Diffuse Parenchymal Lung Disease Flashcards by Daniel Guck

This is the pulmonary alveolar and capillary endothelium and the spaces between the perivascular and perilymphatic tissues. Centrally, it involves the peribronchiolar and peribronchial tissues.

Parenchyma

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Which of the following are “major” idiopathic intersititial pneumonias?

IPF, LIP, NSIP, RBILD, PPFE, DIP, COP, AIP

IPF
NSIP
RBILD
DIP
COP
AIP

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What is the gold standard imaging for DPLD?

HRCT

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True/False: bronchoscopy is indicated for all establishment of diagnosis for DPLD.

FALSE

It’s useful to exlude other infection, DAH, and eosinophilia

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What WBC cell line is increased on BAL in NSIP?

BAL usually shows >20% lymphocytes

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There is usually a mixed pattern of WBCs on BAL in COP, but what happens to the CD4/CD8 ratio?

Usually decreased

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BAL showing >5% CD1a-positive cells is diagnostic of what DPLD on BAL?

Pulmonary Langerhans cell histiocytosis

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BAL showing eosinophilia (>20-25%) is seen in what DPLD?

Acute and chronic eosinophilic pneumonia

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What is the gold standard for diagnosing most IIP?

VATS surgical lung biopsy

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True/False: UIP is seen only on pathology in patients with IPF

False

Can be seen in connective tissue diseases, asbestosis, drug-induced lung disease, and environmental and occupational exposure

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Median survival of IPF after diagnosis?

3-5 years

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Where is the fibrosis on CT in IPF?

Basilar and peripheral fibrosis, microscopic subpleural and paraseptal fibrosis

Also see some honeycoming

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True/False: this patient needs a surgical lung biopsy for the diagnosis of IPF.

65 y/o presents with dyspnea. CT shows fibrosis in subpleural site, basilar predominancy, reticular abnormalities, and honeycoming

FALSE

This pattern is classic for IPF, therefore no Bx needed

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True/False:

There are associations between IPF and history of smoking, GERD, and occupational/environmental exposures

TRUE

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FVC and DLCO cutoffs for lung transplant listing in IPF?

FVC < 60% predicted

DLCO < 40% predicted

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Which one is typically steroid responsive - NSIP or IPF?

NSIP

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This form of NSIP shows mild to moderate intersitial chronic inflammation, type II pneumocyte hyperplasia in areas of inflammation

Cellular NSIP

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This form of NSIP has dense or loose interstitial fibrosis with uniform appearance, lung architexture is frequently preserved, and there are absence of fibroblastic foci

Fibrotic NSIP

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Which is more steroid responsive and has a better prognosis, fibrotic or cellular NSIP?

Cellular NSIP

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What are the known
genetic associations with
pulmonary fibrosis?

Mutations in hTERT and
hTR are risk factors for
pulmonary fibrosis
underlying the inheritance
in 8–15% of familial cases.
In these families, IPF is
inherited as an autosomal
dominant trait with agedependent
penetrance.

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What is seen on CT for NSIP?

Diffuse GGO’s
Reticular opacities
Traction bronchiectasis
NO honeycoming

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This medication used in the treatment of NSIP has the following side effects:

Hyperglycemia, HTN, insomnia

Glucocorticoids

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This medication used in the treatment of NSIP has the following side effects:

GI upset, abnormal LFTs

Azathioprine

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This medication used in the treatment of NSIP has the following side effects:

GI upset, abnormal LFTs, leukopenia

Mycophenolate mofetil

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This medication used in the treatment of NSIP has the following side effects: Bladder cancer, neutropenia

Cyclophosphamide

This form of DPLD is believed to follow an inciting injury that causes chronic lung inflammation, buds of intra-alveolar granulation tissue, this causes alveolar epithelial injury with cell death and denudation of basal laminae. Fibroblasts then deposit matrix in layers creating appearance intra-alveolar buds.

COP/BOOP BOOP is a way better name. Why did they change that.

So what is seen on histopathology in COP?

Intra-alveolar buds of granulation tissue made of myofibroblasts, fibroblasts, and loose connective tissue in similar age

What is seen on CT scans in COP?

Multiple migratory patchy alveolar infiltrates (GGO's or consolidation that are preipheral/basilar, can also see air bronchograms

True/False: most patients need a surgical lung biopsy for the diagnosis of COP

TRUE Have to r/o other conditions, and TBBx are less reliable because of the patchy nature of the disease

Mainstay of treatment for COP?

Steroids, usually need long course (6-12 months)

This form of DPLD is rapidly progressive, can have viral prodrome, similar to ARDS but without a specific cause

AIP (Hamman Rich Syndrome)

What is seen on biopsy of AIP?

Diffuse alveolar damage

What are the 2 stages of AIP?

Initially there is edema, hyaline membranes, and microbascular thrombi --> loose organizing fibrosis with alveolar septa and type II pneumocytes

What is done to confirm the diagnosis of AIP?

Sugical lung biopsy - shows DAD

Treatment of AIP?

Thoughts and prayers

This DPLD is characterized by interstitial process in smokers and lung biopsy shows bronchiolitis

RBILD

Which of the following characteristic(s) is seen on lung biopsy of RBILD: - Fibrosis - Pigmented alveolar macrophages in and around the respiratory bronchioles and surrounding alveoli - Interstitial inflammation - Germinal centers

Pigmented alveolar macrophages in and around the respiratory bronchioles and surrounding alveoli

What is seen on CT for RBILD?

Bronchial wall thickening Fine centrilobular nodules Bilateral patchy GGO in upper and lower lung zones Possible emphysema (these are smokers afterall)

Treatment of RBILD?

