Test 2 Flashcards
metastasis to the lung from?
Carcinoma of the kidney, breast, rectum, colon, and cervix and malignant melanoma are the most likely primary tumors
(pulmonary hypertension secondary to chronic thromboembolism): o This group consists of patients with pulmonary hypertension due to thromboembolic occlusion of the proximal and distal pulmonary arteries.
GROUP 4
treatment of canecer
stage I and II also while receiving chemotherapy (adjuvant). Stages III should undergo multimodality treatment inlcuding (neoadjuvant) chemo or radiotherapy improving survival as well as targeted cytototoxic chemotherapy and immunotherpay. Stage IV patients are treated with systemic ther- apy (targeted therapy, chemotherapy, or immunotherapy) or symptom-based palliative therapy, or both. If surgery is contraindicated stereotactic body radiotherapy especially for stage III. Should be individuallized based on molecular profiling and genetic testing key driver mutations
-Hemoglobin normal (12-15 g/dL) -PaO2 normal - slightly reduced (65-75 mmHg) -SaO2 normal at rest -PaCO2- normal - slightly reduced (35-40mmHg) -CXR shows hyperinflation w/ flattened diaphragm -vascular markings diminished, particularly at apices
emphysema CXR and lab
indications to use corticosteroid for sarcoidosis
most remit w/in two years
Hypercalcemia
Iritis
Uveitis- blurred vision
Arthritis
CNS involvement
Cardiac involvement
Granuloma hepatitis
Cutaneous lesions other than erythema nodosum
Progressive pulmonary lesions
Long termtherapy (months to years)-, requires immunosuppressive medications(methotrexate, azathioprine, infliximab)
-major complaint: chronic cough - productive mucopurulent sputum -frequent exacerbation due to chest infection -presents in late 30 &40s -dyspnea= mild -limitations during exercise -frequently over weight -cyanotic, but comfortable at rest -peripheral edema -Chest is noisy w/ rhonchi; wheezed common
chronic bronchitis (blue bloater)
absolute contraindications for PE relative:
intracranial hemmorhage, cerebral vascular lesion, intracranial neoplasm, stroke, closed-head trauma, aortic disection active bleeding/menses chronic, severe, poorly controlled hypertension or on presentation, stroke 3 months ago, CPR or surgery <3 weeks, internal bleeding 2-4 weeks, pregnancy, ulcer, pericarditis, diabetic retinopathy
- Leukopenia, eosinophilia - Elevated erythrocyte sedimentation rate - Hypercalcemia (5% of patients) - Hypercalciuria (20% of patients) Elevated Angotensin-converting enzyme (ACE) levels (40-80% of patients). PFT: restorative changes and diffuse capacity sometimes: airflow obstruction
Stage 1-BHL (no symptoms-mild) good prognosis
Stage 2- BHL + ILD (moderate symptoms)-
Stage 3- ILD only- poor prognosis
Stage 4- fibrosis predominately in upper lobes(volume loss/ restrictive disease)
Pulmonary Function Test will show restrictive results in advanced stages.
What the test should show to be restrictive:
Normal or increased FEV1/FVC ratio***
Normal or decreased FVC
Decreased lung volume**
Decrease in VC, RV,FRC,TLC
treatment?
