Flashcards in Module 7 pulmonary Deck (41):
The next restrictive lung disease is called nonspecific interstitial pneumonia (NSIP), what are the features of this disease?
2. Better prognosis
3. Younger patients
4. Uniform fibrosis process (Chicken wire)
5. Two variants: cellular (more inflammation) and fibrosing (more fibrosis)
6. Lacks honey combing change, fibroblast foci and heterogeneity
The third fibrosing restrictive lung disease is called cryptogenic organizing pneumonia aka bronchiolitis obliterans organizing pneumonia (BOOP), what are some features?
2. Cough, SOB and recover either spontaneously or with steroids
3.CXR: patchy peribronchial/sub-pleural consolidation
4. Histology: Masson bodies: polypoid plugs of loose CT in alveolar ducts, alveoli and bronchioles ; Alveolar architecture not destroyed
The fourth fibrosing restrictive lung disease is called Collagen Vascular disease (CVD). what are some characteristics?
1. group of diseases that affect collagen
2. Disease of which inflammation or weakness in collagen tends to occur
3. Associated with collagen and blood vessel abnormalities; often autoimmune
4. Can affect joints,skin ,blood vessels or other organs
What are examples of CVD that have pulmonary manifestations?
1. Sjogren's sydrome
4. Systemic Sclerosis
5. Dermatomyositis- polymyositis
What are some pathologic patterns with CVD?
1. Usual interstitial pneumonia (UIP): pattern of fibrosis
2. non specific interstitial pneumonia (NSIP)
3. Organizing pneumonia
4. Bronchiolitis +/- fibrosis
5. Pleural involvement can occur: pleuritis, pleural nodules (thickening) and pleural effusion
Lymphoid Interstitial pneumonia (LIP) is included in the CVD category, what are some features?
2. Expansion of the interstitium by sheets of lymphoid cells
3. Associated with: CT diseases, Autoimmune diseases, HIV
4. May transform to lymphoma
What are some other cause of interstitial fibrosis?
Wegener granulomatosis: inflammation of the blood vessels with granulomas
The next category of restrictive lung disease is granulomatous, what two diseases are included in this?
HP: hypersensitivity Pneumonia
Sarcoidosis is a restrictive lung disease that affects what part of the population?
african american and danish/swedish women
What is the etiology and pathogenesis for sarcoidosis?
Etiology: Idiopathic, dx of exclusion
Path: type IV HSR with non caseating granulomas (also called non necrotizing granuloma) with lymphangetic distribution
What is the most common symptom for sarcoidosis?
---found incidentally on CXR: bilateral hilar lymphadenopathy which eventually leads to honey comb lung with pul fibrosis (restrictive lung disease)
--patients much later on in disease will get dry cough and SOB on exertion
Sarcoidosis starts in the lung but spreads. What are the organs and associated symptoms for spreading?
Skin: Erythema Nodosum (painful rash on anterior aspect of LE)
Neuro: facial nerve paralysis
Kidney: fibrosis and therefore renal failure
Liver: Fibrosis and therefore cirrhosis and HCC
Heart: Restrictive cardiomyopathy (which can lead to arrhythmia's)
Parotid and Lacrimal Glands (sicca syndrome -- dry eyes and mouth)
Milkulicz syndrome: Uveitis with bilateral parotid gland involvement
Arthritis in joints
Sarcoidosis can also spread to the blood, what do you find in the blood?
Lymphopneia (using up all your CD4 T cells in formation of the granulomas)
Why metastatic calcification? Epitheloid histocytes (Activated macrophages) activate vit D precursors -- this gives you hypercalcemia
Elevated ACE: not useful for dx just treatment
What is the best investigation for sarcoidosis?
Biopsy of the lung or hilar lymph nodes, see granuloma with on central necrosis (this is in big pic on slide 8) and mutli nucleated giant cells
On histology for sarcoidosis, what types of inclusion bodies are seen in the granuloma?
Pic on bottom shows ASTEROID BODIES - proteinaceous inclusions of giant cells
Pic on top shows Schaumann body - laminated calcium concretions due to metastatic calcifications
What are the complications for sarcoidosis?
1. Cor Pulmonale
2. Contraction atelectasis
3. Facial nerve paralysis
4. Restrictive cardiomyopathy
5. Renal Failure
6. Liver cirrhosis and HCC
What is the treatment for sarcoidosis?
Lung transplant (problem is that high chance of recurrence after transplant)
Review: What other diseases do non caseating granulomas?
5. Hypersensitvity Pneumonitis
The next granulomatous restrictive lung disease is hypersensitivity pneumonia (HP) also called extrinsic allergic alveolitis (EAA), what is this disease?
Inflammation of alveoli caused by HSR to inhaled organic antigens (Cotton, hay, grain or compost)
What is the etiology and pathogenesis of HP/EAA?
Etiology: Inhaled organic antigens(often called farmers lung and related to occupational exposures) --- damage to level of alveoli --- restrictive lung disease ----- decreased capacity, lung, compliance and total lung volume.
Pathogenesis: immune complexes mediated (type III) and cell mediated (type II) HSR to an extrinsic antigen
What is the histopathology of HP/EAA?
