Restrictive lung disease + neoplasms Flashcards
What is the overarching umbrella of restrictive disease in lungs that is exposure, autoimmune, and idiopathic related w/ insidious onset of progressive dyspnea + nonproductive cough, tachypnea, small lung volumes, bibasilar dry rales, digital clubbing, RHF w/ advanced disease?
interstitial lung disease
What does clubbing in the hands indicate?
years of hypoxia
What would you expect in a restrictive lung disease’s PFT?
REDUCED lung volumes, low DLCO, high FEV1/FVC ratio
What would you see on a CXR of interstitial lung disease?
low lung volumes + patchy distribution of ground glass, reticular, nodular, reticulonodular, or cystic opacities
What’s the standard for diagnosis of diffuse lung disease?
surgical biopsy w/ 2-3 biopsies from multiple sites, usually in patients <60
and can consider bronchoalveolar lavage in cases of infection or malignancy
What should you consider diagnosis in patients >65 w/:
- Idiopathic disease by history and who have inspiratory crackles
- PFT = restrictive
- Characteristic radiographic evidence of progressive fibrosis over several years
- AND diffuse patchy fibrosis w/ pleural-based honeycombing on high resolution CT scan
with symptoms of: DOE, cough, fatigue (gradual onset)?
idiopathic pulmonary fibrosis
In who is idiopathic pulmonary fibrosis common?
men>50, smoking
T/F: idiopathic pulm fibrosis patients need a lung biopsy to confirm diagnosis
F
What would these diagnostic studies indicate?
CT = predominantly basilar, peripheral (bronchiectasis = upper) pattern of dilated bronchiectasis, reticulation, and early honeycombing
PFT: restrictive pattern w/ increased FEV1/FVC + decreased lung volumes
idiopathic pulmonary fibrosis
How do you manage idiopathic pulmonary fibrosis?
pulmonologist
Nintedanib + pirfenidone (reduce lung function decline but do not improve survival, $$)
Oxygen, pulmonary rehab, monitoring pulm HTN
Only definitive treatment = lung transplant
What causes sarcoidosis?
systemic granulomatous inflammation forming in multiple organs (we don’t know why) – lungs, lymph nodes, skin, eyes, nerves, liver, kidney, heart
What could a patient with sarcoidosis come in complaining about?
malaise, night sweats, fever, dyspnea, cough, CP
What is stage 1 of sarcoidosis?
bilateral hilar adenopathy alone
What is stage 2 of sarcoidosis?
hilar adenopathy AND parenchymal involvement (diffuse reticular infiltrates, focal infiltrates, bronchiole changes, nodules, pleural effusion)
What is stage 3 of sarcoidosis?
parenchymal involement alone
What is stage 4 of sarcoidosis?
advanced fibrotic changes in upper lobes
In who is sarcoidosis prominent?
30s or 40s onset, african americans
What could this PE indicate:
- atypical
-crackles uncommon but skin involved w/ erythema nodosum, lupus pernio, iritis, peripheral neuropathy, arthritis, cardiomyopathy
- parotid gland enlargement, hepatosplenomegaly, lymphadenopathy
sarcoidosis
How can you confirm diagnosis of sarcoidosis?
clinical findings + consistent XR (bilateral hilar + right paratracheal lymphadenopathy) and biopsy evidence of noncaseating granuloma from safest organ
BAL fluid = increase in lymphocytes + high CD4/CD8 ratio - monitoring and ophthalmologic evaluation
What could this indicate:
Lymphopenia, elevated ESR, hypercalcemia, hypercalciuria, elevated LFTs, elevated ACE
PFTS = restrictive changes w/ decreased lung volumes + diffusing capacity (may show airflow obstruction)
ECG = conduction disturbances & dysrhythmias
sarcoidosis
What are these indications for:
Disabling constitutional symptoms
Hypercalcemia
Iritis
Uveitis
Arthritis
CNS involvement
Cardiac involvement
Granulomatous hepatitis
Cutaneous lesions other than erythema nodosum
Progressive pulmonary lesions
sarcoidosis = oral steroids - prednisone w/ taper
If someone who has sarcoidosis is intolerant of steroids what do you give instead?