Stop that whacko tobacco The roids dont work.

This form of DPLD on histopathology shows pigment-laden macrophages in alveolar spaces in a homogenous pattern with preserved alveolar architecture and minimal fibrosis or honeycoming

DIP The pigment in the macrophages from the smoke

What is seen on HRCT in DIP?

peripheral GGOs in lower lung zones, no honeycombing, possible small lung cysts and emphysematous changes

What is required to establish the diagnosis of DIP?

Surgical lung Bx

Treatment of DIP?

STOP SMOKING Roids are controversial

What connective tissue disease is associated with LIP?

Sjogrens syndrome

What % of patients die within the first 5 years after the diagnosis of LIP?

50%

What is seen on pathology in LIP?

Interstitial and bronchovascular infiltrate of small lymphocytes and plasma cells Poorly formed nonnecrotizing granulomas with multinucleated giant cells are sometimes seen

What is seen on HRCT in LIP?

Bilateral GGOs, centrilobular nodules, patchy bronchovascular thickening, interlobular septal thickening, and thin-walled cysts lol like everything

What's required for diagnosis of LIP?

Surgical lung Bx

Mainstay of treatment for LIP?

Steroids Response is variable

Name 3 smoking-related ILDs

DIP RBILD Langerhans cell histiocytosis

Give me the connective tissue disorder (CTD) associated with the following antibody: dsDNA

SLE

Give me the connective tissue disorder (CTD) associated with the following antibody: SSA

SLE, SS, myositis

Give me the connective tissue disorder (CTD) associated with the following antibody: SSB

Sjogrens syndrome

Give me the connective tissue disorder (CTD) associated with the following antibody: Scl-70 (topoisomerase I)

Systemic sclerosis

Give me the connective tissue disorder (CTD) associated with the following antibody: CCP

Give me the connective tissue disorder (CTD) associated with the following antibody: RNP

MCTD

Give me the connective tissue disorder (CTD) associated with the following antibody: Jo-1, EJ, PL7

Dermatomyositis/polymyositis/antisynthetase syndrome

This type of CTD can cause ILD, obstructive airway disease, lung nodules, or pleural involvement.

Rheumatoid arthritis (RA)

