Restrictive Pulmonary Disorders Flashcards
List the red flags for chronic cough in adults
Abnormal findings on chest exam or imaging
Dysphagia
Hoarseness
Hemoptysis
New or worsening cough in > 45 y/o smoker
Prominent dyspnea at rest or at night
Vomiting
Weight loss
Chronic cough is > ____ weeks in adults, _____ weeks in kids
> 8 weeks in adults, 4 weeks in kids
List the CC and OLDCARTS for ILD (interstitial lung disease, especially IPF)
CC: cough, dyspnea
Onset, duration, timing: insidious, chronic, persistent, progressive. May be remote from environmental exposure
Location: lungs, may have systemic involvement depending on etiology
Character: progressive of dyspnea & non-productive cough
Alleviate: initially rest
Aggravate: activity, environmental pollutants
Radiation: hypoxia
Associated Symptoms: depends on cause, associated connective tissue disorders (sarcoid, coal dust, silicosis, etc.), medications (bleomycin, methotrexate, amiodarone)
Give examples of interstitial/ restrictive lung diseases
1) Interstitial Pulmonary Fibrosis
2) Pneumoconiosis (more restrictive)
a) Coal miner’s lung
b) Silicosis (sandstone)
c) Asbestosis
3) Sarcoidosis
-(more restrictive)
Idiopathic pulmonary fibrosis (IPF):
1) What is it?
2) How common? Who is it found in?
1) Specific form of chronic, progressing interstitial (restrictive) lung disease (ILD)
2) Rare; incidence increases with age & somewhat more common in men
List 4 risk factors for IPF (Idiopathic pulmonary fibrosis)
1) Age: >55 y/o
2) Male
3) H/o smoking
4) Occupational exposure
Idiopathic Pulmonary fibrosis (IPF)History
1) What is strongly assoc. with it?
2) What are some comorbid conditions?
3) What are some occupational exposures? What professions?
4) What else can be linked?
1) Smoking
2) GERD, hiatal hernia, pulmonary malignancy, CAD, OSA, obesity, COPD, and pulmonary HTN
3) Metal dusts, wood dusts, and aerosolized organic antigens (molds, bacteria, bird antigens): farming, aviary, hair dressing, stone cutting/polishing
4) Some medications
Describe the Sx of IPF
Non-specific:
1) DOE and non-productive cough
2) DOE progresses to resting dyspnea
3) Fatigue and difficulty sleeping
What are the 2 key signs you should focus on when looking for IPF in an exam? How common is each?
1) Rales: Inspiratory crackles (Velcro crackles) heard posterolateral bases: 90%
2) Clubbing late stages: 25-50%
IPF:
1) What should make you think of another Dx?
2) What are 2 sequalae?
1) Rhonchi (squeaks and wheezes)
2) PH and right HF (Cor Pulmonale)
-Rare pneumothorax
IPF diagnostic challenges:
1) What is common?
2) What 3 things is it often misdiagnosed as?
3) What are the Sx like?
4) What are the biomarkers?
1) Misdiagnosis/delayed diagnosis common
2) Asthma, pneumonia, or bronchitis
3) Symptoms (DOE, dry cough) are nonspecific and clinically similar to other more common pulmonary diseases
4) No biomarkers available
IPF:
1) What kind of diagnosis is this?
2) Is there a single clinical factor that indicates it?
3) What types of consult(s) should be done?
4) What is the role of PCPs in this Dx?
1) Diagnosis of exclusion
2) No one clinical factor indicates IPF
3) Multidisciplinary consultation: primary care, pulmonologist, radiologist, & pathologist
4) Early suspicion & appropriate r/o testing and referral
True or false: There is no one clinical factor that indicates IPF
True
When to consider IPF:
1) What should Hx tell you?
2) What are the primary Sx?
1) Rare, but more often occurs in men over 50 that smoke of h/o of smoking
2) Unexplained chronic DOE, chronic non-productive cough
-Bibasilar inspiratory “Velcro” crackles (rales), and finger clubbing
A pt has an unexplained chronic DOE and cough and you suspect IPF:
1) What pattern would you expect to see on PFT?
2) What is the imaging of choice? What will you see?
