Pulmonary Manifestations of systemic disease Flashcards
DDX pneumonic
V = vascular I = infection N = neoplasm D = drugs I = inflamm/idiopathic/iatrogenic C= congenital A = autoimmune T = trauma E = endocrine/metabolic
Anatomic differential for lungs
1) airway
2) alveoli
3) interstitium
4) vascular
5) pleura
6) chest wall
7) extrathoracic
Case 1
62 y/o male dx with ALS difficulty writing and buttoning
presents with dyspnea on exertion and more lethargic with confusion
numerous recent pneumonias
weak cough worse with drinking
a
Define ALS
what is it a disease of?
symptoms
progressive neurodegenerative disease
muscle weakness with upper and lower motor neurons dysfunction
patients with ALS may develop ___
dysphagia due to uncoord pharyngeal muscle movement –> aspiration of fluids and food
pulm manifestations of ALS
1) chronic aspiration due to uncoord pharyngeal muscle movement (bulbar dysfunction)
2) recurrent pneumonia
3) resp muscle weakness
effects of resp muscle weakness in ALS
1) inadeq ventilation to move CO2
2) nocturnal hypoventilation
3) weak cough risk of aspiration
steps to diagnose pulm disease
1) CXR
2) PFT
3) ABG
what will CXR show for ALS
1) right middle lobe infiltrate from chronic aspiration
filling defect next to soft tissue –> obscure right heart border
2) low lung volumes from chest wall weakness
what will PFTs show for ALS (6 things)
RESTRICTIVE
1) decr FEV1, FVC but normal ratio
2) decr TLC
3) decr FVC in supine
4) decr MIP and MEP
5) decr max voluntary ventilation (measure volume after max expiration/inspriation for 12s)
6) normal DLCO initially but can then lead to atelectasis and shunt from prolonged hypoventilation
if you see decr FEV1, FVC but normal ratio
decr TLC
decr FVC in supine
decr MIP and MEP
what could patient have?
ALS
P-V curve for ALS
shifted down due to chest wall weakness and restrictive disease
what will ABG show for ALS
elev PCO2 or hypercarbia
pH 7.35
pCO2 55 mmHg
pO2 68 mmHg
HCO3 32 mmHg
How do you treat pulm effects of ALS
1) noninvasive Positive pressure ventilation with biPAP
2) aspiration precaution
3) cough assistance
CASE 2
what does she have?
32 y/o woman Hx of RA
chest pain, SOB, DOE
SOB worse with supine
Dull to percussion on left chest
bilateral knee, ankle, wrist pain and MCP swelling
rheumatoid effusion
Describe RA
autoimmune disease
symmetric inflamm arthritis
exam of RA
synovitis with tender, swollen, boggy joint
in symmetric small and large joints (wrist/shoulder)
Labs for RA
Positive RF and anti-citrullinated peptide
how do you treat RA
1) NSAIDS
2) DMARDS