What 5 histological patterns can be seen on biopsy on RA associated ILD?

``` UIP (most common) COP Follicular bronchiolitis LIP DAD ```

As HRCT for RA associated ILD can show GGO, reticulation, bronchiectasis, and/or micronodules, what "patterns" can be seen as well?

UIP NSIP Bronchiolitis COP

Treatment for RA associated ILD?

Steroids Cytotoxic drugs (azathioprine, mycophenolate, cyclosporine, cyclophosphamide) Biologics (TNFa inhibitors) Lung transplant

This form of CTD can present with symptoms such as skin thickening, telangiectasias, digital nail pitting, GERD, esophageal dysmotility, and dysfunction

Systemic sclerosis (scleroderma)

What complication can occur in >15% of patients with scleroderma, which can be isolated or secondary to ILD?

Pulmonary arterial hypertension. Elevated levels of endothelin-1 vasoconstriction, vascular endothelail cell proliferation, smooth muscle hypertrophy, and irreversible vascular remodeling in the lungs

Most common CT finding for scleroderma related ILD?

NSIP

Treatment for scleroderma related ILD?

Steroids (low dose only as high dose can cause renal crisis) Cytotoxic drugs (cyclophophamide most common) Lung transplant

Signs and symptoms of inflammatory myopathies (polymyositits, deermatomyositits, anti-synthetase syndrome)?

myalgias muscle weakness fatigue Gottrons papules Heliptrope rash Mechanics hands

Most common CT findings on inflammatory myopathy associated ILD?

NSIP Can also see UIP, COP, DAD

Inflammatory myopathy associated ILD is usually treated with steroids despite their lack of evidence. What is the most common cytotoxic medication used in this condition?

Azathioprine

What cytotoxic agent is used in inflammatory myopathy associated ILD in severe, life threatening disease?

Cyclophosphamide Also, you can use mycophenolate, cyclosporine, methotrexate, IVIG, and rituxan

This form of CTD can present like cough, dyspnea, wheezing, chest pain, and sicca symptoms

Sjogren syndrome

What 4 patterns can be found on HRCT in Sjogren syndrome associated ILD?

NSIP LIP COP UIP

Treatment for Sjogren associated ILD?

Steroids | Azathioprine

This manifestation of lupus presents with acute onset of fever, cough, dyspnea, and hypoxia that can lead to respiratory failure. After infection is ruled out, it's treated with high doses of steroids.

Acute lupus pneumonitis

This manifestation of SLE is characterized by progressive hemorrhage on BAL fliuid with hemosiderine-laden macrophages.

DAH

Treatment for DAH related to SLE? 3 things

High dose steroids Cyclophosphamide Plasmapharesis

This manifestation of SLE associated ILD presents with dyspnea, pleuritic chest pain, small lung volume, and diaphragmatic elevation. Treated with steroids.

Shrinking lung syndrome

This manifestation of SLE associated ILD is seen in older men with insidious onset of cough and dyspnea. Treated with steroids and steroid-sparing agents (azathioprine, MMF). Cyclophosphamide reserved foir refractory cases

Chronic ILD

This form of CTD occurs in white younger smokers, presents with cough and dyspnea, secondary PTX (10-15%), fevers, weight loss, malaise, anorexia, pulmonary HTN.

Pulmonary Langerhans Cell Histiocytosis AKA histiocytosis X, pulmonary Langerhans granulomatosis, or pulmonary eosinophilic granuloma

Staining for which 2 antigens and protein is seen in the pathology for Langerhans?

CD1a antigen | S-100 protein

What are the names of the intracellular inclusions seen on electron microscopy in Langerhans?

Birbeck granules

CT findings for Langerhans?

Nodules and thin-walled cysts, mainly in the upper lung zones

Other than obviously to stop smoking, which things are used in severe/refractory Langerhans?

Steroids | Lung transplant

This form of ILD affects premenopausal women, characterized by hamartomatous proliferation of atypical smooth muscles along lymphatics in the lung, thorax, abdomen, and pelvis

LAM

What genetic condition is associated with LAM? Hint: multisystem genetic disorder caused by mutation of the TSC1 or TSC2 gene

Tuberous sclerosis

True/False: recurrent pneumothorax is common in LAM

TRUE Can affect 50-80% of patients

LAM cells come in 2 flavors: myofibroblast-like spindle-shaped cells and epithelioid-like polygonal cells. What 2 melanola-associated proteins do both cells express?

HMB-45 | CD63

Which cells are pathognomic of pulmonary Langerhans cell histiocytosis on bronchoalveolar lavage?

BAL showing > 5% CD1a- positive cells is virtually diagnostic of pulmonary Langerhans cell histiocytosis.

HRCT findings for LAM?

Multiple thin-walled cysts with no specific region. NO NODULES ARE SEEN (unlike Langerhans)

Which patients do not need a surgical lung biopsy for the diagnosis of LAM?

If they have characteristic features, as in ANY of the following: Renal angiomyolipoma Chronic chylous ascites with abdominal lymphadenopathy Characteristic histopathologic findings on lymph node biopsy

As estrogen is involved in the pathogenesis for LAM, recommendation is to avoid pregnancy or exogenous estrogen. However, what medication is used in LAM?

Sirolimus (mTOR inhibitor)

TRUE/FALSE: corticosteroids can be used in the treatment for LAM

FALSE

The pathogenesis of this condition is from acute cigarette smoke exposure with other proallergic allergenic exposure leads to the release of inflmmatory cytokines, leading to massice recruitment and activation of eosinophils in lungs

Acute eosinophilic pneumonia (AEP)

HRCT findings for AEP?

Patchy alveolar GGOs, interlobular thickening, and pleural effusions

What % of eosinophils do you need to have on BAL in AEP in the setting of normal peripheral eosinophils?

>20-25%

Treatment for AEP?

Smoking cessation and high doses of corticosteroids improves symptoms in first 24-48 hours Keep on therapy for 2-4 weeks

This ILD is characterized by affecting middle-aged women, cough, dyspnea, fever, night sweats, asthma, elevated IgE, elevated peripheral eosinophilia.

Chronic Eosinophilic Pneumonia (CEP)

CXR findings for CEP?

Bilateral diffuse PERIPHERAL infiltrates

Though BAL will show eosinophilia in CEP, what will TBBx show?

Eosinophilic microabscesses Low-grade vasculitis Interstitial fibrosis

Treatment for CEP?

Steroids with a very slow taper

This condition is a chronic, multisystem disease with unknown etiology that is characterized by noncaseating granulomas

Sarcoidosis.

This mimicker of sarcoidosis is characterized by the following: Patient works with circuit boards/electronics, similar findings on CXR, diagnosis made by lymph transfer test on BAL

Berylliosis

This mimicker of sarcoidosis is characterized by the following: Medication used to treat HCV and other conditions that promotes granuloma formation

Interferon therapy

Responders to what national terrorist event have a high rate of pulmonary sarcoidosis?

World trade center responders

True/False: sarcoidosis is more prevalent in younger (<40 y/o) than older

TRUE

True/False: sarcoidosis affects women more than men

TRUE

True/False: sarcodiosis has the highest prevalence in Sweden, Denmark, and among African Americans

TRUE

True/False: sarcoidosis has no genetic links

FALSE 6% heredity in whites 17% in African americans No specific gene, but histocompatability complex on chromosome 6p most studied

True/False: smoking is protective in sarcoidosis

TRUE

True/False: HLA DQB1*0201 is associated with favorable outcomes in sarcoidosis

TRUE

Describe stage 1, 2, 3, and 4 on imaging for sarcoidosis

1- hilar lymphadeopathy 2- adenopathy with lung disease 3- lung disease only 4- fibrosis and honeycoming

What form of sarcoidosis has the highest rate of spontaneous remission?

Nodular sarcoidosis

What are the 3 components to Lofgren syndrome?

Erythema nodosum Hilar LAD Arthralgias

Outcomes for Lofgren syndrome?

Resolves in 2 years in >90% of patients

Which ethnicities does the following extrathoracic forms of sarcoidosis affect: Chronic uveitis Lupus pernio Erythema nodosum Cardiac and ocular disease

Chronic uveitis - african americans Lupus pernio - puerto ricans Erythema nodosum- europeans Cardiac and ocular disease - japanese

PFT findings in sarcoidosis?

Obstruction or restriction Methacholine often positive FVC and DLCO are most predictive long-term markers of disease

What is the role for ACE levels in sarcoidosis?

Not specific for the diagnosis but can be used for disease monitoring

What is the CD4:CD8 in sarcoidosis? Bonus: sensitivity, specificity, PPV, and NPV

>3.5 Sensitivity 53%, specificity of 94%, PPV 76%, NPV 85%

What is the normal distribution of granulomas in the lung?

Near or within the connective tissue sheath of bronchioles and subpleural or perilobular spaces (lymphangitis distrubution)

Spontaneous remission in sarcoidosis based on the stage of disease?

1 - 55-90% 2 - 40-70% 3 - 10-20% 4 - 0%

When do you use chloroquine/hydroxychloroquine in sarcoidosis?

When there is skin and CNS disease

When to use infliximab in sarcoidosis?

Refractory disease and extrapulmonary manifestations

This condition is when there is lipoproteinaceous material accumulates in the alveolar tissue due to GM-CSF dysregulation

Pulmonary Alveolar Proteinosis (PAP)

What is the mutation that causes congenital PAP?

Mutations on genes for surfactant B, C, or beta-c chair of the receptor for GM-CSF

What exposures can cause secondary PAP?

