Systems Path Section 6 - Restrictive Lung diseases (pg 54-79)

disease of the lung that causes a decrease in lung expansion and vital capacity

restrictive lung disease

group of fibrotic disorders that are diffuse, bilateral, and patchy, MC idiopathic, result in hypoxia/dyspnea, and progressively lead to respiratory failure and/or pulmonary HTN

chronic interstitial lung diseases

widespread interstitial fibrosis of lungs leading to dyspnea and cyanosis, non-productive cough, and have a poor prognosis

idiopathic pulmonary fibrosis

how does idiopathic pulmonary fibrosis develop?

repeated alveolar damage causing faulty repair leading to fibrosis

who is most likely to get idiopathic pulmonary fibrosis?

males 55+

traditional pneumoconioses

coal, silica, asbestos

what type of pneumoconiosis is the accumulation of coal/carbon in lungs leading to minimal symptoms (dyspnea) which may progress in certain individuals

coal dust (worker's) pneumoconiosis

who is most likely to get coal worker's pneumoconiosis?

coal workers

Coal Worker's Pneumoconiosis MC affects....

upper lobes

coal dust accumulates in the lungs, asymptomatic, no inflammation

anthracosis

complicated coal worker's pneumoconiosis

progressive massive fibrosis

MC pneumoconiosis which causes significant inflammatory reaction leading to pulmonary fibrosis, dyspnea, for pulmonale and lung cancer & TB

silicosis

characteristics of silicosis

silicotic nodules, egg-shell calcifications

asbestos exposure leading to significant inflammatory response and resulting in fibrosis of lungs, progressive cough, dyspnea, and increased risk for mesothelioma and lung cancer

asbestosis

how does asbestosis develop?

asbestos causes significant inflammatory response with fibrosis

asbestos exposure is most likely to cause

fibrotic pleural plaques

multisystem inflammatory disorder characterized by fibrosis, non-caseating granulomas, substernal chest pain, dry cough and dyspnea

sarcoidosis

how does sarcoidosis develop?

chronic inflammation -> fibrosis, bilateral hilar lymphadenopathy

who is most likely to develop sarcoidosis?

young adults, African Americans, Scandinavians, Hx or TB or borrelia bugodorferi exposure

what occurs in 90% of people with sarcoidosis?

hilar lymphadenopathy

what is found in 25% of people with sarcoidosis?

erythema nodosum

potato nodes is associated with which pathology?

sarcoidosis