19. Pulmonary Path 2 Flashcards
Restrictive lung disease is commonly interchangable with chronic INTERSTITIAL lung disease. Idopathic pulmonary fibrosis makes up about 20% of ILD (interstitial lung disease), including what three diseases?
idiopathic pulomonary fibrosis (IPF)
Non specific interstitial pneumoina (NSIP)
Cryptogenic organizing pneumonia (COP)
What is a heterogenous group of disorders with inflammation and fibrosis of the pulmonary interstitium?
Chronic interstitial lung disease
What disease refers to a clinicopathologic syndrome marked by progressive interstitial pulmonary fibrosis and respiratory failure with unknown cause?
idiopathic pulmonary fibrosis (IPF)
idiopathic pulmonary fibrosis (IPF) damages pulmonary tissues in waves of inflammation injury leading to fibrosis. A patient will usually present with dyspnea, tachypnea, crackles (velcro like), restrictive pattern on PFTs (dec. DLco,FVC) and what on CXR?*2
Bilateral basilar infiltrates Honeycomb lung (end stage)
NOTE: ground glass shadows indicated interstitial fibrosis
There are thought to be many contributing factors to IPF including urban areas (industrialized societies) and SMOKING. What other 2 are common factors?
increasing age (>50) - older pts Genetic factors (in family): *MUC5B, telomerase/surfactant mutations
When all the predisposing factors (age, smoking, genetics) add up, causing persistant alveolar epithelial injury/activation releasing profibrogenic factors, activated fibroblasts lead to increase PI3K/AKT signaling causing?
synthesis and depositition of collagen = interstitial fibrosis & resp failure
The histological pattern of fibrosis in IPF is referred to as ?
Usual interstitial pneumonia (UIP) (more pronounced in subpleural regions and along interlobular septa)
Post pulmonary bx for IPF, once can see UIP, which presents as normal areas, inflammation, fibroblast foci (patchy interstitial fibrosis) and?
peripheral honeycombing - will see all four patterns together
What has dense fibrosis and cystic spaces lined with hyperplastic type II pneumocytes or bronchiolar epithelium?
IPF - honeycomb lung!
IPF is becoming more common and has a progressive course, with most patients expiring from resp disease in 3-5 years. Medications to arrest fibrosis such as tyrosine kinase inhibitors and TGF-B inhibitors may work minimally, but the best therapy for IPF is?
lung transplatation
What disease has diffuse fibrosis of unknown etiology whose lung biopsies lack the diagnostic features of any other intersitial lung dz, but has very unique histology to differentiate it from other fibrosing diseases?
Non specfici interstitial pneumonia (NSIP)
Non specfici interstitial pneumonia (NSIP) histologically has uniform infiltrates and fibrosis, no heterogeneity, no fibroblast foci, no granulomata and a better prognosis than?
usual interstitial pneumonia UIP
*no honeycombing
NSIP has diffuse uniform pattern on inflammation/fibrosis, which are all of the same stage*. Clinically NSIP presents as chronic dyspnea and cough for several?
months
NOTE: cellular pattern patients tend to be younger than those with fibrosing and have even better prognosis
What dz is formerly known as bronchiolitis obliterans organizing pneumonia (BOOP) with a unknown etiology, usually presenting with cough and dyspnea?
Cryptogenic organizing pneumonia (COP)
Cryptogenic organizing pneumonia (COP) presents as a pneumonia like consolidation in patients in their 50-60s, commonly have a past URI. Histologically what is characteristic of this dz?
Fibroblast foci called Masson bodies* which are polypoid plugs/swirls of loose CT within alveoli
Cryptogenic organizing pneumonia (COP) is diagnosed via exclusion( not infection, drug or toxin induced, or related to a connective tissue DO). What is the prognosis and tx?
Good prognosis and patients recovery fully from oral steroids
diffuse interstitial lung fibrosis gives rise to restrictive lung disease characterized by reduced lung compliance and reduced forced vital capacity. the ratio FEV1to FVC is?
