regular, exposure, neutrophils and fibroblasts also become involved

• regular exposure leads to constant collagen disposition by fibroblasts

pneumoconiosis

• group of occupational lung disease caused by inhalation and deposition of mineral dusts in the lungs, leading to chronic inflammation and progressive pulm fibrosis

• fibrotic vs non fibrotic

fibrotic pneumoconiosises

• silicosis

• coal workers pneumoconiosis

• asbestosis

• berylliosis

• byssinosis

pneumoconiosis- occupational hx

• clinically- keep this in mind for respiratory complaints

• PANCE-testing- pay attention to any occupational hx given in questions

coal worker’s pneumoconiosis- causative agent

• inhaled coal dust particles (also silica)

• occupations:

  • coal mining

coal workers pneumoconiosis- sx

• simple

  • usually asx

• complicated

  • progressive dyspnea

  • cough

    • occasionally black tinged

coal worker’s pneumoconiosis- PE

fine crackles

coal workers pneumoconiosis- workup

CXR

• SCWP: small nodules usually in upper lung

• PMF: large opacities classically bilateral and upper lobe predominant

PFTs

• not very helpful

• obstructive pattern (coal dust induced emphysema and airway dz)

lung biopsy

• not needed fro dx

• dark black lung

• black pigment landed macrophages

coal workers pneumoconiosis- mgmt

• exposure cessation: proper use of respirators or protective equipment

• supportive therapy: bronchodilators, O2, smoking cessation, abx fro exacerbations

• pulm rehab

  • breathing re training

  • low to high intensity exercise

  • endurance and strength training

• lung transplant for end stage

silicosis- causitive agent

• inhaled free silica particles (silicon dioxide)

• crystalline silica is in quartz, granite, and sandstone

• occupations that aerosolize these get exposed

• sig increase risk of TB

silicosis- occupations

• mining

• quarrying

  • granite, slate, quartz, sandstone

• drilling/cutting

  • stone

• sandblasting

• ceramics

• glass and cement manufacturing

silicosis- sx

chronic:

• often asx unless severe

• dyspnea on exertion, non productive cough

acute:

• dyspnea

• cough

• WL

• fatigue

silicosis- PE

• mya hear crackles

silicosis- workup

• all pts with silicosis should have TB skin test and chest radiograph to rule out TB

• PFT: mixed obstructive/restrictive pattern

• lugn biopsy: dust alden macrophages and loose reticulin fibers in the lung

silicosis- CXR

simple:

• pattern of small and round or irregular opacities

complicated:

• large conglomerate opacities that equate to progressive massive fibrosis

findings:

• small rounded opacities in upper lung fields

• egg shell calcification= classic

  • calcification of hilar and mediastinal lymph nodes

silicosis- management

• complete avoidance or exposure minimization to silica

  • proper use of respirators or protective equipment

• supportive:

  • stop smoking

  • bronchodilators if obstructive PFT pattern

  • O2

  • pulm rehab

asbestosis

• inhaled asbestos fibers

• asbestos is naturally occurring fibrous mg silicate that can be women into thermal insulating material

asbestosis- occupations

• constriction

  • destruction, repair, renovation of old buildings

• insulation

• shipbuilding, shipyard workers

• pipe fitters

• fire resistant prods

asbestosis- sx

• typical latency of >20 yrs from first exposure to sx onset

  • slowly progressive dyspnea

  • cough

asbestosis- PE

• bi basilar end inspiratory crackles

• clubbing

asbestosis- work up

CXR:

• linear opacities at lung bases

• pleural plaques and calcifications

PFTs

• restrictive pattern

• decreased lung volume and compliance

biopsy

• ferruginous bodies- linear asbestos

asbestosis- CXR findings

• pleural plaques

  • bilateral parietal pleural thickening or calcification

  • usually in mid to lower lung

• lung parenchyma

  • irregular linear opacities in lower lobes with or without honeycombing

asbestosis- mgmt

tx:

• no curative or specific tx

• bronchodilators, supplemental O2 PRN, steroids, lung transplant

complications:

• pleural thickening and pulm fibrosis

• increased incidence of lung cancer, esp when combined with cigarette smoking

• exposure to asbestos is associated with mesothelioma

berylliosis

granulomatous pulm dz similar to sarcoidosis but caused by beryllium exposure

berylliosis- occupations

• aerospace

• nuclear weapons and energy

• electronics

• ceramics

• dental alloys

• computer manufacturing

• military personnel and construction workers

berylliosis- sx

• dyspnea and dry cough

• joint pain, fever, night sweats

• fatigue WL

berylliosis- workup

CXR

• hilar lymphadenopathy

• increased interstitial lung markings

• similar to sarcoidosis

labs:

