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Clin Med II Unit II > 5 ILD > Flashcards

5 ILD Flashcards

(62 cards)

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1
Q

WHat is the definition of interstitial lung disease

A

A large group of pulmonary disorders, most of which cause progressive scarring of lung tissue surrounding the alveoli

Causes:
Idiopathic
Autoimmune
Exposure to hazardous material
Drugs

Generally an irreversible process and cumbersome to treat

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2
Q

Drugs that can cause interstitial lung disease

A 
Amiodarone
Propranolol
Nitrofurantoin
Methotrexate
Rituximab
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3
Q

Pathophysiology of ILD

A

A process of fibrosis and inflammation - not infectious

Alveolar epithelium damage —> Type II alveolar epithelial cells proliferate to repair damage —> Repair process leads to fibrosis and scarring —> Lung stiffens, ability to transport O2 diminishes —> Hypoxemia or hypercapnia

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4
Q

SSx of ILD

A

Progressive dyspnea on exertion
Persistent non-productive cough
WHeezing and chest pain are uncommon

Extrapulmonary symptoms: May be suggestive of a connective tissue disease; MSK pain, weakness, joint pain or swelling, fevers, dry eyes/mouth (related to underlying conditions)

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5
Q

ILDs that can be acute

A

Acute idiopathic interstitial PNA
Eosinophilic pneumonitis
Hypersensitivity pneumonitis
Cryptogenic organizing pneumonitis

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6
Q

ILDs that can be subacute (weeks to months)

A

Sarcoidosis
Alveolar hemorrhage
Cryptogenic organizing pneumonia
Connective tissue disease (SLE, polymyositis)

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7
Q

ILDs that can be chronic (months to years)

A

IPF
Sarcoidosis
Pulmonary langerhans cell histocytosis
Chronic hypersensitivity pneumonitis

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8
Q

Physical exam findings in ILD

A

CRACKLES (usually at the bases; Velcro sounding)

Inspiratory squeaks (high pitched rhonchi)

Cor pulmonale - present in middle or late stages

Cyanosis - uncommon, indicates advanced disease

Digital clubbing - advanced disease

Extrapulmonary manifestations with different diseases (erythema nodusum in sarcoidosis, cottron’s papules in dermatomyositis)

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9
Q

Gottron’s papules are also known as …

A

“Mechanics Hands”

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10
Q

Gold standard for diagnosing ILD

A

Tissue biopsy

Multiple diagnostics available but HRCT offers highest yield as it is non-invasive

Dx may take a long period to make confirmatory Dx

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11
Q

CXR findings suggestive of ILD

A

Reticular “netlike” (most common), modular, or mixed (reticulonodular) opacities —> Honeycombing (small cystic spaces)

Ground glass appearance often an early finding (nonspecific)

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12
Q

CXR finding that indicates a poor prognosis for ILD

A

Honeycombing

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13
Q

Test with a greater diagnostic accuracy than CXR for ILD

A

High Resolution Computed Tomography (HRCT)

Distribution of disease within the lung and pattern of disease both apparent

Comparison to old CT - interval progression of disease

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14
Q

What sorts of serologic studies do we do in ILD?

A

ANA and Rheumatoid Factor to rule out subclinical autoimmune disease, possible Anti-SS-DNA for SLE

If pulmonary hemorrhage or suspicious systemic symptoms, evaluate for vasculitis (Antineutrophil cytoplasmic antibodies (ANCA)

Sed rate and CRP are markers of inflammation but they are nonspecific and generally not helpful in ILD

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15
Q

Most ILDs are associated with a ___________ pattern on PFTs

A

Restrictive

Decreased TLC, decreased flow rates (FEV1 and FVC) but changes proportional to dismissed lung volumes to FEV1/FVC ratio is normal or increased

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16
Q

What do you do next if PFTs show restrictive or normal pattern?

A

Obtain DLCO

If DLCO low, consistent with ILD (may be only finding in early disease)

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17
Q

What do arterial blood gases look like in ILD?

A

Resting blood gas may be normal, or may show hypoxemia (low PaO2) or respiratory alkalosis (low PaCO2)

Despite normal resting blood gas, severe exercise or sleep induced hypoxemia may be present

May need to perform exercise testing with serial ABGs

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18
Q

What is bronchoalveolar lavage and when do we perform it?

A

Minor extension of bronchoscopy, allows sampling from distal airways and alveoli

Cell counts: WBC differential (can help narrow Dx)

Cultures: ID typical/atypical infection

Cytology: ID malignancy

Evaluate source of hemoptysis

NOT typically performed with HRCT findings c/w IPF

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19
Q

Gold standard for Dx of ILD

A

Lung Biopsy

Indications:
Specifying Dx
Atypical or rapidly progressing HRCT findings or progressive disease
Unexplained extrapulmonary manifestations
Excluding neoplasm or infection that can mimic ILD
ID a more treatable process than originally suspected
Predict likelihood of response to therapy

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20
Q

When is lung biopsy CONTRAINDICATED?

