Restrictive Pulmonary Disease

Restrictive Pulmonary Disease-

-Idiopathic Pulmonary Fibrosis-

-Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disorder of the lower respiratory tract that typically affects adults over the age of 40

-The pathogenies of IPF is unknown.  Certain risk facts include cigarette smoking, viral infection, environmental pollutants, chronic aspiration, genetic predisposition, and drugs

-Patients present with progressively worsening shortness of breath and eventually hypoxemia

-The severity of the disease is determined by pulmonary function testing

-Pulmonary function test usually demonstrate a reduction of the FVC and DLCO

-Advance disease is characterized clinically by dyspnea on mild exertion (less than 300 feet) and a requirement for supplemental oxygen at rest or exertion.  Extensive honeycombing is seen on imagine studies such as chest x ray or CT scan of chest

-The prognosis of IPF is poor.  Only 20-30 percent of all patients will survive greater than 5 years

-Elements of supportive care for IPF include eduction, supplemental oxygen if needed, pulmonary rehab, and vaccines against influenza and Streptococcus pneumoniae

-No medications have been show to slow lung damage

-Potential medication therapies include pirfenidone and phosphodiesterase inhibitors

-Lung transplantation is an option if patients with IPF meet certain conditions

-Pneumoconiosis-

-Coal workers pneumoconiosis comes from inhalation and deposition of silica free coal dust particles that induce the formation of coal macules in the alveoli

-Findings with pneumoconiosis give pigment and reticulin fibers that accumulate in the peri-hilar location.  Over time black amorphous masses develop and liquify in the center.  There is cavitation that results in ischemic necrosis

-On x ray you see rounded small nodular opacities less than 1 cm usually in the upper lobes.  Eventually there is a confluence of large opacities that lead to massive progressive fibrosis

-Findings of PFT's are similar to IPF.  There is a reduced FVC and DLCO.

-May have some improvement when the offending agents are removed

-Much like IPF, treatment is largely supportive


-Sarcoidosis-

-Sarcoidosis is a multisystem granulomatous disorder of unknown etiology and is characterized by non-caseating granulomas in involved organs

-Sarcoidosis typically presents in young adults with pulmonary reticular opacities, bilateral hilar adenopathy, and skin, joint, or eye lesions

-The exact etiology and pathogenesis of sarcoidosis are unknown

-Sarcoidosis most often involves the lung (70 percent of the time)

-Diffuse interstitial lung disease is the classic type of lung development

-Common presenting symptoms of sarcoidosis are cough, dyspnea, chest pain, fatigue, malaise, fever, and weight loss

-Patients with lung parenchyma sarcoidosis typically will have crackles heard on lung exam.  Wheezing is heard when there is endobronchial involvement or traction bronchiectasis due to scarring

-Children with sarcoidosis usually do not become symptomatic

-Initial workup for suspected sarcoidosis patient should include:  CBC, BMP, tuberculin skin test, PA and lateral chest x ray, pulmonary function testing, EKG, and eye exam by an ophthalmologist

-Diagnosis is made my biopsy of involved organ.  Can do skin biopsy, or lung biopsy to confirm most commonly

-The majority of patients with pulmonary sarcoidosis have non progressive disease or experience spontaneous remission

-Oral glucocorticoids have been used for the relief of symptoms and control of potentially disabling respiratory impairment