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