``` Silica exposure PJP pneumonia Malignancies Autoimmune disease Immune deficiencies Certain drugs ```

Which of the following suggest PAP on lab findings? ``` Elevated LDH Elevated CEA Cytokeratin 19 Mucin KL-6 Surfactant protein-A, B, or D Serum or BAL showing GM-CSF antibodies ```

ALL OF THEM

PFT findings on PAP?

Restrictive pattern | Decrease in DLCO out of proportion to restriction

HRCT findings for PAP?

Patchy GGOs or cosolidation + Thickening of interlobular space = Crazy paving!!!

Although you can expect to find BAL fluid for PAP to be a milky effluent that shows granular, acellular, eosinophilic, proteinaceous material with foamy macrophages, what finding of electron microscopy is confirmatory?

Lamellar bodies (concentrically laminated phospholipid structures)

True/False: PAS+ proteinaceous alveolar deposits in the absence of a cellular infiltrate and normal septa are characteristic of PAP

TRUE

Average duration of effect for whole lung lavage in PAP?

~15 months

What are the benefits for subQ or inhaled GM-CSG in PAP?

Oxygenation benefit | Some have complete response

True/False: patients with PAP are at higher risk of infections, such as mycobacteria, aspergillus, and nocardia

TRUE

Most common condition that causes primary pulmonary amyloidosis?

Multiple myeloma

What 3 areas of the lung are affected in pulmonary amyloidosis? Also, their symptoms per area?

Parenchymal - cough, sputum production, dyspnea, hemoptysis Tracheobronchial - wheezing, dyspnea, cough, hemoptysis Upper airway - macroglossia with OSA

What is seen on biopsy using polarized microscopy in pulmonary amyloidosis?

Apple-green birefringence

What is seen on bronchoscopy in pulmonary amyloidosis?

Possible focal nodules or skiny pale plaques

Other than obviously treating the underlying cause for pulmonary amyloidosis, what immunomodulator can be used to treat pleural effusions in primary systemic amyloidosis?

Bevacizumab

True/False: median survival is 16 months in nodular-only pulmonary amyloidosis

FALSE Nodular only is typically benign. Otherwise, survival is 16 months.

This is an autosomal dominant disease that causes cystic lung disease, fibrofolliculomas, and skin tags

Birt-Hogg-Dube (BHD)

Which tumor suppressor does the BDH gene encode for on chromosome 17?

Folliculin

Is tracheobronchopathia osteochondroplastica associated with amyloid disease?

No, tracheobronchial amyloid is its own disease entity but can present very similarly. Submucosal involvement (posterior wall) is seen only with tracheobronchial amyloid disease.

What tumors are more common in patients with BDH syndrome?

Renal tumors (RCC, oncocytic hybrid tumor, clear cell carcinoma)

Symptoms of BHD?

PTX in 25% of patients, otherwise can cause dyspnea and cough

How do you diagnose BHD?

Open lung biopsy Helps exclude other things that can cause thin-walled cysts like LAM, LIP, LHC, and malignancies

This condition is when there are lipid-laden macrophages seen on BAL in chronic laxative users (mineral oil)

Exogenous lipoid pneumonia Also can be seen when people use old nasal sprays, aspiration, and bronchography

What can cause endogenous lipoid pneumonia?

Proximal airway obstruction with resultant cholesterol-filled macrophages distally Rarely associated with Neimann-Pick disease

Chest CT scan findings on lipoid pneumonia?

Consolidations with or without GGOs, airspace nodules, and occasional crazy paving when associated with PAP Negative Hounsfield units is diagnostic

What special staining can be done to diagnose lipoid pneumonia?

Sudan black and oil red O They should lipid-filled vacuoles

What clinical presentation can the following medications cause (think drug induced lung injury): ACEi, ARBs

cough

What clinical presentation can the following medications cause (think drug induced lung injury): beta blockers, ASA/NSAIDs, cetuximab

Bronchospasm

What clinical presentation can the following medications cause (think drug induced lung injury): Tocolytics, IL-2, ASA, NSAIDS

Noncardiogenic pulmonary edema

What clinical presentation can the following medications cause (think drug induced lung injury): Methotrexate, taxanes, PCN, sulfa drugs

Hypersensitivity pneumonitis

What clinical presentation can the following medications cause (think drug induced lung injury): bleomycin, taxol, amiodarone, nitrofurnatoin

Chronic fibrosis

What clinical presentation can the following medications cause (think drug induced lung injury): Estrogren (and estrogen receptor agonists, especially with factor V Leiden mutation

VTE

What clinical presentation can the following medications cause (think drug induced lung injury): Hydralazine, isonizaid, penacillamine, procainamide, quinidine

Lupus

What clinical presentation can the following medications cause (think drug induced lung injury): imatinib, dasatinib

Acute lung injury, pleural effusion

What clinical presentation can the following medications cause (think drug induced lung injury): Dasatinib, methamphetamine

pulmonary HTN

Name the following drug that is known to cause lung injury based on the following characteristics: Older patients, pathology shows foamy macrophages with fibrosis and lamellated inclusions, imaging shows fibrosis/COP/mass lesions with or without cavitation

Amiodarone

Treatment for amiodarone lung toxicity?