lab findings for sarcoidosis
prognosis based on symptoms
(Pulmonary venous hypertension secondary to left heart disease): o Often referred to as pulmonary venous hypertension or “post-capillary” pulmonary hypertension, backing up stuff into the heart causing increased pressure> right sided enlargement further propagating problem o Includes: § Left ventricular systolic or diastolic dysfunction Valvular heart disease
group 2 pulmonary hypertension
Diasgnostic tests for PE
ECG, ABG, D-dimer; troponin, plasma BNP; radiography; CT (1st choice); ventilation perfusion screening; contrast venography for venous thrombosis; pulmonary angiography (last resort) ECG- pretty sensitive to find abnormal readings. With normal abnormalities for this disease including: sinus tachycardia, nonspecific ST and T wave changes. Rarely but can include: P pulmonale, right ventricular hypertrophy, RAD, RBBB. ABG- acute respiratory alkalosis due to hyperventilation. Arterial PO2 and alveolar-arterial Oxygen difference are abnormal for patients with this but are not definitive. Profound hypoxia with a normal chest radiograph in the absence of preexisting lung disease is highly suspi- cious for PE. D-dimer (Plasma levels of D-dimer, a degradation product of cross-linked fibrin, are elevated in the presence of thrombus.) , (overused elevated by age cannot be used alone and for low and intermediate risk patients) troponin, plasma BNP- higher in patients with PE not definitive but correlate with adverse outcomes such as mechanical ventilation, prolonged hospitalization, and death. Radiography- necessary to permit interpretation of ventilation perfusion scan-, but will not establish diagnosis by itself. Found atelectasis, parenchymal infiltrates, and pleural effusions. Hump-distal occlusion or central clot enlarged pulmonary artery 1st choice CT-PA- helical CT pulmonary angiography. initial diagnostic study for PE. Requiring intravenous radiocontrast dye and is sensitive for detection of thrombus in proximal pulmonary arteries. Ventilation-perfusion lung screening- Radiolabeled microaggregates albumin are injected into venous system, allowing the particles to embolize to the pulmonary capillary bed. Patient will breath a radioactive gas/aerosol while its distribution is recorded. With a defect on perfusion scanning illustrating diminished blood flow to that region of the lung. For confirmation of PE these findings are used in conjunction with the ventilation scan to a give a high, low or intermediate probability that PE is the cause of these abnormalities. However, having two or more segmental perfusion defects is sufficient to diagnose PE in most cases. If there was low or intermediate probability then it was not confirmatory. Venous thrombosis- most patients w/ PE will have DVT (70%) half of pt with DVT have PE. Contrast venography- is the reference standard for diagnosing DVT with intraluminal filling defect is diagnostic of venous thrombosis only now useful for discrepancy between suspicion and noninvasive testing. The use of venous ultrasonography can detect proximal DVT with inability to compress common femoral of popliteal veins is a strong indicator. Less active in distal thrombi, or patients who are asymptomatic. The use of ultrasonography and impedance plethysmography (relies on changes in electrical impedance between patient and obstructed veins to determine presence of thrombus) when used together can help find DVT. Pulmonary angiography-if all fails and you still suspect reference standard for the diagnosis of PE. An intraluminal filling defect in more than one projection establishes a definitive diagnosis. Findings: abrupt arterial cutoff, asymmetry of blood flow (segment oligemia), prolonged arterial phase with slow filling. Safe but invasive procedure complications are only 5%. Usually used when there is doubt and a high probability of PE
irreversible lung impairemtna s a result of sarcoidosis
Pneumothorax
Hemoptysis
Mycetoma formation in lung cavities
Respiratory failure
Myocardial sarcoidosis
· chronic productive cough · Dyspnea on exertion · Wheezing respirations · Fatigue · Weakness
Dependent edema and RUQ abdominal pain may appear ·
Clubbing and Cyanosis (Cyanosis is more prevalent if there is right to left shunting via a patent foramen ovale) · Distended neck veins heart sounds: · Tricuspid regurgitation · RV heave or gallop (or both)- Right ventricular hypertrophy (only seen in cor pulmonale caused by pulmonary HTN) · Prominent lower sternal or epigastric pulsations · Enlarged and tender liver Ascites
symptoms for cor pulmonale
nonpharmacological treatments for pulmonary embolism
inferior vena cava filter- patients with a major contraindication to anticoagulation have or are at a high risk for development of proximal DVT or PE. Recommended for recurrent thromboembolism despite adequate anticoagulation, for chronic recurrent embolism with a compromised pulmonary vascular bed (pulmonary hypertension) with concurrent performance of surgical pulmonary embolectomy or pulmonary thrombo-endarterectomy. percutaneous transjugular placement- mechanical filter preferred inferior vena cava. educe the short-term incidence of PE in patients presenting with proximal lower extremity DVT. However, they are associated with a twofold increased risk of recurrent DVT in the first 2 years following placement so plans must be usually made for their subsequent removal.
diagnostic of lung cancer
Sputum cytologyis highly specific but insensitive, it is best used when there are lesions in the central airways.
