Pulmonary Manifestations of systemic disease Flashcards by A B

DDX pneumonic

V = vascular
I = infection
N = neoplasm
D = drugs
I = inflamm/idiopathic/iatrogenic
C= congenital 
A = autoimmune
T = trauma
E = endocrine/metabolic

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Anatomic differential for lungs

1) airway
2) alveoli
3) interstitium
4) vascular
5) pleura
6) chest wall
7) extrathoracic

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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

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Define ALS
what is it a disease of?

symptoms

progressive neurodegenerative disease

muscle weakness with upper and lower motor neurons dysfunction

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patients with ALS may develop ___

dysphagia due to uncoord pharyngeal muscle movement –> aspiration of fluids and food

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pulm manifestations of ALS

1) chronic aspiration due to uncoord pharyngeal muscle movement (bulbar dysfunction)
2) recurrent pneumonia
3) resp muscle weakness

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effects of resp muscle weakness in ALS

1) inadeq ventilation to move CO2
2) nocturnal hypoventilation
3) weak cough risk of aspiration

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steps to diagnose pulm disease

1) CXR
2) PFT
3) ABG

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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

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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

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if you see decr FEV1, FVC but normal ratio

decr TLC
decr FVC in supine
decr MIP and MEP

what could patient have?

ALS

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P-V curve for ALS

shifted down due to chest wall weakness and restrictive disease

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what will ABG show for ALS

elev PCO2 or hypercarbia

pH 7.35
pCO2 55 mmHg
pO2 68 mmHg
HCO3 32 mmHg

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How do you treat pulm effects of ALS

1) noninvasive Positive pressure ventilation with biPAP
2) aspiration precaution
3) cough assistance

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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

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Describe RA

autoimmune disease

symmetric inflamm arthritis

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exam of RA

synovitis with tender, swollen, boggy joint

in symmetric small and large joints (wrist/shoulder)

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Labs for RA

Positive RF and anti-citrullinated peptide

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how do you treat RA

1) NSAIDS

2) DMARDS

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Pulm manifestations of RA

1) pleural disease
2) upper/smaller airway obstruction (bronchiolitis/bronchiectasis)

3) ILD (UIP>NSIP)

4) pneumonia
5) pulm HTN
6) vasculitis
7) pulm infection

CXR of RA

can show pleural effusion

PFTs of RA

Restrictive pattern with pleural effusion

decr DLCO b/c 1.5L in pleural space displacing lung

shunt and V/Q mismatch from lung collapse

pleural fluid containing
high glucose and low pH

ddx?

infection but also RA

if culture and cytology negative this means?

no malignancy or infection

ddx of pleural fluid

1) rheumatoid effusion 2) empyema (infected pleural space) 3) TB effusion 4) malignant effusion 5) drug tox

CTD with pulm manifestations

1) SLE 2) RA 3) scleroderma 4) DM/PM 5) mixed CTD 6) Ankylosing spondylitis

CASE 3 what does she have? 36 y/o 1-2 week of swelling in hand and face hemoptysis decr urinary frequency lab shows acute renal failure urine shows protein, RBC, WBC, granular casts

Goodpasture's diffuse alveolar hemorrhage rapidly progressive glomerulonephritis

airway DDx for hemoptysis

1) bronchitis 2) bronchiectasisi 3) cancer 4) aspirated foreign body 5) alveolar hemorrhage syndrome (capillary inflammation, alveolar damage 6) pneumonia/abscess

evaluation of patient what would you order 36 y/o 1-2 week of swelling in hand and face hemoptysis decr urinary frequency lab shows acute renal failure urine shows protein, RBC, WBC, granular casts

1) CXR | 2) bronchoscopy with bronchoalveolar lavage to find blood in alveoli

what would CXR show 36 y/o 1-2 week of swelling in hand and face hemoptysis decr urinary frequency lab shows acute renal failure urine shows protein, RBC, WBC, granular casts

patchy alveolar infiltrates with blood alveolar hemorrhage

what would PFT show 36 y/o 1-2 week of swelling in hand and face hemoptysis decr urinary frequency lab shows acute renal failure urine shows protein, RBC, WBC, granular casts

restrictive disease with incr DLCO all blood filling alveoli ineffective Hb binds CO

pulm manifestation of goodpastures

diffuse alveolar hemorrhage progessive glomerulonephritis antibodies against glomerular basement membrane

CASE 4 what does he have? 27 y/o hx of cramps and pain and diarrhea fever and fatigue productive cough with yellow green sputum SOB numerous pneumonia hx no asthma, no smoker scattered wheezes

IBD bronchiectasis

pulm manifestation of IBD

Obstructive 1) tracheobronchitis 2) subglottic stenosis = fixed obstruction 3) bronchiectasis 4) bronchiolitis Restrictive 5) PE 6) ILD usu ILD 8) infection

CXR with bronchiectasis

decr lung volumes 1) tram tracking = walls of bronchus dilated and thicken

if you see bronchiole walls surrounded by something on CXR what is it?

air bronchogram

CT with bronchiectasisi

dilation of airway --> as go distally, tapered size until terminal bronchiole/alveoli

CASE 5 what does he have? 32 y/o black hx of sickle cell runny nose, sore throat incr SOB with yellowing of eyes severe Chest pain, fever, cough

acute chest syndrome

pulm manifestation of sickle cell

1) infection 2) embolism due to sickle cell occlude vessel --> bone marrow infarct and fat emboli 3) infarct from in-situ thrombosis 4) hypoventilation from rib and sternal infarcts 5) pulm edema from XS hydration 6) pulm HTN 7) chronic lung disease and scarring

describe acute chest syndrome manifestation

new pulm infiltrate chest pain fever cough, wheeze, tachypnea

Acute chest syndrome | CXR

1) bilateral diffuse alveolar and interstitial infiltrates | 2) reticular appearance

Treatment of acute chest syndrome

1) antibiotics for PNA 2) O2 supplementation 3) transfusion

why give O2 supplementation for acute chest syndrome

1) patient's cells are sickling and lysing so not providing adequate O2 delivery 2) if transfuse healthy RBC, dilute sickled RBC so no flow problems

CASE 6 what cause hemoptysis 42 y/o HIV+ hemoptysis x10 days fever, chills, dyspnea smokes cigarette, IVDU unknown CD4

Kaposi's sarcoma in his airway causing alveolar hemorrhage

pulm complications of HIV | infectious

even if CD4 well controlled 1) bacterial pneumonia 2) TB 3) PCP 4) fungal/viral pneumonia

pulm complications of HIV | noninfectious

1) Kaposi's sarcoma = prolif of endothelium 2) non-Hodgkins' lymphoma 3) Lung cancer 4) emphysema 5) ILD: lymphocytic interstitial PNA, NSIP 6) pulm HTN 7) effusions = TB, lymphoma

evaluation of HIV patient with hemoptysis and pulm problems

1) CXR and CT 2) Cultures for infection 3) bronchoscopy due to alveolar hemorrhage

what would CXR show for alveolar hemorrhage

bilateral alveolar filling | and/or bilat pneumonia

what are skin findings of Kaposi's sarcoma

purport that can be on skin and line bronchi affecting endothelium of airway