The NCCPA™ PANCE Pulmonary Content Blueprint includes three types of restrictive pulmonary diseases
 Osmosis |
|
 |
| Idiopathic pulmonary fibrosis | Patient will present as → a 55-year-old female who is a current smoker presents with a 9-month history of respiratory symptoms, including dyspnea on exertion, thoracic pain, and dry cough, which were preceded by a pulmonary infection. On auscultation, you hear inspiratory crackles. Pulmonary function tests (PFTs) show only mild impairment of vital capacity with decreased lung volume and a normal to increased FEV1/FVC ratio. Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason
DX: CXR shows fibrosis
Tx: Few effective treatment options other than a lung transplant
|
| Pneumoconiosis | Patient will present as → a 53-year-old man who presents to the office complaining of progressive dyspnea over the past few years. History reveals that he has worked in construction for the past 20 years demolishing and refurbishing old buildings. He rarely uses any protective breathing equipment. Physical examination demonstrates an afebrile man in mild respiratory distress with inspiratory crackles. The chest x-ray reveals a reticular linear pattern with basilar predominance, opacities, and honeycombing. (asbestosis) Any fibrosis of the lung tissues with a known cause - usually from prolonged environmental or occupational contact
S/SX: SOB + nonproductive cough + chronic hypoxia, cor pulmonale DX: CXR: interstitial fibrosis
Tx: primarily supportive ⇒ oxygen, vaccinations (pneumococcal, flu) and rehab
|
| Sarcoidosis | Patient will present as → a 30-year-old African American female with a cough, fever, and generalized body aches. You order a CXR which shows bilateral hilar adenopathy. Chronic autoimmune inflammatory disease in which small nodules (granulomas) develop in lungs, lymph nodes, and other organs ↑ ACE levels + ↑ calcium levels + bilateral hilar adenopathy Presentation:
Treatment: Steroids = 90% respond to steroid
Prognosis depends on disease severity; spontaneous improvement common
|