Thoracentesis(sensitivity 50–65%) can be used to establish a diagnosis of lung cancer in patients with malignant pleural effusions. Fine-needle aspiration (FNA) of palpable supraclavicular or cervical lymph nodes is frequently diagnostic.
Fiberoptic bronchoscopyallows visualization of the major airways, cytology brushing of visible lesions or lavage of lung segments with cytologic evaluation of specimens, direct biopsy of endobronchial abnormalities, blind transbronchial biopsy of the pulmonary parenchyma or peripheral nodules, and FNA biopsy of mediastinal lymph nodes. The use of fluorescence bronchoscopy improves the ability to identifyearly endobronchial lesions
Nearly all patients with lung cancer have abnormal findings on chest radiography or CT scan. These findings are rarely specific for a particular diagnosis. The sensitivity and specificity of CT imaging for identifying lung cancer metastatic to the mediastinal lymph nodes are 57% (49–66%) and 82% (77–86%), respectively. Therefore, chest CT imaging alone does not provide definitive staging information. CT imaging helps determine where to biopsy, and how the mediastinum should be sampled. Positron emission tomography (PET) using 2-[F] fluoro-2-deoxyglucose (FDG) is an important modality for identifying metastatic foci in the mediastinum or distant sites.
spread along preexisting alveolar structures (lepidic growth) without evidence of invasion.
Adenocarcinomas in situ (bronchioloalveolar cell carcinoma)-
tachypnea, tachycardia, chest wall retractions, expiratory grunting, nasal flaring & cyanosis
atelectasis & profusion w/o ventilation. reticular ground-glass opacities + air bronchograms,* poor expansion. waxy collapsed alveoli - Domed diaphragms
name it, what causes it, and how to treat
hyaline membrane disease
produced in premature infants after C-sectional birth, infant infections, maternal diabetes
corticosteroid
major complaint: dyspnea - severe, after age 50 -cough is rare w/ scant mucoid sputum -pts thin, recent weight loss common -appear uncomfortable -used of accessory mm in respiration -chest is quiet , w/o adventitious sounds -no peripheral edema
Hyperinflation:
-flat diaphragm
↑AP diameter
↓vascular markings (peripheral arterial deficiency)
±bullae
emphysema (pink puffer) symptoms
Common symptoms:
Progressive dyspnea
Inspiratory crackles
Some cases, clubbing and cyanosis
conglomeration and contraction in upper lobes
eggshell calcification vs. nodular interstitial fibrosis and honeycombing
what rare condition is it associated w/?
pneumoconosis
sillicosis vs. asbestos
more likely to develop Tb
Caplan syndrome- rare condition; necrobiotic rheumatoid nodules (1-5cm ) in periphery of lungs with rheumatoid arthritis
Anorexia, weight loss, or asthenia occurs in a majority of patients presenting with a new diagnosis of lung cancer. Most have a new or change in a chronic cough; 6–31% havehemoptysis;and 25–40% complain of pain, either nonspecific chest pain or pain from bony metastases to the vertebrae, ribs, or pelvis.
atelectasis and post-obstructive pneumonia, pleural effusion (12–33%), change in voice(compromise of the recurrent laryngeal nerve), superior vena cava syndrome(SCLC obstruction of the superior vena cava with supraclavicular venous engorgement), and Horner syndrome
cancer symptoms
Cough, sputum production, dyspnea, chest pain, and wheezing, decreased exercise tolerance, and recurrent hemoptysis are typical complaints. Patients also often complain of chronic rhinosinusitis symptoms, steatorrhea (fst in feces), diarrhea, and abdominal pain Digital clubbing, increased anteroposterior chest diameter, hyperresonance to percussion, and apical crackles are noted on physical examination. Sinus tenderness, purulent nasal secretions, and nasal polyps may also be seen.