methotrexate, azathioprine, infliximab
What does long-term follow up look like for sarcoidosis?
yearly physical exam and PFTs, chemistry panel, ophthalmologic evaluation, CXR, ECG
What should you think if a patient presents asymptomatic w/ slightly affected pulmonary function or progressive, massive fibrosis w/ conglomeration + contraction in upper lungs and is characteristic of Caplan syndrome and could be from the inhalation of inorganic dusts, smoking does not?
coal worker’s pneumoconiosis
What would you expect on CXR and PFT for coal worker’s pneumoconiosis?
CXR: formation of coal macules as diffuse small opacities in upper lungs w/ hyperinflation of lower lobes in an obstructive pattern (resembling emphysema)
PFT: obstructive pattern
What is treatment for coal worker’s pneumonocosis, silicosis, asbestosis?
supportive
What is usually caused by inhalation of silica particles that could be asymptomatic (simple) or large conglomerate densities in upper lung w/ dyspnea, restrictive AND obstructive pulmonary dysfunction and should be suspected in construction, mining, sand blasting, or quarry work?
silicosis
What does this CXR indicate:
scattered, small rounded opacities (silicotic nodules) + calcification of periphery of hilar lymph nodes “egg shells”
silicosis
With silicosis patients, what should you consider with testing?
all patients should have a tuberculin skin test + current chest XR
What can be caused by exposure to asbestos fibers over many years that can worsen with smoking and cause progressive dyspnea, inspiratory crackles, clubbing and cyanosis, and is common in shipyard and construction workers, pipe fitters, insulators of older buildings, ~15 years post exposure?
asbestosis
What do these diagnostics indicate:
CXR: nodular + linear streaking at lung bases, opacities of various shapes + sizes, advanced = honeycomb changes, pleural calcifications
Shaggy heart sign = indistinct heart border, “ground glass” appearance
CT scan is best imaging study to detect fibrosis + pleural plaques
PFTs = restrictive dysfunction + reduced diffusing capacity (increased FEV1/FVC + decreased lung volumes)?
asbestosis
In who is hypersensitivity pneumonitis most common?
30s-50s
What is this indicative of:
Acute - fever, chills, malaise, 4-8 hours post prolonged exposure, inspiratory crackles
Subacute - gradual development of dyspnea, productive cough, usually NO fever/chills
Chronic - progressive worsening and is inflammation from organic antigens?
hypersensitivity pneumonitis
How do you treat hypersensitivity pneumonitis?
Can be reversal in case of prompt diagnosis + remove offending agent
Oral steroids
What does this characterize:
Profound dyspnea, cyanosis, tachypnea, intercostal retractions, crackles that can be from various diseased states and is #1 caused from sepsis?
ARDS
ARDS levels
Mild: paO2/FlO2 ratio = 200-300
Moderate: paO2/FlO2 ratio = 100-200
Severe: paO2/FlO2 ratio = <100
What is required for an ARDS diagnosis?
~ 7 days of trigger (but could be up to 2 hours after trigger)
Bilateral pulmonary opacities (within alveoli)
NON cardiogenic
Impaired gas exchange
3 inclusion, 1 exclusion criteria
What would you see on CXR for ARDS?
CXR: diffuse patchy infiltrates that proceed rapidly and become confluent
Air bronchograms, normal heart size
How do you treat ARDS?
Mechanical ventilation w/ PEEP (lowest levels + O2 required)
Prone positioning
Central venous catheter
Fluid management
Severe - ECMO
What could this indicate:
Anorexia, weight loss, or asthenia, new cough, hemoptysis, pain in chest or bone
Neurological = brain mets
Lymphadenopathy >1 cm
Pneumonia, pleural effusion, change in voice (laryngeal nerve), SVC syndrome, Horner syndrome (ipsilateral ptosis miosis, and anhidrosis), brain metastases (headache, nausea, vomiting, seizures, dizziness, or AMS)?
lung cancer, more common NSCLC
How can small cell present a little bit differently in lung cancer (more aggressive, but less common)?
