Restrictive Lung Disease Flashcards
Coal Workers Pneumoconiosis is caused by
- Inhalation of coal mine dust
- Coal is inhaled into alveolus and engulfed by macrophages.
- Macrophages release reactive O2 species, triggering release of cytokines (interleukins, tumor necrosis factor) responsible for inflammation and fibrosis.
- This leads to the development of coal macules.
- These enlarge to coal nodules
Pneumoconiosis Symptoms
Cough, sputum production, SHOB/DOE
What is found on CXR for pneumoconiosis?
Small nodules that predominate the upper lungs
Pneumoconiosis treatment
Supportive- O2 supplementation and rehab
Silicosis is caused by
prolonged inhalation and retention of silica particles
What is the hallmark lesion of silicosis?
- Silicotic nodule
- Small, rounded opacities in upper and mid lungs, 3-5 mm
- 1st involve hilar lymph nodes and may calcify or erode the airway.
- The lung parenchyma is subsequently involved, usually upper lobes.
Silicosis symptoms
Tachypnea, fine crackles, SHOB
Diagnosis of silicosis
need open lung biopsy for definitive diagnosis
Silicosis treatment
avoid exposure, stop smoking, lavage if needed
What is increased in silicosis patients?
Increase in TB
Asbestosis characteristics
- Interstitial fibrosis and pleural thickening, similar to Silicosis
- Can be seen 15-20 years after lengthy exposure
- Exposure via renovation/destruction of old buildings, ship building
Asbestosis symptoms
DOE, Non-productive cough
What is found on asbestosis CXR?
Fibrosis, pleural plaques.
*Make sure to get CT to evaluate plaque. The fewer the opacities, the better the prognosis.
Asbestosis treatment
- Oxygen if needed and pulmonary rehab
- Must stop smoking.
Risk of lung cancer in nonsmoking asbestosis worker, 5.2 –> risk of lung cancer in smoking asbestosis worker, risk increases 53 fold
Risks in asbestosis patients
increased risk of developing Mesothelioma of the pleura, bronchogenic carcinoma, and TB
Sarcoidosis definition
- Multisystem granulomatous disorder of unknown etiology
- Can effect: lungs (90%), lymph nodes, eyes, skin, liver, spleen, heart, joints, and nervous system
Sarcoidosis is most commonly seen in…
young African American females, 20-40 y/o, and Northern Europeans
A sarcoidosis pathology report will read…
“non caseating granulomatous inflammation”
Sarcoidosis symptoms
Cough, SHOB, fever, wt loss, sweats, or nothing
What will be seen on CXR for sarcoidosis?
- Bilateral hilar adenopathy, interstitial infiltrates or both
- Get a chest CT for closer evaluation
Sarcoidosis diagnosis
Bases on pathology from a TBB (transbronchial biopsy) or FNA (fine needle aspiration)
Sarcoidosis treatment
- is based on symptoms
- 90% respond to corticosteroids
- 50-60% have spontaneous remissions
What baseline labs should you draw for sarcoidosis?
Complete PFTs, BNP, ECHO, 24 hour urine Ca, Bilateral hand Xrays, ACE level, and refer to Ophthalmology
-Follow pt with serial PFTs and CXR or HRCT
What joint is affected first in sarcoidosis?
The wrist joint
Nonspecific Interstitial Pneumonitis (NSIP) characteristics
- Younger patients, 40-50 y/o
- Onset usually gradual, 6 months to 3 years before dx
NSIP symptoms
cough, wt loss, breathlessness
NSIP physical exam
diffuse fine basilar crackles
What will be seen on CT for NSIP?
- diffuse, symmetrical GG changes w/wo traction bronchiectasis
- confluent and homogenous pattern.
- Honeycombing is rarely seen
NSIP treatment
corticosteroids
Cryptogenic Organizing Pneumonia (COP) characteristics
- Organizing pneumonias
- Most common in nonsmokers, roughly 50-60 y/o.
- Males = Females
- Short duration, <3 month history of SHOB, dry cough, may have malaise, wt loss, myalgias
- Chest infection that it slow to resolve after several rounds of antibiotics
COP physical exam
may be normal or hear crackles
What will be seen on imaging studies in COP?
- CXR: patchy consolidations or even a solitary mass
- HRCT: areas of consolidation with air bronchogram, GGO (these areas can migrate spontaneously)
Definitive diagnosis of COP
TBB (transbronchial biopsy) or open lung biopsy
COP treatment
long steroid taper over months, may need 6-12 months of therapy.
*Follow with CT
Idiopathic Pulmonary Fibrosis
- Diffuse lung disease of unknown etiology
- Involves the interstitium of the lung the fibrous framework of the lung
- Onset of 9-24 months
- MC in males
- Mean age is 65
Idiopathic Pulmonary Fibrosis exam findings
fine basal and late inspiratory crackles
What would you find on imaging for idiopathic pulmonary fibrosis?
CXR: peripheral and basal reticular shadowing
HRCT: bilateral, peripheral, and subpleural reticulation with honeycombing and traction bronchiectasis
What is the prognosis for idiopathic pulmonary fibrosis?
- Very poor prognosis
- Stable vs. decline over years vs. decline rapidly
- Median survival is 2.5 to 3.5 years at time of diagnosis
- Prior to 11/2014 there were no treatment options other than supplemental oxygen.
- -2 new medication to help slow down progression –> Ofev and Esbriet
