Pulmonary manifestations of systemic diseases

Question 1

Mnemonic for differentials

Answer 1

VINDICATE: Vascular, Infection, Neoplasm, Drugs, Inflammatory/idiopathic/iatrogenic, Congenital, Autoimmune, Trauma, Endocrine/metabolic

Question 2

Amyotrophic lateral sclerosis- description

Answer 2

Amyotrophic lateral sclerosis (aka Lou Gehrig’s disease) is a progressive neurodegenerative disease that causes progressive muscle weakness which causes upper and lower motor neuron dysfunction. May develop dysphagia

Question 3

Pulm manifestations of ALS

Answer 3

Chronic aspiration due to uncoordinated pharyngeal muscle movement (“bulbar dysfunction”). Recurrent pneumonia and Respiratory muscle weakness (Inadequate ventilation, Nocturnal hypoventilation,
Weak cough)Chronic aspiration due to uncoordinated pharyngeal muscle movement (“bulbar dysfunction”). Recurrent pneumonia and Respiratory muscle weakness (Inadequate ventilation, Nocturnal hypoventilation,
Weak cough)

Question 4

Diagnosis of Pulm diseas in ALS

Answer 4

Chest X ray, PFTs, ABG

Question 5

Chest X ray results of ALS

Answer 5

Low lung volumes from chest wall weakness and infiltrates from chronic aspiration.

Question 6

PFT results of ALS

Answer 6

•Restrictive pattern: reduced FEV1, FVC; normal ratio; reduced TLC. Reduced FVC in supine position. Reduced maximal inspiratory pressure (MIP) and reduced maximal expiratory pressure (MEP). Reduced maximum voluntary ventilation. Generally normal DLCO initially; prolonged hypoventilation results in atelectasis and shunt.

Question 7

what is atelectasis

Answer 7

collapse of lung

Question 8

ABG results in ALS

Answer 8

elevated PCO2 - hypoventilation

Question 9

Treatment of pulm manifestations of ALS

Answer 9

Noninvasive positive pressure ventilation, Aspiration precautions, Cough assistance

Question 10

Describe Rheumatoid arthritis (RA)

Answer 10

•RA is an autoimmune disease with a hallmark of symmetric, inflammatory arthritis. Prolonged morning stiffness, synovitis, small and large joints. Positive for rheumatoid factor and anti-CCP

Question 11

Pulm manifestations of Rheumatoid arthritis

Answer 11

Pleuritis, pleural effusion and thickening, pneumothorax, upper airway obstruction, small airway obstruction, interstitial lung disease, organizing pneumonia, nodules, pulmonary hypertension, vasculitis, drug rxns, infections

Question 12

Connective tissue diseases with pulmonary manifestations

Answer 12

Systemic lupus erythematosus, Rheumatoid arthritis, Systemic sclerosis (scleroderma) Sjogren’s disease, Dermatomyositis/polymyositis, Mixed connective tissue disease, ankylosing spondylitis

Question 13

Goodpastures syndrome description

Answer 13

Idiopathic disease that manifests as diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. The disease is thought to be mediated by antibodies directed against glomerular basement membrane

Question 14

Goodpastures syndrome CXR and PFTs

Answer 14

CXR shows patchy alveolar infiltrates that contain blood from alveolar hemorrhage. PFTs show restrictive disease (low volumes) with increased DLCO (b/c the extra blood in the lungs absorbs the O2 during the DLCO test)

Question 15

Pulmonary manifestations of inflammatory bowel disease

Answer 15

Tracheobronchitis, Subglottic stenosis, Bronchiectasis, Bronchiolitis, Pleural Effusion, Interstitial Lung Disease, Pulmonary embolism

Question 16

Describe PFTs in IBS

Answer 16

Will be obstructive if Tracheobronchitis, Bronchiectasis
or Bronchiolitis. Will be restrictive if pleural effusion or interstitial lung disease. Will be obstructive if Tracheobronchitis, Bronchiectasis
or Bronchiolitis. Will be restrictive if pleural effusion or interstitial lung disease.

Question 17

Pulmonary manifestations of sickle cell disease

Answer 17

infection, Embolic phenomena due to bone marrow, infarction and fat emboli, Infarction caused by in-situ thrombosis, Hypoventilation due to rib and sternal infarctions, Pulmonary Edema due to excessive hydration, Pulmonary hypertension, Chronic lung disease

Question 18

What is acute chest syndrome

Answer 18

New pulmonary infiltrates(bilateral, diffuse alveolar and interstitial), chest pain, fever, respiratory symptoms (cough, wheeze or tachypnea) seen in sickle cell disease

Question 19

Treatment of acute chest syndrome

Answer 19

Antibiotics for pneumonia, Oxygen supplementation, Transfusion, Supportive care

Question 20

Pulm complications in HIV

Answer 20

Infection, Kaposi’s sarcoma, cancer, emphysema, ILD, pulmonary HTN, effusions