SIADH, hypercalcemia, digital clubbing, anemia, peripheral neuropathy
What are the risk factors for lung cancer?
~70, unusual <40, smoking = major risk factor
cigar, pipe smoking, secondhand smoke, air pollution, alcohol, low fiber diet, inheritance
What are the types of NSCLC (MCC)?
Adenocarcinomas - most common, from any epithelial cell within or distal to terminal bronchioles
Squamous cell - from bronchial epithelium + intraluminal masses
Large cell - aggressive + rapid doubling, central or peripheral masses
(staging: T,N,M)
Adenocarcinomas are ________ located
peripherally
Squamous cell CCCP
centrally located
cavitary lesions
hypercalcemia
Pancoast syndrome
SCLC is most associated w/
paraneoplastic syndromes, central, small dark blue cells w/ Rosette formation
Staging: T,N,M or limited/extensive
Regional or distant metastasis on presentation
MYC gene mutation
SCLC
What do these diagnostics indicate
PE: wheeze/stridor, dyspnea
Hct <40%m, <35%w
abnormal findings on CXR or CT?
lung cancer
What other Dx can indicate lung cancer?
sputum cytology (specific but insensitive)
CT-guided biopsy of peripheral nodules (risk of pneumothorax)
Thoracentesis (malignant pleural effusions)
Fine needle aspiration
What is recommended screening for lung cancer?
Recommended screening with low-dose chest CT in high-risk individuals - 50-80 years who have no symptoms of lung cancer + 20 PPY smoking history who currently smoke OR have quit within 15 years
What is staging for bone cancer?
Bone scan for STAGING – TNM staging system
T - size + location of primary tumor
N - presence + location of nodal metastases
M - presence or absence of distant metastases
What are the stages of cancer?
Stage I = cancer confined to lung tissue w/ no involved lymph nodes
Stage II = confined to lung tissue w/ involved surrounding lymph nodes or within chest cavity
Stage III = confined to lung tissue or involving adjacent structures + lymph nodes in mediastinum
Stage IV = spread to opposite lung or any organ and disseminated
What is the treatment of NSCLC
Surgical resection
No surgery in risky situations
Chemotherapy
Radiotherapy
What is the treatment for SCLC
Highly aggressive → chemotherapy (cisplatin + etoposide)
Palliative care - Yag laser, radiation, brain resection, thoracentesis, pain control
What separates a benign pulmonary nodule from a malignant one?
Smooth, defined edge = benign
Ill defined margins, speculated, lobular = malignancy
Pulmonary nodules are ___ in <30
rare, increasing w/ age, smokers
What are benign pulmonary nodules?
infectious granulomas
What is the diagnosis steps for pulmonary nodules?
CXR –> chest CT for any suspicious nodule –> assess risk
if concerning, transthoracic needle aspiration/bronchoscopy, PET scan, sputum cytology
How would you treat a pulmonary nodule?
Biopsy vs. excision
Video-assisted surgery (VATS) resection of all solitary pulmonary nodules w/ intermediate probability of malignancy
What are some concerning signs of a mediastinal mass?
Insidious onset of retrosternal CP, dysphagia, dyspnea
What are middle mediastinal masses?
lymphadenopathy, pulmonary artery enlargement, aneurysm of aorta, developmental cyst, dilated azygos vein
What are anterior mediastinal masses?
thymoma, teratoma, thyroid lesions, lymphoma, mesenchymal tumors (lipoma, fibroma)
What are posterior mediastinal masses?
hiatal hernia, neurogenic tumor, meningocele, esophageal tumor, thoracic spine disease, extramedullary hematopoiesis
What are diagnostic tests for mediastinal masses?
CT scanning
Barium swallow if esophageal disease is suspected
Doppler sonography or venography of brachiocephalic veins + SVC + angiography
MRI
tissue diagnosis is necessary if a neoplastic disorder is suspected
Adenocarcinoma is one of the only lung cancers that have no association with ____
smoking – may be more correlated with genetics or family history
