Restrictive Diseases

Interstitial Lung Disease
Causes
• Vasculitis (eg, granulomatosis with polyangiitis)
• Infections (eg, fungal, tuberculosis, viral pneumonia)
• Occupational & environmental agents (eg, silicosis, Common hypersensitivity pneumonitis)
• Connective tissue disease (eg, systemic lupus erythematous, scleroderma)
• Idiopathic pulmonary fibrosis, interstitial pneumonia
• Cryptogenic organizing pneumonia
S/S
• Progressive exertional dyspnea or persistent dry cough
• Pulmonary findings due to other underlying conditions (eg, silicosis, connective tissue disease)
• 50% of patients with significant smoking history
• Lung examination with fine crackles during mid-late inspiration, possible digital clubbing
Dx
CXR: reticular or nodular opacities
High-resolution chest CT: fibrosis, honeycombing, or traction bronchiectasis
PFTs: or ⤴️ FEV1/FVC ratio, ⤵️ DLCO,
• Resting arterial blood gas can be normal or show mild hypoxemia
• Exertion usually causes significant hypoxemia due to V/Q mismatch
Sarcoidosis
#
 African Americans
S/S
Pulmonary: hilar LN I Interstitial infiltrates
Skin
·       Papular, nodular, or plaque-like lesions
·       Erythema nodosum
Ophtha:
·       Anterior/posterior uveitis
·       Keratoconjunctivitis sicca
Hema: Peripheral LN I HSM
MSK: Acute polyarthritis (especially ankles)
CVS:
·       AV block
·       Dilated or restrictive cardiomyopathy
CNS:
·       7th CN Palsy
·       Central DI
Imaginc
Hilar LN 
Labs
·       ⤴️ Ca / ⤴️ Ca in urin
·       ⤴️ ACE level
Bx
Noncaseating granuloma
Tx
Steroids 
Obesity Hypoventilation Syndrome
Dx criteria
·       BMI ≥30
·       Awake daytime hypercapnia (PaCO2 >45)
·       No altemate cause of hypoventilation
Work-up
·       ABG on room air (hypercapnia, normal A-a gradient)
·       No intrinsic pulmonary disease on chest x-ray
·       Restrictive pattern on PFTs
·       Normal TSH
·       Polysomnography
Tx
·       Noctumal positive-pressure ventilation as first-line therapy
·       Weight loss (bariatric surgery in select cases)
·       Avoidance of sedative medications
·       Respiratory stimulants (Acetazolamide) as last resort
Asbestosis
RF
·       Prolonged Asbestos exposure (Shipyard, mining)
·       Sx develop ≥20 years after initial exposure
S/S
·       DRY COUGH 
·       Progressive SOB,
·       Basilar fine crackles,
·       Clubbing
·       ⤴️ Risk for Lung cancer & Mesothelioma
Dx
·       Pleural plaques on chest imaging
·       Imaging, PFT & histology consistent w/ pulmonary fibrosis

Leave a comment