Module 7 pulmonary Flashcards
The next restrictive lung disease is called nonspecific interstitial pneumonia (NSIP), what are the features of this disease?
- Idiopathic
- Better prognosis
- Younger patients
- Uniform fibrosis process (Chicken wire)
- Two variants: cellular (more inflammation) and fibrosing (more fibrosis)
- 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?
- Idiopathic
- Cough, SOB and recover either spontaneously or with steroids
- CXR: patchy peribronchial/sub-pleural consolidation
- 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?
- group of diseases that affect collagen
- Disease of which inflammation or weakness in collagen tends to occur
- Associated with collagen and blood vessel abnormalities; often autoimmune
- Can affect joints,skin ,blood vessels or other organs
What are examples of CVD that have pulmonary manifestations?
- Sjogren’s sydrome
- Lupus
- RA
- Systemic Sclerosis
- Dermatomyositis- polymyositis
What are some pathologic patterns with CVD?
- Usual interstitial pneumonia (UIP): pattern of fibrosis
- non specific interstitial pneumonia (NSIP)
- Organizing pneumonia
- Bronchiolitis +/- fibrosis
- 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?
- Idiopathic
- Expansion of the interstitium by sheets of lymphoid cells
- Associated with: CT diseases, Autoimmune diseases, HIV
- May transform to lymphoma
What are some other cause of interstitial fibrosis?
Anticancer drugs Radiation pneumonitis Intravenous heroin Autoimmune disorders 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?
Sarcoidosis
HP: hypersensitivity Pneumonia
Sarcoidosis is a restrictive lung disease that affects what part of the population?
Young female
non smokers
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?
Asymptomatic
- –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)
Elevated calcium
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?
- Cor Pulmonale
- Contraction atelectasis
- Facial nerve paralysis
- Restrictive cardiomyopathy
- Renal Failure
- Liver cirrhosis and HCC
What is the treatment for sarcoidosis?
Steroids Lung transplant (problem is that high chance of recurrence after transplant)
Review: What other diseases do non caseating granulomas?
- Sarcoidosis
- Berryliosis
- Crohns
- Leprosy
- 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?
- Airway centered process
- Chronic inflammatory process
- Organizing pneumonia
- 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?
- Seen in smokers in their 40s and 50s
- Accumulation of smoker’s macrophages within the lumen of respiratory bronchioles
- Associated with peribronchiolar fibrosis
- Responds to steroids and smoking cessation