1) Restrictive pattern
2) High resolution chest CT; honeycombing
Describe a diagnostic w/u and its findings for unexplained chronic DOE and cough if you suspect IPF
1) PFT: Restrictive pattern
-Decreased FVC
-Normal to mildly low FEV1
-Normal to increased FEV1/FVC ratio
-Decreased DLCO
2) CXR: interstitial pattern
3) High resolution chest CT (imaging of choice): Honeycombing + subpleural and bibasilar opacities
IPF (Idiopathic Pulmonary Fibrosis):
1) What are the 3 goals of Tx?
2) What are some Txs?
3) What is the goal SaO2?
4) What is the definitive Tx?
1) Smoking cessation
2) -Vaccinations (early pneumonia vax, annual flu + COVID, TDAP when due, RSV >60)
-Supplemental oxygen (home oxygen)
-Antifibrotic drugs and pulmonary rehabilitation
-Manage comorbidities
3) >/= 88%
4) Refer to transplant center
1) List 2 antifibrotic drugs for IPF
2) What do both do?
1) Nintedanib and Pirfenidone
2) Inhibit fibrous tissue production
BLUF for IPF
1) IPF most common _______________, diagnosis is frequently missed (you have to suspect it to consider it in DDX)
2) What else may be seen?
3) What is the cornerstone for diagnosis?
4) What are common sequalae?
5) What is considered early in course of disease?
6) Tx?
1) Idiopathic ILD
2) DOE and chronic dry cough
3) HRCT +/- histopath
4) PH (group 3) and Cor Pulmonale
5) Referral
6) Antifibrotics Rx and lung transplant
Do not need to memorize
For each type of pneumoconiosis, list the profession(s) it may be found in:
1) Black Lung Disease
2) Brown lung
3) Berylliosis
4) Silicosis
5) Siderosis
1) Coal miners lung
2) Cotton industry from farmers to manufacturing
3) Aerospace, fluorescent bulbs
4) Sand related
5) Arc welding
Do not need to memorize
For each type of pneumoconiosis, list the profession(s) it may be found in:
1) Stannosis
2) Asbestosis
3) Diacetyl
1) Tin welding
2) Ships, demolition (insulation in ships and older buildings)
3) Compound used to give movie popcorn its buttery flavor, inhaled fumes = popcorn lung
ILD (interstitial lung disease) Pneumoconiosis:
1) What is it?
2) What causes it?
3) Onset?
4) What does it lead to?
1) Specific cause of interstitial lung disease (ILD)
2) Occupational exposure from inhalation of dust and other substances
3) Insidious onset, symptoms often remote from exposure
4) Chronic inflammation, massive fibrosis, and scar tissue formation
Pneumoconiosis pathophysiology:
1) Describe the physical effects
2) Complicated pneumoconiosis results in progressive massive fibrosis (PMF); give 3 examples
1) Simple pneumoconiosis causes a small amount of scar tissue.
X-ray = diffuse nodules
2) Interstitial lung disease.
Bronchogenic carcinoma
Mesothelioma in asbestosis
Pneumoconiosis in coal workers:
1) What causes it?
2) What does it look like? Sx?
3) DDX?
4) Why do the Sx occur?
1) Inhalation of coal dust
2) Nodular opacities in upper lung fields; initially asymptomatic
3) MTB
4) Progressive massive pulmonary fibrosis in complicated forms noted in upper lung zones
Coal miner’s lung CC + OLDCARTS
CC: chronic cough
Onset, Duration, Timing: chronic, insidious, progressive, remote from exposure
Location: lungs
Character: cough, black sputum, dyspnea
Aggravating/Alleviating: smoking, RTI/none
Radiation: progress to PH, RHF ….
Associated symptoms: progressive PH, hypoxia, and RHF
Coal miner’s lung:
1) What will you hear on exam?
2) What 2 things are diagnostic? Explain
3) What is a complication?
1) Exam: Velcro crackles
2) PFT: restrictive, decreased DLCO
-CXR: nodular opacities in upper lobes
3) Caplan syndrome = RA + pneumoconiosis manifesting with pulmonary nodules
What is silicosis pneumoconiosis?