NORMAL (because both decrease)
Autoimmune/CT disease can manifest as ILD, including SLE, systemic sclerosis (NSIP more common) and?
rheumatoid arthritis
If ILD occurs as a manifestation of these conditions, they are not diagnosed as pure disease, prognosis is linked to?
the severity of the underlying condition
What is a multi-system granulomatous disease of unknown etiology that can involve several organs and tissues, lung involvement or bilater hilar lymphadenopathy in 90% of cases?
Sarcoidosis
With sarcoidosis, you will see granulomatous inflammation with epithelioid histiocytes, mø, giant cells, WITHOUT what?
necrosis!
sarcoidosis manifests as non-caseating, non-necrotizing granulomata
Clinically it presents as dyspnea or more commonly incidental abnormal radiograph. There are 4 stages of sarcoidosis, 1 including only hilar lypmhadenopathy, stage 2 hilar lymphadenopathy and pulm infiltration, stage 3 is pulm infiltration only, and stage 4?
Fibrosis
most common is stage 1 NOTE: stages are not in order- people can jump around
sarcoidosis has granuloma inclusions such as laminated concretions composed of calcim and proteins known as Schaumann bodies and also have stellate inclusions known as?
Asteroid bodies (both these are found within giant cells 60% of the time)
Patients with sarcoidosis tend to be under the age of 40 years, and commonly involve lungs, lymph nodes, anyplace. There is a 10x increased risk in?
African americans
Again, sarcoidisis will present as dyspnea or incidental finding on radiology, likely to be immune related etiology with possible genetic predispostition. What serum levels will be elevated?
Serum angiotensin converting enzyme ACE
20% of patients have progressive lung disease with sarcoidosis, death may occur from pulmonary, cardiac or ?
neurologic involvement
Along with the lung disease, sarcoidosis pts may have spleen/liver involvments, lesions of bone resorption on phalangeals, subcutaneous nodules/red plaques, mucus membane lesions, iridocyclitis (corneal opacities, glaucoma), and what syndrome which is bilateral sarcoidosis of the major salivary gland?
Mikulicz syndrome
note: sarcoidosis is diagnosed via biopsy with noncaseating granulomas
What dz is a spectrum of immunologically mediated interstitial disorders due to inhaled organic antigens, primarily affecting the alveoli = extrinsic alveolar alveolitis?
Hypersensitivity pneumonoitis
Hypersensitivity pneumonoitis can be seen histologically with chronic inflammation and almost no normal parynchema at all along with granulomatous inflammation. What are the 3 different types of immune reaction to inhaled antigen?
Pigeon breeders lung (protein from bird poo) Farmers lung (actinomycetic spores in hay) Hot tub (rxn to mycobacterium avium complex MAC)
Hypersensitivity pneumonoitis is an airway centered granulomata reaction with associated lympochytes. What is a very important part of the dx of this dz?
THE HISTORY
Noncaseating granulomas present in 2/3 of the patients with Hypersensitivity pneumonoitis, T cell mediated type 4 HS. Clinically it is variable: recurring fever, dyspnea, cough. Pulmonary tests indicate what?
restrictive lung dz (FEV1/FVC is normal)
What is a past or present smokers disease seen with larger patchy collections of smokers macrophages in alveoli of a current or former smoker?
DSIP (Desquamative interstitial pneumonia)
DSIP (Desquamative interstitial pneumonia) occurs in smokers in their 40-50s and have restrictive lung disease presentation with histology showing VERY characteristic ‘stuffed’?
alveolar spaces full of macrophages
alveolar septa are thickened by inflam infiltrate of lymphcytes, plasma cells, sometimes eosinophils
DSIP (Desquamative interstitial pneumonia) has a good prognosis - greater than 95% survival rate at 5 years, and be treated in what two ways?
STOP SMOKING
corticosteroids
(excellent response)
What dz is part of a spectrum with DSIP but less symptomatic and has an earlier presentation of 30-40s and is smoking related?