CF symptoms
heterogeneous group of undifferentiated cancers that do not fit into other categories. Typically aggressive and have rapid doubling times. They present as central or peripheral masses. Large cells
Large Cell Carcinoma- (2%)
- (acute or subactue) sudden onset Fever or hypothermia - Tachypnea - Cough without sputum - Dyspnea Common: - Chest discomfort pleuritic chest pain - Sweats of rigors, chills, or both - Pleurisy - Hemoptysis - Fatigue - Myalgia - Anorexia - Headache Abdominal pain Inspiratory crackles (airway obstruction and fluid)
Pneumonia symptoms
dyspnea (pain on inspiration), cough, chest discomfort of sudden onset (involvement of pleural surface of the lung). leg pain, hemoptysis, palpitations (tachypnea), and wheezing. In certain cases (name it) can cause syncope, hypotension and signs of right sided CHF
2- distention of pulmonary artery,
3-right ventricular wall stress and/or subendocardial ischemia related to acute pulmonary hypertension
2&3 found in larger emboli resembles MI]),
right to left shunt changes in cardiac output and atelectasis s
what is the main mechanism?
pulmonary embolism symptoms
virchow’s triad- venous stasis damage to vessel wall, hypercoagulability (factor V)
(pulmonary hypertension secondary to lung disease or hypoxemia): o This group is caused by advanced obstructive and restrictive lung disease. o Includes: § COPD § Interstitial lung disease § Pulmonary fibrosis § Bronchiectasis As well as other causes of chronic hypoxemia, such as sleep-disordered breathing, alveolar hypoventilation syndromes, and high altitude exposure.
GROUP 3
-Chronic excessive cough, dyspnea (noted initially on heavy exertion later on at rest), sputum production exacerbations of these usually precipitated by infection or enviornomental -Ronchi, decreased intensity of breath sounds, prolonged expiration of physical examination -Airflow limitation on pulmonary function testing that is not fully reversible and is most often progressive
symptoms of COPD
arise from mucous glands or from any epithelial cell within or distal to the terminal bronchioles.They usually present as peripheral nodules or masses and stainfor TTF-1 and Napsin-A on immunohistochemistry.
adenogland carcinomas-42%
(pulmonary arterial hypertension secondary to various disorders): o This group gathers diseases that localize directly to the pulmonary arteries leading to structural changes, smooth muscle hypertrophy, and endothelial dysfunction. o Includes: § Idiopathic (formerly primary) pulmonary arterial hypertension (Most Common in middle aged or young women) § BMPR2 gene defect. Normally inhibits pulmonary vessel smooth muscle growth and vasoconstriction. Rare. § heritable pulmonary arterial hypertension § HIV infection § Portal hypertension Drugs and toxins
Group 1 pulmonary hypertension
No dyspnea, fatigue, chest pain, or near syncope with exertion. Pulmonary hypertension resulting in slight limitation of physical activity.
No symptoms at rest but ordinary physical activity causes dyspnea, fatigue, chest pain, or near syncope. Pulmonary hypertension resulting in marked limitation of physical activity.
No symptoms at rest but less than ordinary activity causes dyspnea, fatigue, chest pain, or near syncope with inability to perform any physical activity without symptoms. Evidence of right heart failure.
Dyspnea and fatigue at rest and worsening of symptoms with any activity.
Class I
Class II
Class III
Class IV
dyspnea, cyanosis, restlessness, confusion, anxiety, delirium, tachypnea, bradycardia or tachycardia, hypertension cardiac dysrhythmias, and tremor. b. New, bilateral radiographic opacities not explained by pleural effusions, atelectasis, bilateral infiltrates, air bronchograms, or nodules minimal edema . crackles (rales) popping open collapses alveoli (fluid, exudates, lack of aeration)
ARDS symptoms
pharmacological treatment for PE
heparin- during hospital stay low molecular weight- patients w/ cancer unfractioned for most people subpar level can cause PE 34-48 hours later then: 3-6 months of oral warfarin Patient’s age and D-dimer, Potential reversible risk factors. Likelihood and potential consequences of hemorrhage
interstitial infiltrates
§ Dyspnea on exertion (Most common), at rest (if severe) § Chest pain § Weakness § Fatigue § Cyanosis § Edema § Syncope on exertion (if there is insufficient cardiac output or an arrhythmia) Hemoptysis (rare but life threatening)
Signs of right-side heart failure: Jugular venous distension, Peripheral edema, ascites
Accentuated S2
S3 heart sound- after S2 fluid hitting closed pA and aorta
Tricuspid regurgitation murmur
Right ventricular heave
Systolic ejection click
Hepatomegaly
Cyanosis (seen in patients with a patent foreman ovale -> right to left shunt)
symptoms for pulmonary hypertension
“coin lesion,” is a less-than-3-cm isolated, rounded opacity on chest imaging outlined by normal lung and not associated with infiltrate, atelectasis, or adenopathy. Most are asymptomatic
pulmonary nodule carry significant risk of malignancy
PET scans are popular and highly sensitive and specific except for adenocarcinomas, carcinoids and bronchioloalveolar tumors.