Rock mining, sand blasting, or masonry, stone cutting – inhalation of silicone dioxide particles … fibrosis
Give the OLDCARTS for silicosis
CC: cough
Onset, Duration, Timing – may be acute or chronic, persistent, progressive, remote from exposure if chronic
Location – lungs
Character – chronic cough
A/A – smoking
Radiation – associated with ILD complications … PH, RHF
Associated symptoms
Acute - fever, fatigue, weight loss, pleuritic CP
Chronic – dyspnea, pleuritic CP
Silicosis PE:
1) What will be heard on exam?
2) How is it diagnosed?
3) What is a complication?
1) Adventitious BS
2) CXR - Eggshell calcification of hilar nodes
PFT - ?
3) Increased risk of TB and progresses to massive pulmonary fibrosis
Pneumoconiosis asbestosis:
1) What causes it?
2) Risk factors/Industries?
3) Sx?
4) What will you hear on lung auscultation?
1) Inhale asbestos particles; inflammation attacks macrophages and fibroblast leading to fibrosis
2) Mining, construction workers, shipbuilding, pipe fitters, asbestos mitigation in older buildings
3) Initially asymptomatic - latent period between exposure and clinical onset
Symptomatic: DOE
4) Bibasilar adventitious sounds – rales/crackles
Berylliosis:
1) What is it?
2) What occupations are at risk?
3) What will be on CXR?
4) How do you Tx?
1) Type of metallic poisoning
2) High tech fields, aviation, nuclear power, ceramics, foundries
3) Diffuse infiltrates and hilar adenopathy
4) Chronic steroids
Pneumoconiosis general management/prevention:
1) How is it managed (3 things)
2) What are 4 ways to prevent it?
1) Pulmonary rehab
Supplemental oxygen
Steroids and bronchodilators
2) Exposure precautions
Smoking cessation
Vaccination
Industrial hygiene
Sarcoidosis: Fun Facts
1) What is a key concept? What is the etiology?
2) What organ(s) do(es) it affect?
3) What are the risk factors?
1) Multisystemic granulomatous inflammatory disease characterized by noncaseating granulomas
-Etiology unknown; idiopathic inflammatory response, recruitment of T lymphocytes & cytokines leading to inflammation and granuloma formation
2) Predominantly pulmonary but any organ
3) Most commonly affects middle aged black women
All ages but rare in kids; Women (2x) > men
Annual incidence ~ 8-11/100,000
Describe the disease course of Sarcoidosis
Variable:
~½ of pts experience spontaneous remission after ~2 yrs
~1/3 of pts experience sarcoid progression: radiographic or pulmonary function
(Overall mortality like general population)
Give the CC for and the onset, duration, timing, location, and character of Sarcoidosis
CC: cough & dyspnea
Onset: Insidious to acute (see Lofgren’s Syndrome next slide)
Duration: chronic, about ½ spontaneous remission after 2 years
Timing: symptoms wax and wanes
Location: pulmonary & lymph nodes, MSK, skin … but any organ
Character: Pulmonary – dyspnea, dry cough, and pleuritic chest “pain”
~ 50% of patient asymptomatic with bilateral hilar adenopathy found incidentally on CXR
Give the alleviating + aggrevating factors, radiation, and assoc. Sx for sarcoidosis (OLDCARTS pt 2)
Alleviating: many asymptomatic, rest
Aggravating: exertion
Radiation: symptoms Range from asymptomatic to acute onset, mainly pulmonary but MSK, skin and others.
Associated Symptoms: constitutional ~ 1/3 may report fatigue, unintentional weight loss, & fever,
~1/3 develop skin lesion: red, brown papules and plaques (erythema nodosum), cutaneous granulomas within tattoos
Some with anterior uveitis, RUQ pain and jaundice
Describe the signs of sarcoidosis on an exam
1) VSS
2) Pulmonary
-Normal to adventitious sounds: wheezing, rales
3) Lymphadenopathy
4) Extrapulmonary
-Skin lesions: erythema nodosum, Lupus pernio on ala of nose, anterior uveitis
-Eye: DDX anterior uveitis
-GI: RUQ pain, jaundice
What structures are involved in anterior uveitis? Symptoms? Exam findings?
(This question is on the slide)
1) Inflammation of the iris and the front part of the ciliary body.
