Term
| restrictive pulmonary disease: AKA |
|
Definition
| interstitial lung disease |
|
|
Term
| unlike obstructive lung diseases, restricted lung diseases are assoc w: what kind of lung volumes? |
|
Definition
| reduced; can't get a good breath into the lungs |
|
|
Term
| given that there are reducede lung volumes which lung capacities are affected and how? |
|
Definition
| decreased: TLC, VC, FRC but with preserved air flow. |
|
|
Term
| there are 2 groups of restricive lung diseases: |
|
Definition
1. intrinsic; diseases of the parenchyma
2. extrinsic: outside the parenchyma |
|
|
Term
| pathophysiology of intrinsic lung disease: |
|
Definition
| disease cause inflammation &/or scaring of the lung tissue (intersittial) OR air spaces fill with exudate (pneumonitis) |
|
|
Term
| define: interstitial lung disease, which group of restrictive lung disease does it fall into? |
|
Definition
| interstitial: disease that causes inflammation &/or scaring of the lung tissue which would fall into the intrinsic group of restrictive disease bc it involves the parenchyma |
|
|
Term
|
Definition
| filling of air spaces with exudate and debris; also considered an interstitial lung disease |
|
|
Term
| which structures may be involved in the extrinsic restrictive pulmonary disorders? give two examples of anatomical disorders leading to restrictive pulmonary disorder? |
|
Definition
chest wall, pleura, respiratory muscles, neuromuscular disorders
ex: kiphosis, lordosis |
|
|
Term
| 3 classifications of interstitial lung disease: |
|
Definition
1. idiopathic interstitial pneumonia
2. hypersensativity pneumonitis
3. interstitial lung disease assoc w collagen vascular disease |
|
|
Term
| 2 diseases under the class of idiopathic interstitial pneumonia: |
|
Definition
1. idiopathic fibrosing interstitial pneumonia
2. respiratory bronchiolitis |
|
|
Term
| 2 types of hypersensativity pneumonitis |
|
Definition
1. occupational/environmental
2. drug induced/iatrogenic |
|
|
Term
| 4 ILD assoc w collagen vascular disease: |
|
Definition
1. RA
2. SLE
3. sjogren's syndrome
4. ankylosing spondylitis |
|
|
Term
| which type of restrictive lung disease does sarcoidosis fall? |
|
Definition
|
|
Term
| what is the outcome of breathing at such low lung volumes? |
|
Definition
| V/Q mismatch; arterial hypoxemia> hyperventilation |
|
|
Term
| define: idiopathic fibrosing interstitial pneumonia |
|
Definition
| chronic, progressive fibrosing interstitial lung disease of unknown etiology, characterized by poor prognosis and no proven effective Tx |
|
|
Term
| describe the pathophys of idiopathic fibrosing interstitial pneumonia |
|
Definition
| intra-alveolar inflammation caused first by lymphocytes and plasma cells then come fibroblasts laying down collagen leading to chronic fibrosis of the lung |
|
|
Term
| what is the characteristic radiographic sign in idiopathic fibrosing interstitial pneumonia? |
|
Definition
|
|
Term
| what is the median survival rate post Dx of idiopathic fibrosing interstitial pneumonia? |
|
Definition
|
|
Term
| what are 2 contributing factors of idiopathic fibrosing interstitial pneumonia? |
|
Definition
|
|
Term
| clinical presentation for idiopathic fibrosing interstitial pneumoinia: |
|
Definition
most common Sx = exertional dyspnea then nonproductive cough in the am.
also: finger clubbing, pulm HTN, end of inspiration lung crackles @ base of lungs |
|
|
Term
| which lab is elevated in 50% of pts with idiopathic fibrosing interstitial pneumomia? |
|
Definition
|
|
Term
| CXR for idiopathic fibrosing interstitial pneumonia shows: |
|
Definition
| BILATERAL infiltrates @ periphery and bases of lungs |
|
|
Term
| what other imaging may be helpful in idopathic fibrosing interstitial pneumonia? |
|
Definition
| HRCT- High Resolution Computed Tomography |
|
|
Term
| what is seen in HRCT in idiopathic fibrosing interstitial pneumonia |
|
Definition
| reticular opacities, honeycombing and architectural distortion |
|
|
Term
| what is an independant predictor of mortality seen in HRCT of an idiopathic fibrosing interstitial pneumoinia patient? |
|
Definition
| higher extent of honeycombing |
|
|
Term
| in idiopathic fibrosing interstitial pneumonia, what does the blood gas look like? why? |
|
Definition
| aPO2 & aPCO2 = decresed, pH = normal; PO2 decreased bc of the increased distance of diffusion from fibrosis, CO2 = decreased bc of hyperventilation, pH = normal bc its a chronic cond and is compensated from alkilosis |
|
|
Term
| 4 major criteria for idiopathic fibrosing interstitial pneumonia: |
|
Definition
1. exclusion of other known causes of interstitial lung disease.