Stop amio Steroids for >6mo

Name the following drug that is known to cause lung injury based on the following characteristics: F>M, acute HP, COP, cellular NSIP, no zonal predominance, used in UTIs

Nitrofurnatoin

Name the following drug that is known to cause lung injury based on the following characteristics: Pneumonitis, fever, +/- rash, pulmonary eosinophilia, COP, NSIP pattern

5-ASA/Sulfasalazine sulfa causes more than 5-ASA

Name the following drug that is known to cause lung injury based on the following characteristics: Acute/subacute/chronic presentation, HP, NSIP, AIP, occasional eosinophilia, rarely associated with NHL

methotrexate

Name the following drug that is known to cause lung injury based on the following characteristics: Used in CML therapy, patho shows pneumocyte dysplasia and typical broncial lining cells, imaging shows NSIP pattern, bronch rarely shows PAPq

Busulfan

Name the following drug that is known to cause lung injury based on the following characteristics: Various times of presentation, HP, DAD, NSIP, and UIP all possible, avoid high O2 delivery to avoid pulmonary toxicity

Bleomycin

Name the following drug that is known to cause lung injury based on the following characteristics: Acute vs subacute presentation, noncariogenic pulmonary edema or AIP, imaging shows ALI and pleural effusions, pussible pulm HTN

Imatinib/dasatinib

Name the following drug that is known to cause lung injury based on the following characteristics: Potentially life-threatening pneumonitis, path shows sarcoidosis/ALI/COP, imaging shows ALI/effusions/COP, needs early discontinuation if suspected

Interferon

Name the following drug that is known to cause lung injury based on the following characteristics: Acute and chronic sequelae, forein body granulomatosis, ALI, imaging shows septic emobli/DAH/bullae/edema

IVDU

Name the following drug that is known to cause lung injury based on the following characteristics: Subacute or chronic, path shows COP or NSIP, imaging shows GGO's/COP/NSIP in a specific region

Radiation therapy

Sandblasting, tunneling, drill operation, digging, glass manufacturing, hard rock mining, oven-brick making, stone cutting, masonry, foundry work, natural gas extraction via fracturing, and sandy and dry soil agriculture pose one to what pneumoconiosis?

Silicosis

What are the timeframes for the chronic, accelerated, or acute silicosis based on presentation after exposure?

Chronic: 10-30yr after exposure Accelerated: <10yr Acute: weeks to 4-5yr

Presentation of simple silocosis?

Mid-upper long zone nodules <1cm

Presentation of massive fibrosis in silcosis?

Coalescence of lung nodules >1cm (with possible eggshell calcification), upward hilar retraction, lower lobe hyperinflation

Presentation of acute silicosis?

Mid-lower zone GGOs with consolidation

What stage of silicosis is seen on pathology given the following results (early, nodule, or acute)? Peribronchovascular paraseptal, and subpleural dust-laden macrophages

Early

What stage of silicosis is seen on pathology given the following results (early, nodule, or acute)? Concentric collagen fibers around a hyaline center

Silicotic nodule

What stage of silicosis is seen on pathology given the following results (early, nodule, or acute)? Alveolar filling with proteinaceous material, PAS+

Acute

Most common presentation for coal workers pneumoconiosis?

Chronic bronchitis

TRUE/FALSE: coal miners pneumoconiosis can present with a simple upper zone nodules or massive fibrosis, similar to silicosis

TRUE

Based on the following description, is this a coal macule or a Caplan nodule? Focal lesions with necrotic center surrounded by lymphocytes and plasma cells

Caplan nodule A coal macule is a focal collection of coal dust in pigment-laden macrophages

Piperfitters, steamfitters, electricians, insulation worders, boilermakers, welders, construction workers and shipbuilders, plastic and rubber manifacturing workers, yarn/thread/fabric millworkers, and those who work with brakelining or cement are subject to which pneumoconiosis?

Asbestosis

Latency period of asbestosis?

10-40 years

HRCT findings for asbestosis?

Subpleural lines, parenchymal and interlobular septal fibrosis, honeycoming, pleural plauqes

Tell me the name of the large asbestos fibers coated with iron from hemosiderin

Ferruginous bodies

Aerospace, aircraft, and alloy production, automotive, ceramics, defence, dental and prosthetics, electronics, nuclear, recyclinf of electronics, and telecommunications are at risk for which pneumoconiosis?

Beryllium

What form of beryllium (acute or chronic) is more rare and is associated with pneumonitis, tracheobronchitis, and nasopharyngitis?

Acute

What are the clinical symptoms of chronic beryllium pneumoconiosis?

~10 yrs from exposure there is dyspnea, cough, fever, night sweats, fatigue, and/or weight loss

HRCT findings for beryllium pneumoconiosis?

parenchymal nodules, septal thickening, GGOs, hilar and mediastinal LAD

Pathology findings for beryllium pneumoconiosis?

Noncaseating granulomas

Which interstitial lung disease resembles acute silicosis histologically and on imaging?

Secondary pulmonary alveolar proteinosis, which causes alveolar filling with proteinaceous material that consists mostly of phospholipids and surfactant, staining with periodic acid–Schiff reagent (silicoproteinosis). Other exposures with similar findings are aluminum, silica, titanium, cement, insulation, sawdust, paint, varnish, chlorine, nitrogen dioxide, and fertilizer.