A

Honeycombing on imaging, as prognosis is poor and biopsy won’t change management at this point

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21
Q

Different types of lung biopsy

A

Transbronchial biopsy (via bronchoscopy) - helpful for central locations

Surgical biopsy - done under general anesthesia with one lung ventilation, allowing for larger sample, especially on periphery

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA)

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22
Q

Types of surgical biopsy

A

Video assisted thoracoscopic surgery (VATS) - two small incisions to the lateral chest wall, associated with less morbidity

Thoracotomy - 5-6 cm incision required

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23
Q

When is endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) indicated?

A

Special bronchoscope used to evaluate hilar and mediastinal lymph nodes

Done under general anesthesia

Can be done in conjunction with transbronchial lung biopsy but need to pass different scope

Especially useful if SARCOIDOSIS suspected

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24
Q

Most common interstitial lung disease

A

Idiopathic Pulmonary FIbrosis

Age >50 (generally between 50-85)

Males > Females (5:1)

Histopathology referred to as “Usual Interstitial Pneumonia”

Genetic predisposition

Poor prognosis (2-5 year survival from time of diagnosis)

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25
Clinical features of IDF
Chronic, progressive, Fibrosis interstitial pneumonia of unknown cause Gradual onset of exertional dyspnea and non-productive cough (>6 months) is a common presentation Velcro crackles (inspiratory) Digital clubbing in 25-50%
26
Dx of IPF
6 minute walk test offers good baseline (non-invasive) CXR: peripheral reticular opacities (predominately in bases) —> Honeycombing HRCT: more definitive - bibasilar reticulonodular opacities PFTs: Restrictive pattern Lung biopsy, bronchoscopy/BAL NOT required Echo: if cor pulmonale
27
Diagnostic criteria for IPF
``` Need HRCT to determine if: • UIP pattern • Probable UIP pattern • Indeterminate UIP pattern • Alternative diagnosis ``` Surgical lung biopsy + BAL if: • Probably UIP pattern • Indeterminate UIP pattern • Alternative diagnosis
28
Treatment recommendations for IPF
Treat for GERD even if no symptoms Nintedanib Pirfenidone (reduces lung fibrosis) Quit smoking, stay UTD on vaccines Supplemental O2 if needed
29
“Ivan IPF”
Male 60ish Common man Smoker
30
Multisystem inflammatory disease mainly affecting lungs and intrathoracic lymph nodes, with a more central predominance
Sarcoidosis Non-caseating granulomas that secrete ACE (angiotensin converting enzyme) and includes elevated Ca levels More common in African Americans More common in women Usually younger patient (age 20-40)
31
Patient Hx in sarcoidosis
DOE, chest pain, cough, hemoptysis (rare) Systemic complaints (fever, anorexia) in about 45%
32
Extrapulmonary findings in sarcoidosis
Erythema nodosum Lupus pernio Granulomatous uveitis Arthralgias
33
Diagnosing sarcoidosis
Usually restrictive pattern PFTs, 15-20% with obstructive pattern, isolated reduced DLCO may be only finding CXR: adenopathy Labs: Elevated serum ACE, elevated serum Ca, hypercalciuria, elevated alkaline phosphatase Biopsy usually required (EBUS-TBLB)
34
Staging of sarcoidosis based on CXR
Stage 0: normal Stage 1: hilar adenopathy Stage 2: hilar adenopathy + diffuse infiltrates Stage 3: only diffuse parenchymal infiltrates Stage 4: pulmonary fibrosis
35
Treatment of Sarcoidosis
Most won’t require treatment unless stage 2 and symptomatic High dose corticosteroids PCP prophylaxis (Bactrim) Methotrexate if you want a non-steroid alternative Chloroquine for cutaneous lesions Topical corticosteroids for ocular disease Consider lung transplant in stage 4 patients
36
Typical CXR findings for sarcoidosis
Bilateral symmetric hilar and right paratracheal mediastinal adenopathy
37
“Sally Sarcoidosis”
``` Female African American 30’s No smoker Hilar adenopathy She’s an ACE ```
38
General term for lung disease caused by inhalation and deposition of mineral dust
Pneumoconiosis Examples: Asbestosis Silicosis Coal Worker’s Pneumoconiosis
39
Pneumoconiosis characterized by fibronodular lung disease due to inhalation of silica dust
Silicosis Occupations at risk: mining, construction, granite cutting, pottery making
40
CXR findings for silicosis
Enlarging opacities even after exposure eliminated, can cavitate (so rule out TB) EGGSHELL CALCIFICATIONS
41
PFTs in silicosis have a predominantly ________ pattern
Restrictive Massive fibrosis may lead to severe restriction
42
What is the difference between chronic simple and chronic complicated silicosis?
Simple: 10-12 years of exposure May be asymptomatic Non-progressive once exposure eliminated Chronic: >20 years exposure Progressive even after exposure eliminated PE: tachypnea, prolonged expiration, rhonchi, wheezing, rales Digital clubbing uncommon Cyanosis = advanced disease Cor pulmonale = advanced disease