1. Airway centered process
2. Chronic inflammatory process
3. Organizing pneumonia
4. GIant cells and poorly formed non-necrotizing granuloma
In HP/EAA acute symptoms occur soon after exposure and symptoms improve when what happens?
when patient leaves the environment where Ag is located
The next set of restrictive lung diseases are smoking related, what are the associated diseases?
Respiratory Bronchiolitis-Interstitial Lung Disease (RBILD)
Desquamative Interstitial Pneumonia (DIP)
In RBILD, what are some features?
1.Seen in smokers in their 40s and 50s
2. Accumulation of smoker's macrophages within the lumen of respiratory bronchioles
3. Associated with peribronchiolar fibrosis
4. Responds to steroids and smoking cessation
In DIP ,what are some features?
1. Rare pneumonia, seen in smokers
2. Prominent filling of alveolar spaces by alveolar macrophages
3. Minimal alveolar fibrosis
Now moving on to the infectious diseases of the lung, first lets talk about pneumonia. In general what is pneumonia?
1. Alveoli fill with fluid, hindering oxygenation
2. Infection of the lung --acute or chronic
3. 1/6th of all US deaths
4. Fever, cough, SOB and CP
Review: what are the stages of bacterial pneumonia?
Acute congestion: exudate rich in fibrin, RBC and PMNs in alveolar lumen
Red hepatization: more PMNs controlled infection
Grey Hepatization: congestion and fibrin disappear, PMNs replaced by macrophages
Resolution: 10-14 days complete
(not that lobar will resolve with or without abx just might take longer)
What are some complications in bacterial pneumonia?
Organization (Scar tissue)
Recurrent pneumonias, bronchiolitis obliterans
Typical pneumonia involves the alveolar spaces and includes lobar and lobular (Bronchopneumonia), what are some features of lobar pneumonia?
---complete resolution (with or without abx)
--spreads via the pores of kohn (Architecture stays intact, confluent consolidation of entire lobe and bronchi are not involved)
what are some features of lobular or bronchopneumonia
Lobular: Patchy and bronchi & bronchioles are damaged
--Klebsiella: alcoholics, diabetics --- aspiration (red currant jelly sputum)
---Pseudomonas: CF, ventilators, burns, lung abscesses and cavitations
NOTE: Klebsiella and Pseudomonas both cause necrotizing pneumonia because they are angio invasive --- endothelial damage
--S. aureus: following viral infection (like flu)
--Legionella: contaminated H2O and ACs, become septic very fast GI and pulmonary and liver (hep) and Kidney (oliguria) and neuro (because of SIAHD --- retain excess water and retain sodium so hyponatremia)
What are complications of bronchopneumonia?
Septicemia --- DIC and pleural thickening
In community acquired acute pneumonia, what are the symptoms and associated bacterial agents?
High fever, shaking, chills, pleuritic chest pain
1. S. pneumonia: most common, lower lobe or right middle lobe. Underlying chronic diseases: CHF, COPD, DM. patient with decreased splenic function -- give vaccine
2. H. Influenza: most common cause of COPD exacerbation. Underlying: COPD, CF and Bronchiectasis
Capsule B present: target for vaccine
3. Moraxella Catarrhalis: 2nd most common cause of COPD exacerbation (old ppl)
4. S. Aureus: most likely to cause abscesses after a viral resp illness (measles in kids and flu in adults) . IVDA = right sided endocarditis.
5. K. pneumonia: extensive destruction: malnourished, chronic alcoholics and DM. Red currant jelly
6. P. aeruginosa: progressive necrotizing pneumonia in CF and burn patients
7. L. pneumophilia: prevents phagosome fusion: aquatic environments
In community acquired atypical pneumonia (aka walking pneumonia) this involves the interstitium, what is the etiology?
Obligate intracellular bacteria:
most common= mycoplasma
Chlamydia (psittaci (in parrots) and pneumonia) and Coxiella Burnetti (from sheep; no rash in Q fever)
Virus: CMV and Parainfluenza
What is the hematological complication of mycoplasma?
Cold autoimmune hemolytic anemia (Happens in cold temps in tips of finger and toes)
what is the pathogenesis for community acquired atypical pneumonia?
Alveolar spaces are free empty, inflammation in alveolar wall (Thickened septae) ---- cough is dry
What is the presentation for community acquired atypical pneumonia?
mild symptoms in the beginning (dry cough and low grade fever)
Pneumocystis Jirovecii, is a fungal cause to community acquired atypical pneumonia, however how is the histology different?
Alveolar spaces are filled with this acellular cotton candy exudate (so no production of mucus because the exudate is so thick)
What would you find on examination, CXR and serology for a patient with community acquired pneumonia?
No dullness on percussion --- alveolar spaces are empty
Worse than what xray shows
Xray: interstitial infiltrates; ground glass opacity
Serology: IgM and IgG (most accurate) with antibody titers if bacteria
If viral PCR
If p. jirovecii: then bronchoalveolar lavage
What is a complication of ARDS?
Hyaline membrane on autopsy
(damage to type II pneumocysts because the organism is right there is the interstitium
Does atypical pneumonia respond to abx?
Yeh if its a bacterial cause it will