Restrictive Disorders highlights Flashcards
What does DOE stand for?
Dyspnea on exertion
IPF and sarcoidosis are 2 of the ____________ common causes of chronic cough in adults.
least
Interstitial Lung Disease (ILD) =
progressive __________________ (DOE) and persistent __[productive/ non-productive]__ dry, hacking cough
dyspnea; non-productive
Interstitial Lung Disease (ILD)
1) A non-productive, _____________ __________ cough is characteristic.
2) List the 2 constitutional Sx.
3) What systemic Sx may accompany it?
1) dry, hacking cough
2) fatigue and sleep disorder
3) CP, fever, rash, weight loss, myalgia, and arthralgia
CP, fever, rash, weight loss, myalgia, and arthralgia may accompany what?
ILD (Interstitial Lung Disease)
ILD (Interstitial Lung Disease):
1) What Hx is a risk?
2) Is CXR normal?
3) Is spirometry normal?
1) Occupational or environmental dust exposure
2) Abnormal CXR
3) Abnormal; especially restrictive pattern
What do you look at on spirometry results to Dx restrictive lung diseases?
FVC
Idiopathic pulmonary fibrosis (IPF):
1) What is the mechanism occurring here? What are its 2 effects?
2) What is decreased on spirometry?
Fibrosis & scarring of lung parenchyma (interstitium)
= Decreased lung compliance
= Restrictive lung disease
2) FVC decreased
What should make you think that a pt does NOT have IPF?
Rhonchi (squeaks and wheezes)
What type of drugs can you Tx IPF with?
Antifibrotic
IPF BLUF:
1) In practice as a PCP, what do you need to do regarding this condition?
2) How does it present?
3) What is the cornerstone for Dx?
1) You have to suspect it to consider it in DDX
2) DOE and chronic dry cough
3) HRCT +/- histopath
IPF BLUF: What 5 things should primary care consider to manage IPF in all pts?
1) Home oxygen supplementation
2) Pulmonary rehab
3) Anti-reflux Rx
4) Smoking cessation
5) Vaccination
What can cause ILD (interstitial lung disease) pneumoconiosis?
Inhalation of dust and other substances (occupational)
Complicated pneumoconiosis results in what?
progressive massive fibrosis (PMF)
Pneumoconiosis in coal workers:
1) What may you see on CXR?
2) Why?
1) Nodular opacities in upper lung
2) Massive pulmonary fibrosis in upper lung zones
1) What is a common CC for coal miner’s lung?
2) What is a common CC for silicosis?
1) Chronic cough
2) Cough
What is a complication of Coal miner’s lung? Why does it occur?
Caplan syndrome = RA + pneumoconiosis manifesting with pulmonary nodules
Need to know this
1) What will be seen on CXR of silicosis?
2) What can silicosis progress to?
1) Eggshell calcification of hilar nodes
2) Massive pulmonary fibrosis
Asbestosis:
1) What is seen on CXR?
2) What is another unique characteristic?
1) Reticular opacities, especially at bases of lungs, +/- honeycombing
2) Calcified plaques
Asbestosis:
1) What is seen on HRCT chest?
2) What abt PFT?
3) What is one complication of asbestosis?
1) Parenchymal fibrosis and pleural plaques
2) Restrictive pattern
3) Mesothelioma
What does berylliosis look like on CXR?
Diffuse infiltrates and hilar adenopathy
Sarcoidosis:
1) What is its hallmark?
2) What is the etiology? Explain
1) Noncaseating granulomas
2) Unknown; idiopathic inflammatory response, recruitment of T lymphocytes & cytokines leading to inflammation and granuloma formation
Sarcoidosis:
1) Where is it located?
2) Risk factors?
1) Predominantly pulmonary, but any organ
2) Most commonly affects middle aged black women
-All ages but rare in kids; women (2x) > men
Extra pulmonary manifestations of sarcoid are possible, but ________% are in the lungs
90%
How long do sarcoidosis Sx last?
May resolve in 2-3 years or may persist
A pt presents with acute cough and dyspnea. What should be high on your differential list?
need to know
Sarcoidosis
If you see some skin lesions in a young adult female with a chronic cough and some dyspnea and adenopathy, what should you start to think abt?
Sarcoidosis
What is Erythema nodosum? Describe
SQ skin inflammation.
-Results in reddish, painful, tender lumps commonly located in the front of the legs below the knees
Pathognomonic syndromes that do not require confirmatory tissue biopsy: List 3
1) Lupus pernio
2) ACUTE Onset sarcoid
3) Uveoparotid fever
Pathognomonic syndromes: Give an example of a cutaneous sarcoidosis assoc. w. poor prognosis and lung involvement
Lupus pernio
Give an example of an acute onset sarcoid
“put a star by this bc it’s different”
Lofgren’s Syndrome
Sarcoidosis labs: What may you see on a CMP?
Hypercalcemia; secondary to increased circulating 1,25-dihydroxyvitamin D (calcitriol)
Sarcoidosis labs
1) What might you see on urinalysis?
2) Serum ___________ levels are elevated in ~50–60% of patients but does not correlate radiographic disease severity and lacks specificity – not routinely recommended
1) Hypercalciuria
2) ACE
Pulmonary sarcoidosis Dx:
1) What will you see on CXR?
2) What abt tissue biopsy?
1) Bilateral hilar lymphadenopathy
2) Noncaseating granulomas
List 3 things that help w. Pathologic diagnosis of sarcoidosis
Noncaseating granulomas (fix this card)
What group of meds is the first line for sarcoidosis Tx?
Oral corticosteroids
Wegener’s granulomatosis:
Necrotizing granulomatous vasculitis affecting upper and lower _________________ with focal segmental _________________
respiratory tract; glomerulonephritis
(lung and kidneys)
Wegener’s granulomatosis: _________________ are mainstay of treatment
Corticosteroids