Increase in density of the affected lung
Displacement of the fissures or the mediastinum towards the atelectasis
Crowding of the vessels and bronchial tree in the area of volume loss
Elevation of the hemidiaphragm
Overaeration of the opposite lung
Passive -
• patient with pain not taking full breaths
– Post-obstructive
• patient with lung CA
– Cicatrization
• from scar, radiation injury, old pneumonia
– Adhesive
• from loss of surfactant (e.g. ARDS)
atelectasis
tumors of bronchial origin that typically begin centrally, infiltrating submucosally to cause narrowing of the bronchus without a discrete luminal mass. They are aggressive cancers that often involve regional or distant metastasis on presentation.
Small Cell Carcinoma- (13%) t
pneumoconosis name the kinds
Pneumoconiosis- a type of occupational pulmonary diseasedescribed as achronic, fibrotic lung diseasecaused byinhalation of inorganic dusts.
inorganic dust stimulates alveolar macrophages –> inflammation–> release of chemical mediators –>parenchymal fibrosis –> restrictive lung disease = decrease in lung compliance
Coal worker’s pneumoconiosis (black lung disease)-
Silicosis- inhalation of rock, quarry, stone cutting, tunneling, sandblasting, pottery, diatomaceous earth.
asymptomaticand no changes in PFT will be seen
Incidence of pulmonary TB is increased with silicosis
Silicotic nodules- small rounded opacities(0.3-5 mcm) throughout the lung
Eggshell calcification- strongly suggest disease
Large conglomerate densities in upper lungwith complicated silicosis
Asbestosis- Mining, insulation, construction, shipbuilding, pipe fitters
Described as a nodular interstitial fibrosis.
Linear streaking at lung bases (lower lobes)
Opacities of various shapes and sizes
Honeycombchanges in advanced stages
Pleural calcifications
(from pearls) shaggy heart sign CT is the best method (parenchymal fibrosis and pleural plaque)
malaise fever dyspnea of insidious onset erythema mnodsum (bilateral tender) lupus pernio- violet raised discoloration of nose, cheek, ear, and chin (frost bite) uveitis- inflammation of iris and ciliary body causing blurred vision, ocular discomfort, photophobia, ciliary flush, and floaters, CN & palsy, arthritis carydiomyopathy parotid gland enlargement hepatoand lymphadenopathy (hilar) Noncaseating granulomas>fibrosis
sarcoidosis symptoms
-Hemoglobin elevated (15-18g/dL) -PaO2-reduced (45-60mmHg) -PaCO2- slightly-markedly elevated (50-60 mmHg) -CXR- increased interstitial markings (dirty lungs) -especially at bases
±Signs of cor pulmonale* (peripheral edema, cyanosis)
Respiratory acidosis*
↑Hct/RBC*(chronic hypoxia stimulates erythropoiesis)
↑AP diameter
↑ vascular markings
Enlarged right heart border
Nonspecific peribronchial and perivascular marking
bronchitis CXR and lab
indications for length of time for pharmacological treatment of PE complications;
Extended therapy indefinite recommended for an unprovoked episode with low-moderate risk of bleeding, cancer intracranial hemorrhage,
hemoptysis, cough, focal wheezing, and recurrent pneumonia. edunculated or sessile growths in central bronchi. Fiberoptic bronchoscopy may reveal a pink or purple tumor in a central airway. These lesions have a well-vascularized stroma, and biopsy may be complicated by significant bleeding. CT scanning is helpful to localize the lesion and to follow its growth over time
what it is and the medical condition associated w/ it?
carcinoid tumor
Very common and can cause Carcinoid syndrome (flushing, diarrhea, wheezing, hypotension) is rare.