Respiratory bronchiolitis-Interstitial lung disease (RB-ILD)
Respiratory bronchiolitis-Interstitial lung disease (RB-ILD) can be diagnosed via chest CT which would prompt for a?
biopsy
Respiratory bronchiolitis-Interstitial lung disease (RB-ILD) histologically you can see macrophages present to a lesser extent (smokers macrophages like DSIP), may have mild peribronchiolar fibrosis and ?
peribronchiolar metaplasia (abnormally located ciliary cells
Respiratory bronchiolitis-Interstitial lung disease (RB-ILD) is often reversible if?
smoking is stopped early
What is a rare disease of young adult smokers, with most cases resolving with smoking cessation suggesting that the lesions are reactive inflammatory process?
Pulmonary langerhans cell histiocytosis (LCH)
Pulmonary langerhans cell histiocytosis (LCH) has progressive scarring leading to cysts which can rupture and cause?
pneumothorax
histologically, Pulmonary langerhans cell histiocytosis (LCH) has eosinophils, nodules with langerhans cells (immature dendritic cells w grooved nuclei) and varying fibrosis/cysts.. What is a characteristic of this dz?
Stellate (star) lung lesions
Pulmonary langerhans cell histiocytosis (LCH) has eosinophils (pink tinge throughout langerhans) and will also test positive for ? 2
S100
CD1A
What is a rare entity characterized by surfactant accumulation in alveoli and bronchioles due to defects related to GM-CSF (granulocyte macrophage colony stimulating factor) or pulmonary macrophage dysfunction?
Pulmonary alveolar proteinosis (PAP)
There are 3 types of Pulmonary alveolar proteinosis (PAP), including autoimmune (most common >90%), secondary and hereditary (least common-neonates fatal). Typically is middle aged female and due to impaired metabolism of surfactant = too much, how can it be tx?
GM-CSF subcutaneously
Pulmonary alveolar proteinosis (PAP) is characterized by granular pink proteinaceous collection filled in alveolar spaces. If a lung lavage is done, what does it show?
COPIOUS amounts of milk fluid (so much surfactant)
Pulmonary alveolar proteinosis (PAP) is periodic acid schiff (PAS) positive, with MARKED increase in size and weight of the lung, clinically pts present with cough with abundant sputum containing chunks of?
gelatinous material - pathognomonic
What is common in bedridden pts or those with hypercoagublability, such as severe burns, trauma, frature pts, with DVT causing 95% of the cases?
Pulmonary embolism
Pulmonary embolism occurs in pts predisposed to clotting such as cardiac issues prolonged imobilization, hip frature, hypercoagulability (factor V leiden), obesity, IV lines and what in women?
Oral contraceptives/pregnancy
Emboli can cause hemorrhage or infarct, infarcts occuring in the lower lobes, causing what shaped lesions due to radiating pattern vessels?
wedge shaped infarcts
What should one look for on autopsy to know of the embolism was post mortem or ante mortem?
Lines of Zahn = ante-mortem
An pulmonary infarct over time is classically hemorrhagic and appears red-blue in early stages with the apposed pleural surface covered by a fibrinous exudate overlying fibrinous pleuritis which can form a ?
pleural friction rub
Large (saddle) pulmonary embolism causes instantaneous death due to electromechanical dissociation (no pulse cause no blood). Does the patient die d/t heart or lung problems?
HEART! right heart failure/cor pulmonale
no pulmonary infarcts are seen
Emboli present as transient chest pain, hemoptysis, cough and fever and on CXR a wedge shaped infiltrate 12-36hrs after.. DVTs are diagnosed via?
duplex ultrasound
Bone marrow embolisms are seen in trauma (breaking ribs when doing CPR) and are usually accompanied by fat embolism. What can be seen in IVDU on histology, seen as irridescent and shiny lesions, which under polarized light looks refractile?
Talc Embolism
What can cause nodules in IVDU, which come off of heart valves and cause neutrophilic inflammation leading to infection with abscesses/cysts?
Septic Emboli from vegetations usually off the tricuspid valve