Types of infections that are increased in silocosis?

Myocobacterial and fungal

True/False: lung cancer is increased in silociosis

TRUE

True/False: connective tissue disease are increased in silociosis

TRUE Scleroderma, RA, and vasculitis

Which autoimmune disease is associated with Caplan syndrome?

Rheumatoid arthritis. Caplan syndrome is a nodular reaction that occurs in individuals exposed to coal dust who also have rheumatoid arthritis or who will have rheumatoid arthritis within the next 5–10 years. The nodules can vary in diameter (0.5–5.0 cm) and are usually multiple, bilateral, and peripherally located. They contain a necrotic center surrounded by lymphocytes and plasma cells and a very small amount of coal dust.

Which pneumoconiosis is associated with melanoptysis?

Coal workers’ pneumoconiosis. Melanoptysis is the expectoration of black sputum containing carbonaceous particles. It occurs when a conglomerate of nodules cavitates and ruptures into the airway. Melanoptysis has also been reported with freebase cocaine smoking, malignant melanoma, and aspergilloma caused by Aspergillus niger, as well as in progressive massive fibrosis caused by silicosis.

This is the accumulation of coal dust in the lungs, often asymptomatic and can be seen in people living in large urban areas where there is significant air pollution

Anthracosis *cough cough* I think I'm getting the black lung, pop

This type of asbestosis fiber are curly stranded, curved, easily degraded by macrophages, and have a low malignancy risk

Serpentine (chrysotile)

This type of asbestos fiber is rod-like, straight, insoluble, cannot be degraded by macrophages, and has a high malignancy risk