(pulmonary arterial hypertension secondary to hematologic, systemic, metabolic, or miscellaneous causes): o These patients have pulmonary hypertension secondary to: § Hematologic disorders such as chronic hemolytic anemia, myeloproliferative disorders, and splenectomy § Systemic disorders such as sarcoidosis, vasculitis, pulmonary Langerhans cell histiocytosis, and neurofibromatosis type 1 § Metabolic disorders such as glycogen storage disease, Gaucher disease, and thyroid disease Miscellaneous causes such as tumor embolization, external compression of the pulmonary vasculature, end-stage renal disease on dialysis.
GROUP 5
parenchymal pulmonary opacity -atypical- patchy air space opacities -typical- lobar consolidation w/ air bronchograms to diffuse alveolar or interstitial opacities Additional findings: -pleural effusions -cavitation
Pneumonia CXR
arise from the bronchial epitheliumand often present as an intraluminal mass. They are usually centrally located and can present with hemoptysis.
Squamous cell carcinoma- (23%)
sinus tachycardia, nonspecific ST and T wave changes. Rarely but can include: P pulmonale, right ventricular hypertrophy, RAD, RBBB. elevated D-dimer troponin, plasma, BMP higher atelectasis, parenchymal infiltrates, and pleural effusions.
lab findings of pulmonary embolism
Pneumothorax w/ no precipitating event, lung disease (or unrecognized), subpleural bleb
Risk factors, smoking, marfan syndrome, thoracic endometriosis
complication of underlying lung diesease
primary
secondary
Tests to conduct for pneumonia
treatment for it
Sputum Gram Stain (40% of pt.s cannot produce a sputum sample)
- S aureus (including MRSA) not S. pneumoniae
- gram - rods
Urinary antigen test not affected by antibiotic therapy
- S pneumoniae
- legionella species
3. rapid antigen test for influenza
CAP: treat w/ antibiotics right away 1st: macrolide or doxycycline minimum of 5 days therapy
HAP: beta lactam + macrolide or broad spectrum FQ
charactersitics of COPD lung structure
Smoking–> chronic inflammation & decreased protective enzymes ( α-1 antitrypsin) while increasing damaging enzymes ( ↑elastase release from macrophages & neutrophils) –>loss of elastic recoil(airway collapse, expiration becomes an active process) & ↑compliance–>airway obstruction (↑ air trapping)
Increase in residual volume (RV)
Increase in TLC ( total lung capacity)
Increase of RV/TLC ratio–> air trapping (particularly in emphysema)
diagnosing pulmonary hypertension
Right-sided heart catheterization (Gold Standard)
Definitive diagnosis and quantification
Mean pulmonary arterial pressure ³ 25 mm Hg at rest (or >30 mm Hg during exercise)
Useful for determining if hypertension stems for the venous side or the arterial side.
CXR, EKG, PFT- stress test
echocardiogram w/ doppler- assess and underlying cardiac issuewhile the doppler flow can estimate the right ventricular systolic pressure
CBC- Polycythemia with increased hematocrit (slow growing blood cancer)
V/Q lung scanning- Useful to help rule out chronic thromboembolic pulmonary hypertension in unexplained area. very sensitive test that can differentiate chronic thromboembolic pulmonary hypertension from idiopathic pulmonary arterial hypertension.
treatment for idiopathic pulnonary hypertension
poor prognosis especially if w/ cor pulmonale
Vasoreactivity trial with inhaled nitric oxide, IV adenosine, or calcium channel blocker. If vasoreactive, treatment with calcium channel blockers is 1stline therapy. DO NOTtreat with calcium channel blockers if no vasoreactivity, as they can be harmful.
If not vasoreactive, other medications include:
Prostacyclins (Epoprostenol, Iloprost)- limitations short medication half life requiring reliable continuous infusion, difficult tiration, and high cost. implants
Phosphodiesterase-5 inhibitors (Sildenafil, Tadalafil)
Oral Endothelin receptor antagonists (Bosentan) - these cause vasodilation.
