Diffuse Parenchymal Lung Disease Flashcards
Interstitial lung disease is often times, called __________.
Diffuse parenchymal lung disease (DPLD)
Diffuse parenchymal lung disease (DPLD) all have involvement of _________ on histopathology.
Distal lung parenchyma
How can we classify Diffuse Parenchymal Lung Diseases on histopathology?
- 1. Inflammation and fibrosis
- 2. Granulomatous changes
What histopathologic patterns have interstitial involvement?
- UIP (usual interstitial pneumonitis)
- NSIP (Nonspecific interstitial pneumonitis)
- BOOP (bronchiolitis obliterans organizing pneumonia)/ COP (cryptogenic organizing pneumonia)
What histological pattern is seen with UIP?
Heterogenous involement of lung with different stages of progression of fibrosis.
Usual interstitial pneumonitis is associated with several diseases or exposures including what?
- pneumoconioses
- radiation injury
- end-stage hypersensitivity pneumonitis
- advanced sarcoid
If no underlying process is ID’d, UIP is instead diagnosed as what?
Idiopathic pulmonary fibrosis
- What pattern is seen in non-specific intersitial pneumonitis (NSIP) on histo?
- CT?
- Assx with what diseases?
- Uniform involement of lung parenchyma, with cellular infiltartion or fibrosis.
- Ground glass infiltrates
- Autoimmune CT disorders
What pattern is seen in BOOP/COP on histo?
CT?
Assx with what diseases?
- Small airway bronchiolitis with granulomas + organizing pneumonia
_________ granulomas are typical for sarcoidosis.
________ granulomas are typical for hypersensitivity pneumonitis.
- well-formed non-caseating
- loosely formed granulomas
How are DLPD’s diagnosed?
Thorough hx, PE, lab and imaging studies, open lung biopsy.
What symptoms raise the possibility of DPLD?
- Progressive dyspnea, over months
- Reduced excercise tolerance
- Persistant dry cough
- Idiopathic pulmonary fibrosis most often occurs in patients ______.
-
Interstitial lung disease assx with … occurs in patients ________
- CT disease
- Sarcoidosis
- Lymphangioleimyomatosis
- LCH
- Over 50 YO.
- 20-40 YO
What DPLDs occurs most in females?
Lymphangioleimyomatosis
What DPLDs are most associated with smoking?
- 1. RB-ILD
- 2. Desquamative intersitial pneumonia
- 3. Langerhan cell histiocytosis
If patient has exposure history to birds, hay, mold or other organic material, what DPLDs should we consider?
Hypersensitivity pneumonitis
If patient has occupational exposure, what DPLDs should we consider?
1. Asbestosis
2. Silosis
It patient has acute onset, what DPLDs should we consider?
- 1. Acute Interstitial Pneumonia
- 2. Acute eosinophilic pneumonia
- 3. COP
- 4. Hypersensitivity pneumonitis
- 5. Drug-induced interstiial lung disease
- 6. Diffuse alveolar hemorrhage syndrome
What findings are indicative of sarcoidosis or CT diseases?
- Erythema nodosum
- Uveitis/conjunctivitis
- Arthritis
What findings are indicative of sarcoidosis and Sjrogens syndrome?
Enlargement of lacrimal/salivary gland
Lymphadenonapthy/hepatosplenomegaly is indicative of what DPLD?
Sarcoidosis
Muscle weakness is associated with what DPLD?
- Polymtositis
- Dermatomyositis
Clubbing is indicative of what DPLD?
Idiopathic pulmonary fibrosis.
Hx of someone with suspected DPLD should be focused on what?
Looking for underlying causes
What should docs look for on PE of DPLDs?
- Oxygenation status (desaturation with exertion, which is an early sign)
- Crackles on inspiration
- Clubbing
- Autoimmune diseases
- Right sided heart failure, a consequence of DPLD.
What is used as a diagnostic evaluation of DPLD to tell us about distribution and extent of disease.
HRCT (High-resolution CT)
What can lead to a diagnosis without the need for biopsy?
HRCT
What will people with DPLD show on PFT?
- Decreased lung volumes (total lung capacity, residual volume, functional residual capacity)
- Decreased diffusing capacity
Is a lung biopsy always needed to diagnosis DPLD?
If so, how?
No, not always.
Best performed via an open procedure using a thorascopic approach.
What 2 diseases produce a granuloma response?
-All others will produce inflammation and fibrosis-
Sarcoidosis and hypersensitivity pneumonitis
- Drug-induced parenchymal lung disease
- Smoking-related DPLD
- Connective Tissue-Associated DPLD
- Pneumoconiosis
- Idiopathic Pulmonary Fibrosis
- Acute
What drugs cause drug-induced parenchymal lung disease?
- 1. Amiodorone
- 2. Methotrexate
- 3. Nitrofurantoin
DPLD is associated with which CT disorders?
- 1. RA
- 2. Progressive systemic sclerosis (scleroderma)
- 3. Polymyositis/dermatomyositis
- 4. Sjogen syndrome
- 5. Behcet disease
When DPLDs is associated with asbestos, silicon or beryllium, they are called ______.
Pneumoconiosis
Imaging of pneumoconiosis will show what?
- Basilar and subpleural bilateral, linear intersitial markings
- Calcified pleural plaques
Idiopathic pulmonary fibrosis is important to diagnose, why?
Poor prognosis
Histopathology of Idiopathic Pulmonary fibrosis shows what?
UIP
Acute intersitial pneumonia is also called __________.
Imaging:
Diagnosis:
Histopathology:
- Hamman-Rich syndrome
- Bilateral alveolar disease with ground-glass change
- Open lung biopsy
- DAD (diffuse alveolar damage)
When is organizing pneumonia termed BOOP or COP?
Tx?
- Proximate cause is IDd => BOOP
- No cause found => COP
- Systemic glucocorticoids
Hypersensitivity Pneumonitis
- Occurs when?
- HRCT shows
- Histo shows:
- Tx:
- Repeated immunologic reaction to inhalation of antigens
- Ground glass opacities with centrilobular nodues
- Noncaseating granulomas
- Avoid causes and steroids for chronic sx.
Sarcoidosis
- Is?
- Age?
- PFT shows
- Imaging:
- Histo shows:
- Tx:
- Formation of granulomas all over body, including lung that affects mostly blacks
- Bimodal: Peaks over 18 YO and ppl 50-60
- Restriction, reduced diffusing capacity, obstruction of airways
- Bilateral hilar adenopathy and/or interstitial infilteates or patchy alveolar infiltrates
- Bronchocentric noncaseating granulomas
- Systmic glucocorticoids
Many patients with severe/chronic DPLD develop what?
Pulmonary HTN
Treatments for people with DPLD?
- Stop smoking
- Supp O2
- Symptomatic tx for breathing and treatment of infections
- Vasodilating agents to reduce right side vascular resistance