2. restrictive pattern on PFT/impared gas exchange
3. bilateral basilar reticular opacities on HRCT
4. Bx or BAL show no features to support alternative Dx |
|
|
Term
| 4 minor criteria for idiopathic fibrosing interstitial pneumonia: |
|
Definition
1. >50
2. unexplained dypnea upon exertion
3. illness >3mo
4. bibasilar inspiratory crackles |
|
|
Term
| what is the standard therapy for idiopathic fibrosing interstitial pneumonia: |
|
Definition
| Corticosteroid: prednisone & O2 therapy |
|
|
Term
| prednisone therapy is combined with what for idiopathic fibrosing interstitial pneumonia? |
|
Definition
| immunosupressive therapy; azathioprine |
|
|
Term
| who is most likely to be Dx w IFIP? |
|
Definition
|
|
Term
| Sarcoidosis is considred what type of restrictive lung disease? |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| multisystem granulomatous disease of unknown etiology that predominately affects the lungs(80%) |
|
|
Term
| what plays a central role in the pathophys of sarcoidosis? |
|
Definition
T cells; propogate excessive immune rxn
B cells; immunoglobulin production |
|
|
Term
| 4 clinical presentations of sarcoidosis: |
|
Definition
1. Asymptomatic in 30-60%
2. systemic complaints in 30%
3. Respiratory Sx in 50%
4. Lofgren's sendrome 10-20% |
|
|
Term
| Lofgren syndrome is characterized by: |
|
Definition
| bilateral hilar adenopathy, fever, polyarthalgias |
|
|
Term
| skin of sarcoidosis presentation: |
|
Definition
| lupus pernio, erythema nodosum |
|
|
Term
| physical exam of lungs in sarcoidosis may reveal: |
|
Definition
| crackles or could be normal |
|
|
Term
| what other organs may be involved in sarcoidosis: |
|
Definition
| eyes, liver, heart, kidneys, skin, everything? |
|
|
Term
| which analyte may be elevated in sarcoidosis? |
|
Definition
| calcium; hypercalcemia/hypercalciuria |
|
|
Term
| TEST Q: sarcoidosis tissue Bx reveals: |
|
Definition
|
|
Term
| how would a CXR compare btwn sarcoidosis and IFIP? |
|
Definition
| IFIP is bilateral and basilar infiltrates and sarcoidosis is hilar or mediastinal adenopathy |
|
|
Term
| what are the mainstay of therapy for sarcoidosis? |
|
Definition
corticosteroids: Prednisone
NSAIDs are being used more freq |
|
|
Term
| what is the second line therapy for sarcoidosis: |
|
Definition
| azathioprine; immunosupessant |
|
|
Term
| prognosis of sarcoidosis:3 markers for porr prognosis: |
|
Definition
many resolve spontaneously
1. advanced CXR stage
2. extrapulmonary disease
3. pulm HTN |
|
|
Term
| disease of the lung caused by chronic inhalation of dust: |
|
Definition
|
|
Term
| extrinsic allergic alveolitis = AKA |
|
Definition
| hypersensativity pneumonitis |
|
|
Term
| Hypersensative pneumonitis is caused by an allergic rxn to inhalation of: |
|
Definition
|
|
Term
| epidemiology of hypersensative pneumonitis |
|
Definition
| prevalence and incidence are low; farmers & bird fanciers |
|
|
Term
| clinical manifestations of acute hypersensativity pneumonitis: |
|
Definition
| sudden onset: malaise, NV, cough, dyspnea, chest tightness |
|
|
Term
| subacute hypersensativity pneumonitis: |
|
Definition
| gradual devel. productive cough, dyspnea, fatigue, anorexia |
|
|
Term
| hypersensativity pneumonitis BAL reveals: |
|
Definition
| lymphocytosis; CD4:CD8 = <1 |
|
|
Term
| HRCT of hypersensativity pneumonitis shows: |
|
Definition
| mid to upper central lobar opacities with sign of air trapping |
|
|
Term
| which procedure is required for Dx of hypersensativity pneumonitis? |
|
Definition
| Bx; histology shows poorly formed noncaseating granuloma near terminal bronchioles |
|
|
Term
| hypersensativity pneumonitis Tx: |
|
Definition
| avoid trigger, severe cases = glucocorticoid therapy (prednisone) |
|
|