Inhaled and subcu prostanois (iloprost and treprostinil) used in patients unable to tolerate continuous iV
right sided heart failure secondary to pulmonary disease. attendant hypoxia or from pulmonary vascular disease (pulmonary hypertension).
causes include: PHT, COPD IPF
disease and treatment?
cor pulmonale
Inotropic agents are useful when acute decompensation occurs.
Idiopathic disease by history and inspiratory crackleson PE
Restrictivepulmonary function test
CXR showsprogressive fibrosis over several years
Diffused, patchy fibrosis with pleural based honeycombing**on high CT scan
IPF
bronchoalveolar lavage- identify infection (p. jirove, mycobacteria, malignint cells
transbronchial biopsy (also used to identify sarcoidosis) -A specific diagnosis CANNOT be confirmedwith this biopsy because its only one sample rather than viewing a pattern of changes.. Its good for excluding IPF from differential
surgical lung biopsy- preferred 2-3 biopsies
Increased # of macrophages evenly distributed in alveolar space
Minimal honeycomb change; rare fibroblast foci
Macrophage accumulation in bronchiolar air spaces
Alveolar integrity intact
CXR:
Has a nodular or reticulonodular pattern
Honeycombing rare
Maybe indistinguishable from UIP
CT:
Diffused ground glass opacitiesand upper lobe emphysema
Similar to UIP just in a younger population
Similar PFT results but less severe abnormalities
Seen in heavy smokerswith respiratory bronchiolitis
Respiratory bronchiolitis- associated interstitial lung disease (RB-ILD)
stop smoking and corticosteroidsare thought to be effective but no evidence to support
Nonspecific criteria per the rest
Varying ranges of inflammation and fibrosis
Patchy at first, more uniform over time suggesting response to a single injury
Most have lymphocytic and plasma cell inflammation without fibrosis
Scant honeycombing
CT:
Bilateral areas of ground glass and fibrosis
Honeycombing is rare
May be indistinguishable from UIP
Slight female predominance
Similar to UIP but onset of cough and dyspnea is over months, not years
Nonspecific interstitial pneumonia (NSIP)
Diffused alveolar damage
Similar to UIP butno honeycomb changeand more homogenous
Diffused, bilateral airspace consolidation with areas of ground glass
Also known as Hamman-Richsyndrome
Many are young patients
Acute dyspnea and respiratory failure
Half report viral syndrome preceding lung disease
Acute Interstitial Pneumonia (AIP)
- mechanical ventilation is essential
- high initial mortality= 50-90% die within 2 months after diagnosis
- not progressive if patient survives
- lung function can return to normalor be permanently impaired
-Masson bodies(buds of loose CT) and inflammatory cellsfill alveoli and distal bronchioles
CXR:
Lung volumes are normal
Interstitial and parenchymal disease with discrete, peripheral alveolar and ground glass infiltrates
Nodular opacities common
CT:
Subpleural consolidation
Bronchial wall thickening and dilation
Age 50-60 but wide range
Abrupt onset, frequently weeks to a few months with flu-like symptoms:
Fever, fatigue, weight loss
Prominent dyspnea and dry cough
PFT: most show restrictive, but 25% shows restrictive and obstructive
Cryptogenic organizing pneumonia (COP) formerly bronchiolitis obliterans organizing pneumonia (BOOP)
- rapid response to corticosteroidsin 2/3 of patients
- overall good prognosis for those who respond
- relapses are common
name disease and treatment
Nonuniform/patchy fibrosis
Honeycomb change
Type I pneumocytes lost, proliferation of alveolar type cell II
Fibroblast fociactively proliferating fibroblast and myofibroblasts
Inflammation is mildwith small lymphocytes
Intra alveolar macrophage accumulation is present but not predominant
CXR:
diminished lung volume
Increasedlinear or reticular bibasilar opacities, usually bilateral
CT:
shows minimal ground glass
Variable honeycombing
Slight male predominance
Insidious dry coughand dyspnealasting months to years
Clubbing**present(25-50%)
Diffusedfine late inspiratory crackles
Restrictiveventilatory defect and reduced capacityon PFT
usual interstitial pneumonia
Nintedaniband pirfenidonereduce the rate of decline in lung infection