Amphibole | crocidolites, amosite, anthophyllite, tremolite-actinolite

TRUE/FALSE: shorter asbestos fibers are more carcinogenic than long ones

FALSE Longer are worse

Pathology of benign asbestos pleural effusions?

``` Exudative Eosinophilic Bloody Can resolve spontaneously Low malignancy risk ```

TRUE/FALSE: pleural plaques with asbestosis by themselves have an increased malignancy risk

FALSE

TRUE/FALSE: there is a multiplactive risk of cancer in those with history of asbestos exposure and those who smoke

TRUE

This presentation in asbestosis shows diffuse visceral pleural thickening, usually unilateral, concomitnt with asbestosis, considered direct extension of parenchymal fibrosis

Pleural fibrosis

This presentation in asbestosis is when you see a pleural-based "mass-like" lesion with hilar structure "comet-tail" sign, carries no evidence of malinancy risk

Visceroparietal reaction (rounded atelectasis)

Timeline for the development of bronchogenic carcinoma after asbestos exposure?

10-40yr

CT findings for mesothelioma?

Unilateral parietal pleural mass, nodularity, or thickening with pleural effusion, volume loss right>left side

This disease results from exposure to hydrated magnesium silicate (working in pharmaceuticals or inhaling baby powder if inhaling illicit drug tablets), CT shows fibrosis/nodules/lower lobe emphysema, path shows multinucleated giant cells

Talcosis

Most common metal that can cause hard metal lung disease?

Cobalt Can cause COPD, AIP, or fibrosis

What blood or bronchoalveolar lavage test is used to assist in diagnosing chronic beryllium disease?

Beryllium lymphocyte proliferation test

Type of hypersensitivity reaction(s) for hypersensitivity pneumonitis (HP)?

Combined type III and type IV

Name the disease of HP associated with the following antigen and exposure: Antigen - Faenia rectivirgula (bacteria) Exposure - mold hay, grain, silage

Farmers lung

Name the disease of HP associated with the following antigen and exposure: Antigen - Thermoactinomyces vulgaris (bacteria) Exposure - contaminated forced-air systems, water reservoirs

Humidifier lung, air conditioner lung, ventilation pneumonitis

Name the disease of HP associated with the following antigen and exposure: Antigen - T. vulgaris (bacteria) Exposure - moldy sugar cane

Bagassosis

Name the disease of HP associated with the following antigen and exposure: Antigen - Aspergillus clavatus (fungi) Exposure - moldy barley

Malt workers lung

Name the disease of HP associated with the following antigen and exposure: Antigen - Cryptostroma corticale (bacteria) Exposure - Moldy maple bark

Maple bark strippers lung

Name the disease of HP associated with the following antigen and exposure: Antigen - Penicillum casei (bacteria) Exposure - Moldy cheese

Cheese washers lung Seriously i cant make this stuff up. What am i even writing right now. More to follow.

Name the disease of HP associated with the following antigen and exposure: Antigen - Thermoactinomyces viridus (bacteria) Exposure - Moldy cork

Suberosis

Name the disease of HP associated with the following antigen and exposure: Antigen - Mucor stolonifer (fungi) Exposure - Moldy paprika pods

Paprika slicers lung ..see!

Name the disease of HP associated with the following antigen and exposure: Antigen - Avian droppings, feathers, serum Exposure - Parakeets, pigeons, chickens, turkeys

Pigeon breeders or fanciers lung

Does is this more consistent with acute, subacute, or chronic HP? Pathology shows poorly formed noncaseating granulomas, peribronchiall mononuclear cell infiltration

Acute

Does is this more consistent with acute, subacute, or chronic HP? HRCT shows nodules, GGO's, and honeycombing

Chronic

Does is this more consistent with acute, subacute, or chronic HP? Pathology shows loose and poorly formed arranged noncaseating granulomas with mononuclear alverolitis in the presence of fibrosis with thick alveolar septa

Chronic

Does is this more consistent with acute, subacute, or chronic HP? Pathology shows more well formed, but loose and poorly arranged noncaseating granulomas, bronchioloitis with or without organizing pneumonia

Subacute

What is seen on BAL in HP?

Elevated lymphocytes (>50%) Inversion of CD4:CD8 (proliferation of CD8 cells)

True/False: Although inhalation of gases/antigens/fumes result in hyperacute cytokine release without cosequent lung injury, with or without lung alveolar damage, or subacute parenchymal disease, exposure does NOT result in HP.

TRUE

Exposure to welding and brass work can lead to the inhalation of what toxic agent?

Metal fume fever (Zn oxide)

Exposure to contaminated grains, moldy hay, silage, flour, textile materials, and wood chips can lead to the inhalation of what toxic agents?

Organic dust toxic syndrome, thermophilic bacteria, and fungal spores

True/False: inhalation of metals or organic dust can lead to flu-like symptoms that take 2-3 weeks to resolve.

FALSE Typically resolves in 12-48 hours

True/False: inhalation of metals or organic dust typically leads to no radiographic changes

TRUE

The following inorganic low water solubility agents can cause what effects on the lung if inhaled? Chlorine, chloramine (from cleaning products or swimming pools) Nitrogen dioxide (silo fillers disease and explosives), ozone (welding), aerosols, phosgene (welding) Metals (cadmium, mercury, nickel carbonyl) from welding, brazing, flame cutting, electronic recycling

Acute irritant lung injury with DAD

Symptoms of acute irritant lung injury with DAD from inorganic low solubility agents?

HEENT symptoms, acute dyspnea, bronchiolitis, may develop 2-6 wks later

Radiographic changes from acute irritant lung injury with DAD from inorganic low solubility agents?

Airspace opacities, noncardiogenic edema, GGOs (with metals)

The following organic low water solubility agents can cause what effects on the lung if inhaled? Fire eaters lung (flame blowing performances) Paraquat (ingestion) Popcorn workers lung (diacetyl butanedione from flavor industry workers)

Acute irritant lung injury with DAD

Symptoms of acute irritant lung injury with DAD from organic low solubility agents?

Acute dyspnea, respiratory failure, bronchiolitis 2-6 weeks later, GI symptoms (from paraquat)

Radiographic changes from acute irritant lung injury with DAD from organic low solubility agents?

Airspace opacities, noncardiogenic edema

Smoke inhalation (with high water solubility) can cause what effect on the lungs?

Acute irritant lung injury and DAD

Exposure to nylon (flock workers disease from textile manufacturing) or ardystil (from textile printing sprayers) can cause what effects on the lung?

Lung parenchymal disease

Symptoms from exposure to nylon or ardystil?

Dyspnea progressing to ILD over months to years (usually NSIP or COP)

What properties of toxic gases and fumes determine their pattern of lung injury?

Water solubility, duration of exposure, and depth of inhalation. For example, more water-soluble gases such as chlorine cause more upper-airway irritation and symptoms than less water-soluble agents such as nitrogen dioxide, which cause more small airways disease and alveolar damage.

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Absent latency period

Irritant

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? onset within months to years

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Usually from fumes, gases, smoke, vapors

Irritant

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? From complete antigens (MW > 10kd) or incomplete antigens (haptens, chemicals with MW <10kd)

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? From animal and plant proteins, enzymes, flour, cereal, isocyanates, anhidrates, metals, drugs, dyes and bleaches, amines, glues and resins, and wood dust

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? From chlorine gas, aldehydes, alkaline dusts, bleaching agents, cleaning products (ammonia), accidental spills, smoke from fires

Irritant

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Immunologic response

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Diagnosis is made by confirming asthma diagnosis (PFTs followed by methacholine)

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Diagnosis made by asthma diagnosis, then by establishing occupational relationship, and then possibly by specific inhalation challenge

Irritant

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Pathology shows either epithelial sloughing and hemorrhage (acute) or epithelial cell regeneration with increased basement membrane thickness due to collagen deposition (chronic)

Irritant

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Pathology is similar to asthma

Sensitizer

Based on the following characteristics, is this more consistent with sensitizer-induced asthma or irritant-induced asthma? Symptoms present while at work, improvemet on weekends or vacations

Sensitizer

Tell me what the following organ does to acclimate to high altitude: Ventilation

Hyperventilation, respiratory alkalosis

Tell me what the following organ does to acclimate to high altitude: Brain

Hypoxia induced vasodilation Hypocapnia induced vasoconstriction Constant cerebral blood flow

Tell me what the following organ does to acclimate to high altitude: Kidneys

Increased bicarb excretion, EPO production

Tell me what the following organ does to acclimate to high altitude: Heart

Increase blood pressure Increased CO Decreased SV (bicarb diuresis and fluid shifts)

Tell me what the following organ does to acclimate to high altitude: Pulmonary vasculature

Hypoxia-induced vasoconstriction

Tell me what the following organ does to acclimate to high altitude: Hematologic

Increased Hgb synthesis

Tell me what the following organ does to acclimate to high altitude: Oxyhemoglobin curve

Left shift due to alkalosis

Prevention medications for altitude motion sickness?

Acetazolamide, dexamethasone

Treatment for mild and moderate altitude motion sickness?

Mild - conservative (meds for symptoms) Moderate/severe - descent, oxygen, hyperbaric therapy, acetazolamide, dexamethasone

Mechanism for high-altitude pulmonary edema?

Hypoxic vascoconstriction leads to capillary stress fractures, resulting in pulmonary edema

Treatment for high-altitude pulmonary edema?

Oxygen, descent, hyperbaric therapy, nifedipine, tadalafil, or sildenafil

Medications used to prevent high-altitude pulmonary edema?

Nifedipine, tadalafil, sildenafil, dexamethasone, salmeterol

This condition at altitude can cause headaches, ataxia, confusion, hallucinations, stupor, and coma.

High-altitude cerebral edema

Medications for the treatment of High-altitude cerebral edema?

Acetazolamide, dexamethasone

Which transcription factor is responsible for most of the adaptive changes that occur during acclimatization?

Hypoxia-inducible factor-1α stimulates vascular endothelial growth factor, which itself stimulates angiogenesis and nitric oxide synthesis. These changes result in greater blood flow and oxygen delivery to tissues.

Supplemental oxygen use amount that is a contraindication to air travel?

4lpm

Altitude of air cabin pressure on a plane?

8000 ft

How does a high-altitude stimulation test work?

A 15.1% oxygen concentration with nitrogen mixture is given via a tight-fitting mask, SpO2 is measured ABG taken after 20 minutes If oxygen remains 88% or greater and PaO2 is >55mmHg, then no supplemental oxygen is required.

How many days do you have to avoid air travel after radiographic resolution of pneumothorax?

7 days

How long should you postpone air travel if you dove once per day? Extra: how long to postpose if you dove multiple time or have had to have decompression stops?

Once/day - 12 hours Multiple times - 48 hours

This is when, on ascending from deep water, tissues become loaded with increased quantities of oxygen and nitrogen leading to liberation of these gasses causes vascular obstruction, vessel rupture, or tissue compression --> inflammation and clotting

Decompression sickness (the bends)

This is when there is increased partial pressure of nitrogen in the nervous system tissue that can result in confusion (100ft) or the loss of consciousness (>300 ft)

Nitrogen narcosis

In diving medicine, which gas law explains the pathophysiology of most types of barotrauma?

Boyle’s law. At a constant temperature, the volume of a gas is inversely proportional with the pressure to which it is subjected.

Which gas law explains the pathophysiology behind decompression sickness and nitrogen narcosis?

Henry’s law. At a constant temperature, the amount of a gas dissolved in a liquid is directly proportional to the partial pressure of that gas.

What 3 conditions can be a complication of barotrauma in diving?

Pneumomediastinum Pneumothorax Arterial gas embolism

How to avoid barotrauma (and resultant clinical conditions) during diving?

Avoid breath holding during ascent

True/False: barotrauma to the middle ear in diving and eustachian tubes can cause tympanic membrane rupture

TRUE

Symptoms of type 1 decompression sickness?

MSK pain, skin pruritis/erythema, lymphatic problems (LAD, peau d'orange)

Symptoms of type 2 decompression sickness?

Neurologic - parasthesias, weakness, parlysis, memory loss, ataxia Pulmonary - chest pain, wheezing, dyspnea, pharyngeal irritation, acute RHF

Treatment of decompression syndrome?

Hydration 100% oxygen L lateral decubitus position Hyperbaric oxygen

Prevention of decompression sickness?

Hydrate Avoid rapid descent Avoid alcoholic beverages

This therapy increases oxygen delivery by increasing its dissolution in plasma (Henry's law), reduces volume of gas bubbles (Boyle's law), replaces nitrogen with oxygen, promotes vasoconstriction, ameliorates ischemia-reperfusion inflmmation, facilitates angiogenesis and fibroblast proliferation, and increases neutrophil bactericidal activity

Hyperbaric oxygen therapy

What is hyperbaric oxygen therapy used to treat?