• beryllium lymphocyte proliferation test

PFT:

• restrictive > obstructive

Biopsy:

• non caseating granulomas

berylliosis- mgmt

supportive

• prevention: flu, and pnemo vaccination, smoking cessation

• avoidance of beryllium

• PRN; O2, pulm rehab

tx:

• asx or mild exertional dyspnea: ICS

• sx with respiratory impairment of PFT: oral pred

hypersensitivity pneumonitis

• pulm illness caused by hypersensitivity to environmental antigens:

  • organic antigens: microbial, avian, animal

  • inorganix agents

• acute (non fibrotic) or chronic (frbrotic)

• not associated with asthma or atopy

• occupational pulm dz

hypersensitivity pneumonitis - patho

• repeated exposure to antigens

• acute neutrophilic and mononuclear alveolitis

• interstitial lymphatic infiltration AND non caseating granulomas reaction

• continued exposure

• progressive fibrotic dz with bronchodilator obliteration in chronic dz

hypersensitivity pneumonitis- etiologies

• environmental antigens- look for occupation given in question stem

  • think dairy farmer, pigeon fancier, mushroom picker

  • constantly inhaling some antigen

hypersensitivity pneumonitis- clinical presentation- acute

non fibrotic (rapid onset)

• sx:

  • occur 4-8 hours after porlonged exposure to antigen

  • dry cough

  • dyspnea

  • malaise

  • low grade fever, chills

• mimics viral syndrome

• physical exam:

  • inspiratory squeaks

hypersensitivity pneumonitis- clinical presentation- chronic

• insidious chronic dry cough

• slowly progressive dyspnea, anorexia, adn WL

• often from lon term, low level exposure

  • birds or household molds

hypersensitivity pneumonitis- dx

difficult to dx

• exposure hx

• CT findings:

  • nonfibrotic: ground glass opacities, ill defined centrilobal nodules, mosaic attenuation, air trapping in a diffuse bilateral dist

  • fibrotic: superimposed traction bronchiectasis, reticulation, and honeycombing; may mimic UIP or NSIP patterns

• serum IgG antibodies to sus antigens

• bronchoalveolar lavage with lymphocytes >20-30%

• lung biopsy (if others inconclusive)

hypersensitivity pneumonitis- tx

• id offending agent and avoid further exposure

• severe cases:

  • prednisone fro 2 weeks then taper

acute respiratory distress syndrome (ARDS)

• clinical syndrome of acute hypoxemic respiratory failure characterized by bilateral pulm infiltrates due to noncardiogenic pulm edema

• results from diffuse injury to alveolar capillary barrier

ARDS- RF/etiology

• develops in critically ill pts

  • no pulm sepsis

  • PNA

  • severe trauma

  • aspiration of gastric contents

ARDS- patho

• an inflammatory response occurs (ex. sepsis)

• alveolar epithelium and capillary endothelium are damaged

• fluid fiils alveoli (diffuse pulm infiltrates)

• gas exchange is prevented

• hypoxemia + dyspnea

ARDS- dx

suspect ARDS in pts with:

• dyspnea: rapid onset; often progresses to respiratory failure

• hypoxemia: usually refractory

  • alveoli full of fluid, therefore cannot get air in

• alveolar infiltrates: on CXR within 6-72 hrs of precipitating event

ARDS- physical exam findings

• tachypnea

• accessory muscle use

• crackles

ARDS- CXR findings

• bilateral diffuse pulmonary infiltrate (airspace dz)

• similar to CHF but classically spares the costophrenic angles

ARDS- mgmt

1) treat underlying cause that led to it

2) prone positioning >12 hrs/day fro severe

• helps ventilate and decrease ventilation injury

3) supplemental O2

• goal: PaO2>55mmHg, SPO2 88-95%

4) lung protective ventilation

ARDS- lung protective management

1st try: low tidal volume ventilation

• limit TV and airway pressure to prevent ventilator induced lung inry

• TV 6mL/kg predicted body weight with plateau pressure <30

• if not working, proceed to PEEP

Positive end - respiration pressure (PEEP)

• prevents alveolar collapse (prevent recurrent atelectasis of the alveoli at the end of expiration)

• improves hypoxemia, VQ mismatch, increased FRC

• high PEEP is preferred over low

additional ARDS mgmt

• corticosteroids

• neuromuscular blockade (we can control actual ventilation)

• fluid mgmt

• VV-ECMO if refractory to everything else