restrictive lung disease Flashcards
(30 cards)
what are the two general causes of extrapulmonary restrictive disease?
- neuromuscular (Guillain-Barre, Myasthenia Gravis, Poliomyelitis, Postpolio syndrome, ALS, Diaphragmatic disease)
- diseases of the chest wall (kyphoscoliosis, obesity, pickwickian syndrome)
what is pickwickian syndrome?
a serious consequence of obesity-hypoventilation which is characterized b a reduced central drive to breathe.
4 requirements for ARDS and ALI (acute lung injury)
- reduced art O2 to inspired O2 ratio (ALI, 200 < PaO2/FIO2 < 300 mmHG, in ARDS 200 < PaO2/FIO2… normal = 500)
- Acute onset
- Bilat. lung infiltrates
- pulm cap wedge P < 18 mmHg (or normal LAP) to rule out left-seided CHF as a cause of the lung infiltrates
what is ARDS and ALI (acute lung injury)
acute-onset diffuse alveolar damage and leakage of fluid out of pulm cap. into interstitium and alveoplar spaces (a restrictive disease)
what are hyaline membranes?
pockets of eosinophilic, acellular material that are seen inside the alveoli is ARDS and ALI (acute lung injury) and NRDS (neonatal resp distress synd) in histology
predisposing factors to NRDS (neonatal resp. distress synd) (3)
- prematurity
- maternal diabetes mellitus
- delivery by c-section
How can you assess fetal pulm. maturity?
measure the ratio of surfactant lecithin to sphingomyelin in the amniotic fluid. A ratio of 2:1 or greater indicates lung maturity
4 txt for NRDS
- exogenous surfactant admin
- mech. vent w/PEEP
- Inhaled NO
- Antenatal maternal corticosteroid therapy to promote surfactant production
4 common inorganic causes of Pneumoconiosis (a group of ILD (intersitial lung dis) caused by inhalation of inorganic and organic particulate matter)
- silicosis
- coal workers pneumoconiosis (CWP)
- Asbestosis
- Berylliosis
silicosis is assoc with an increased risk of…
TB
berylliosis can mimic what disease?
sarcoidosis (with granulomas in multiple organs)
silicosis is associated with what 3 finidings?
- lung nodules
- “egg shell calcification in hilar nodes
- TB
CWP (coal worker’s pneumoconiosis) is assoc with what histo finding?
“dust ells” (alveolar macrophages with anthracotic pigment)
asbestosis is associated with what two things?
branchogenic carcinoma > > malignant mesothelioma
berylliosis is assoc with what findings?
granulomas mimicking sarcoidosis
Ferruginous bodies (yellow-brown rods with clubbed ends) are seen in what disease?
asbestosis: the asbestos fibers are taken up by alveolar macrophages and coated with iron (which stains with prussian blue) to form ferruginous bodies that cause diffuse interstitial fibrosis
lab findings in sarcoidosis: calcium calciuria IgG ACE
calcium: hypercalcemia (due to increased 1-alpha-hydroxylase production of activated macrophages leading to increased 1,25-(OH)-Vit. D)
calciuria: hypercalciuria
IgG: hypergammaglobulinemia
ACE: increased ACE activity
what on biopsy is suggestive of sarcoidosis?
noncaseating granulomas (core macrophages sur. by T-lymphocytes) in the lung w/neg microbio workup
what lung sounds are heard in Idiopathic pulm. fibrosis?
dry cackles or rales
“honeycombing” is seen in what?
Usual Interstitial pneumonia (UIP)
honeycombing is seen in ______ whereas ground glass opacities are seen in ____
honeycombing: Usual Interstitial pneumonia (UIP)
ground-glass opacities: nonspecific interstitial pneumonia (NSIP)
causes of Hypercalcemia “Chimpanzees”
Calcium excess intake (milk-alkali syndrome) Hyperparathyroidism and Hyperthyroidism Iatrogenic (thiazides) Multiple myeloma Paget disease Addison disease Neoplasm (PTH, PTHrP etc) z Excess Vit D Excess vit A Sarcoidosis
Z can remind you that both hyperparathyroidism and Zollinger-Ellioson syndrome can be seen in multiple endocrine neoplasia (MEN1)
what is goodpasture syndrome?
autoimmune disease targeting the lungs and kidney
what is goodpasture syndrome caused by?
T2 hypersensitivity against the alpha3-chain of type IV collagen, located in the bsement membranes of alveoli and glomeruli