``` CO and CN poisoning Decompression sickness and air embolism Acute traumatic or thermal injury Radiation injury Nonhealing ulcers, skin grafts, and wounds Clostridial myositis or myonecrosis ```

Case: patient presents to the ER with malaise, dzziness, and lactic acidosis. EKG shows NSTEMI. Carboxyhemoglobin level 18%. Cause?

CO poisoning

Indications for hyperbaric oxygen therapy in CO poisoning?

``` Unconscious Mental status changes Neurologic deficit End-organ ischemia CO-Hb >25% (20% in pregnancy) ```

This is when there is the conversion of the ferrous (Fe2+) to ferric (Fe3+), for which oxygen cannot bind.

Methemoglobinemia

Medications that can increase formation of methemoglobinemia?

``` Dapsone Topical benzocaine or lidocaine Inhaled NO Nitroglycerin Nitroprusside Chloroquine Metoclopramide Sulfonamides Aniline derivatives ```

What is the saturation gap in methemoglobinemia?

Difference between pulse oximetry and measured oxygen saturation in blood gas

Treatment for methemoglobinemia?

Methylene blue if methemoglobinemia level >30%

Symptoms of thermal lung injury from smoke inhalation?

Cough, stridor, wheezing, hoarseness, carbonaceous sputum, blistering, and oropharyngeal edema

Exposure to this substance can cause headache, anxiety, confusion, vertigo, coma, seizures, tachycardia, HTN, arrhythmias, and "cherry-red" lip color due to inhibition of OXPHOS by bidning to cytochrome oxidase a3

CN poisoning

Drug most commonly assoicated with CN poisoning?

Nitroprusside

Treatment for CN poisoning?

Hydroxycobalamin Induce methemoglobinemia (amyl nitrate, sodium nitrite0 Sodium thiosulfate

Bleomycin, amiodarone, nitrofurnatoin, mitomycin, and paraquat can cause the formation of what?

Free radicals

Treatment for radiation-induced pneumonitis?

Corticosteroids