2) Sx include pain, redness, light sensitivity (photophobia), and blurred vision.
3) Exam findings include: cells and flare in the anterior chamber, circumlimbal injection, and potentially synechiae and keratic precipitates.
Answer from Google AI
1) Lupus pernio: What is it? What is it associated with?
2) Does lupus pernio resolve spontaneously? What does it involve?
3) Uveoparotid fever (Heerfordt syndrome) is characterized by what?
1) Cutaneous sarcoidosis; assoc. w. poor prognosis and lung involvement
2) Rarely resolves spontaneously
Extensive involvement with nasal cavity and maxillary sinus leading to nasal obstruction and disfigurement; pulmonary fibrosis
3) Uveitis, parotitis, fever, and occasional CN VII palsy (DDX Bell Palsy)
Describe Lofgren’s Syndrome
1) ACUTE Onset sarcoid characterized by erythema nodosum, uveitis, fevers, polyarthritis, and bilateral hilar lymphadenopathy
2) Self limiting, favorable prognosis
How do you Dx a Sarcoid?
1) Has a broad DDX with multiple organs affected; diagnostic challenge bc also no diagnostic standard for testing & asymptomatic state
2) Multiple organs: clinical overlap for pulmonary and extrapulmonary
3) Clinical presentation: high index of suspicion and radiographic findings
Sarcoidosis: What are the baseline labs to screen for organ system involvement?
1) CBC
2) CMP – renal and liver, electrolytes, & calcium (hypercalcemia secondary increased circulating 1,25-dihydroxyvitamin D (calcitriol) from macrophages within non-caseating granulomas)
3) Urinalysis and hypercalciuria
Sarcoidosis labs:
1) Additional may include what?
2) ___________ levels are elevated in ~ 50 – 60 % of patients but does not correlate radiographic disease severity and lacks specificity – not routinely recommended
1) HIV, TB, fungal
2) Serum ACE
There are 4 radiographic stages of ___________ ___________, ranging from basically nonexistent (just some bilat + mediastinal lymphadenopathy) to hilar distortion and fibrosis
pulmonary sarcoidosis
Sarcoidosis: List and describe the 3 aspects of pathologic diagnosis
1) Noncaseating granulomas on lung tissue bx
2) Schaumann bodies: calcium and protein inclusions within the Langhans giant cells of the granulomas
3) Asteroid bodies: stellate inclusions within giant cells of granulomas
Non-caseating granulomas consisting of aggregates of epithelioid histiocytes, giant cells, & mature macrophages may be seen in what condition?
Sarcoidosis
Sarcoidosis:
1) How severe is this? Where is it in the body?
2) What is the common outcome?
1) Asymptomatic or mild disease limited to hilar adenopathy
2) Observed without treatment as spontaneous remission is common
Oral corticosteroids for sarcoidosis:
1) Are these your first choice Tx?
2) What does a low dose long term do? What is key to prescribing?
3) What is the optimal dose?
4) How may you taper? When?
5) When do you know it doesn’t work? And what is not uncommon with this med?
1) Yes; first line treatment
2) Low dose long term - improves chest radiographic appearance, symptoms over 3-24 months
-Balance disease response with side effects
3) Usually ~ 20-40 mg / day prednisone ~ 4-6 weeks, then assess for clinical response & disease progression via symptoms, CXR, & PFT
4) Responding to steroids may taper dose slowly to 5-10 mg QD or EOD x 12 months
5) If not by 3 months, unlikely to respond
-Relapse not uncommon after in 1st 2-6 months after corticosteroids stopped
Wegener’s granulomatosis:
1) What is it?
2) Etiology? Prognosis?
3) Sx?
4) Dx?
1) Necrotizing granulomatous vasculitis affecting upper and lower respiratory tract with focal segmental glomerulonephritis (Lung and Kidneys)
2) ?? Etiology, young or middle-aged males
- Early Dx can lead to full recovery, can be fatal without treatment
4) Sx: cough, chest pain, dyspnea, malaise, blood in urine
5) Dx: ESR, anti-neutrophil cytoplasmic bodies, CBC, UA
Corticosteroids are mainstay of treatment
Azathioprine, MTX, others
Consult pulmonary and nephrology
