Sarcoidosis & Pneumoconiosis Flashcards
Define sarcoidosis
- chronic, multisystem, inflammatory, granulomatous disorder
What is the etiology of sarcoidosis
- unknown
How does sarcoidosis manifest?
- noncaseating granulomas in lungs & intrathoracic LN
What presents with caseating granulomas?
- TB
Define pneumoconiosis
- occupational and environmental restrictive lung disease
What is the pathophysiology of sarcoidosis?
- T cells cause an excessive cellular immune reaction
- leads to central immune system activation
- excessive T cell collection leads to granuloma formation
What is the identifying hallmark of sarcoidosis?
- non-caseating granulomas
What are the MC organs affected by sarcoidosis?
- lungs
- skin
also LN & eyes
What pulmonary findings will present with sarcoidosis?
- usually normal
- crackles
- exertional O2 desat
What are the dermatological findings with sarcoidosis?
- erythema nodosum
- LE maculopapular rash
- lupus pernio (most specific)
- violacious rash on the cheeks or nose
What are the ocular findings of sarcoidosis?
- uveitis
How is sarcoidosis dx’d?
- CXR
- CT
- gallium scan
- bx
- PFTs
What is stage 0 sarcoidosis?
- normal CXR
What is stage I sarcoidosis?
- (B) hilar LAD
What is stage II sarcoidosis?
- (B) hilar LAD
- infiltrates
What is stage III sarcoidosis?
- infiltrates only
What is stage IV sarcoidosis?
- fibrosis
What is the MC abnormality on PFTs
- isolated decrease in DLCO
What else must be tested in sarcoidosis?
- CV, not diagnostic
What is the tx of sarcoidosis for pulmonary dz?
- prednisone
- steroids
What is the tx for extrapulmonary sarcoidosis dz?
- steroids
What is the tx for ocular sarcoidosis dx?
- steroids
When is a lung transplant part of the tx of sarcoid?
- advanced pulmonary fibrosis
Define asbestosis
- diffusing pulmonary interstitial fibrosis occurring in asbestos exposed workers/bystanders/community
Describe the pathology of asbestosis
- inhalation of asbestos fibers leads to inflam with scarring of alveolar-capillary membrane leading to pulm fibrosis & functional deterioration
What is asbestosis directly related to?
- intensity & duration of exposure
When does asbestosis manifest?
- 10-20y s/p exposure
What are the S&S of asbestosis?
- dyspnea on exertion
- chest pain
- productive cough
- concomitant bronchitis or respiratory infx
- rales
What is seen on CXR in asbestosis?
- pleural plaques
- pleural effusion
- irregular, linear opacities in lower lung
What is seen on PFT in asbestosis?
- normal FEV1/FVC ratio
- decreased FC, TLC, and DLCO
What is seen on bx of asbestosis?
- fibrosis
- fibers/asbestos bodies
What is the tx for asbestosis?
- smoking cessation
- tx early S&S PNA
- bronchodilator/steroids/O2
- lung transplant
What are potential complications of asbestosis?
- cor pulmonale
- malignancy
- PAH
- R sided heart failure
What is the primary prevention of asbestosis?
- follow OSHA guidelines
What is the secondary prevention of asbestosis?
- vaccinations
Define Coal Worker’s Pneumoconiosis (CWP)
- chronic, usually occupational acquired, restrictive lung disease from respirable coal dust
What is the pathophysiology of CWP?
- dust laden macrophages accumulate in the alveoli and trigger immune response
- coal macules form discrete areas of fibrosis
What is seen on PE in CWP?
- asx
- chronic bronchitis/COPD sx
- cough, dyspnea, lung function impairment
What test should be done for CWP?
- CXR
- CBC
- PFTs
What is the tx for CWP?
- symptomatic
- O2 for hypoxemia
- serial CXR monitoring
- smoking cessation
- immunizations
- transfer jobs
What are potential complications from CWP?
- progressive massive fibrosis
- Caplan Syndrome
- TB
- CA
Define silicosis
- fibronodular lung dz caused by inhalation of dust containing crystalline dust
How does silicosis present?
- dyspnea
- cough
- weakness
What is the testing for silicosis?
- CXR
- CT
- PFTs
- PPD (mantoux)
What is seen on CXR in silicosis?
- (B) alveolar filling
- ground glass
- small nodules
- eggshell calcifications
What is the tx for silicosis?
- prevent further exposure
- quit smoking
- immunize
- steroids
- lung transplant
- tx active TB & other complications
What is the pathophysiology of chronic beryllium dz?
- Tc –> proinflam cytokines –> non-caseating granulomatous inflam
What are the S&S of chronic beryllium dz?
- dyspnea
- cough
- chest pain
- arthralgia
- fatigue
- wt loss
- inspiratory crackles
- LAD
- rash
- hepatosplenomegaly
How is chronic beryllium dz diagnosed?
- blood/bronchoalveolar lavage (BAL)
- beryllium lymphocyte proliferation test (BeLPT)
- CT
- spirometry
What will be seen on CT in chronic beryllium dz?
- ground glass opacification
What is the tx for chronic beryllium dz?
- refer
- symptomatic tx
- steroids, if they fail methotrexate
- O2