43
Treatment for silicosis
Smoking cessation No specific therapy will alter disease course but corticosteroids may benefit in ACUTE phase only Eliminate exposure Vaccinate Pt has elevated TB risk, test and treat any latent TB Evaluate for lung transplant candidacy
44
“Sam Silicosis”
``` Male Miner Indeterminate age Smoker - making things worse Eggshell calcifications ```
45
Asbestosis is due to ...
Chronic inhalation of asbestos fibers, usually presenting after 15-20 years of exposure - dose dependent Predominant age at diagnosis = 40-75 y/o Males>Females due to work history Occupation history is important Smoking greatly increases risk of bronchogenic CA in Asbestosis patients
46
PE for asbestosis
No specific SSx Insidious onset - dyspnea, reduced exercise tolerance, non-specific chest discomfort Dry cough End-inspiratory rales Digital clubbing - doesn’t correlate with severity
47
Dx studies for asbestosis
CXR - opacities in LOWER lungs, thickened pleura, PLEURAL PLAQUES Open-lung biopsy usually not indicated but will provide definitive Dx (histology shows fibrosis and asbestos bodies through light microscopy) PFTs - restrictive pattern (used to follow disease pattern, progression)
48
PLEURAL PLAQUES and
Asbestosis
49
Management of asbestosis
May need long-term O2 Smoking cessation Eliminate exposure No immunotherapy drugs or steroids Goal: eliminate progression of Sx, reduce risk of other lung disorders Vaccinate Promptly treat resp infections and evaluate lung lesions Prevention: avoid exposure, protective mask
50
Individuals who smoke and have asbestosis exposure have greatly increased risk to develop ...
Bronchogenic carcinoma
51
Form of CA almost always associated with asbestos exposure
Mesothelioma Poor prognosis - median survival 6-12 months after presentation
52
“Al Asbestosis”
``` Male 50ish Pipe fitter Smoker - making things worse Pleural plaques ```
53
Multisystem autoimmune disease of unknown etiology characterized by necrotizing granulomas of upper and lower respiratory tracts
Granulomatosis with Polyangitis (GPA) It’s an immune-mediated systemic vasculitis of small-medium vessels (ANCA-associated vasculitis)
54
PE exam/organ manifestations of GPA
Ocular involvement: conjunctivitis, episcleritis, uveitis ENT: chronic sinusitis, rhinitis, epistaxis, SADDLE NOSE Pulmonary: infiltrates (71%), cough (34%), hemoptysis, dyspnea, stridor MSK: polyarticular arthralgias Renal: renal failure, erythrocytes casts Nervous: cranial nerve palsies, sensorimotor polyneuropathy Skin: palpable purpura, skin ulcers Cardiac: pericarditis, coronary vasculitis
55
Dx of GPA
CXR: highly variable, nodules which may cavitate CT: stellate shaped peripheral pulmonary arteries, feeding vessels leading to nodules, cavities and diffuse alveolar hemorrhage Bronchoscopy: usually not indicated unless hemorrhage Tissue biopsy: Histo pathologic evidence of vasculitis, granulomatous inflammation Labs: ESR/CRP elevated, CBC/CMP - look for normocytic anemia, thrombocytosis, leukocytosis, renal involvement, UA - proteinuria, C-ANCA Consider running RF/ANA
56
Management of GPA
Consult rheumatologist, pulmonologist Initial treatment with cyclophosphamide (immunosuppressant) and corticosteroids Prognosis improved with cyclophosphamide but look out for toxicities (CHF, thrombocytopenia, acute hemorrhagic cystitis, acute tubular necrosis)
57
The only reversible ILD
Hypersensitivity Pneumonitis - inflammatory syndrome of the lung caused by repetitive inhalation of antigens in a susceptible host Can be either acute, subacute, or chronic
58
Hx for hypersensitivity pneumonitis
Flu-like syndrome developing within hours of exposure if acute Insidious onset productive cough, dyspnea, fatigue over weeks if subacute Progresssive dyspnea, cough, fatigue, malaise in chronic process
59
PE findings for hypersensitivity pneumonitis
Diffuse, fine, bibasilar crackles, fevers, tachypnea, muscle wasting, clubbing, weight loss
60
Imaging findings for hypersensitivity pneumonitis
Interstitial inflammation, honeycombing, centrilobullar fibrosis, peribronchiolar fibrosis
61
Management of hypersensitivity pneumonitis
Antigen avoidance/proper prevention techniques Acute disease remits without therapy Corticosteroids may speed recovery in severe cases but more often used for chronic process Bronchodilators, antihistamines, inhaled corticosteroids are adjunctive therapies to be considered
62
Main complications of ILD
COR PULMONALE - hypertrophy or dilation of the right ventricle associated with lung disease (JVD, hepatomegaly, pedal edema, cyanosis) Pulmonary HTN Pneumothorax PE Pulmonary infection Elevated cancer risk Progressive respiratory insufficiency

Clin Med II Unit II (7 decks)

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