Interstitial Lung Disease - Lehman

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Can you name the Interstitial Lung Disease - Lehman ?

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QuestionAnswerExtra Info
_______ aka Hamman-Rich Syndrome. 60% mortality within 6 months.
ILD affects the interstitial tissues in the lung parenchyma. They cause dyspnea, show up with reticular opacification on imaging and a ______ defect on PFTs.
Sarcoidosis Stage __: Hilar lymphadenopathy without parenchymal infiltrates.
ILD treatment is steroids then ______, but response isn't always great.
Functional Evaluation of ILD include ______, ABGs, and Cardiopulmonary Exercise Testing.
Inflammatory disease characterized by presence of noncaseating granuloma. Often in young healthy adults. Often resolves in 2-5 years, but 20% progress to a chronic form
Known or Unknown ILD: 60-70%. Occupational and environmental exposures. Associated connective tissue disorder or other primary disease (chronic pulmonary edema, neurofibromatosis, tuberous sclerosis, vasculitides, chronic gastric aspiration)
COPD tends to affect airways while RLD tends to affect the lung _____.
The most common radiographic abnormality of ILD is a _______ pattern.These findings are due to alveolar filling and increased interstitial markings
Mainstain therapy for ILD despite low success rate. Usually, start therapy, re-evaluate in 4-12 weeks, try to taper to maintenance dose.
Obstructive or Restrictive: Characterized by a limitation of expiratory airflow so that airways cannot empty as rapidly compared to normal. Examples are Asthma, COPD, Bronchiectasi
Name a relevant history for ILDs.
Underlying Histopathology: Accumulation of T lymphocytes, macrophages, epitheliod cells organized into discrete structures in the parenchyma. Can progress to fibrosis.Often asymptomatic. Most common are Sarcoidosis, hypersensitivity pneumonitis
Sarcoidosis Stage __: Fibrotic lung disease. Marked scarring and parenchymal distortion in the upper lobes. Bulla formation and cavities.
Sarcoidosis Stage __: Lymphadenopathy with parenchymal changes. Apical predominance. Diaphragm flattened suggests hyperinflation.
ILDs have a very poor prognosis of either inexorable progression (asbestosis) or ______ compromise (IPF, UIP, AIP)
Miliary nodules can be found in Sarcoidosis and ____. Nodules 1-2mm in diameter. Prominent bilateral hilar lymph node enlargement.
______ aka Hamman-Rich Syndrome is rare. Acute presentation with fever, cough, dyspnea*1-2 wk
______ of the fingers is when the distal part of the finger is enlarged compared to the proximal part
Imaging workup of ILD include CXR and ______.
QuestionAnswerExtra Info
Incidental finding on CXR for ILD include interstitial opacities in a reticular, nodular, or reticulonodular pattern. _______ lymphadenopathy for early stage Sarcoidosis.
Respiratory symptoms of ILD include progressive exertional dyspnea and persistent usually nonproductive ______.Hemoptysis, wheezing, chest pain less common
As many as 10% of patients with ILD will have a normal CXR, especially those with _________ pneumonitis.
Occupational lung disease caused by dust inhalation.
Complete PFTs include spirometry, lung volumes, and _______ capacity.
IPF has poor response to therapy with 50-70% mortality in ____ years.
Obstructive or Restrictive: Characterized by reduced lung volumes and decreased lung compliance. Examples include Interstitial Fibrosis, Scoliosis (severe), Obesity, Lung Resection
Underlying Histopathology: Starts with inflammation the interstitial tissues. Ultimately leads to fibrosis (irreversible scarring) of the alveolar walls. Histo patterns of UIP, NIP
Present with chronic onset (>12wk) exertional dyspnea, nonproductive cough, inspiratory crackles with or without digital clubbing. HRCT shows patchy, basilar, subpleural opacities.Honeycomb= classic pattern of IPF
Tissue diagnosis of ILD include bronchoscopy with bronchoalveolar lavage or transbronchial biopsy and ______ biopsy.
Known or Unknown ILD: 30-40%. Idiopathic interstitial pneumonias. Idiopathic conditions that have distinct histology, clinical features, and so are considered unique diseases.Sarcoidosis, Eosinophilic pulmonary diseases, hypersensitivity pneumonitis, pulmonary Langerhans' cell, granulomatosis
CXR with diffuse bilateral opacification. HRCT ground glass attenuation, air space consolidation. Fulminant course with rapid development of ARDS.
Imaging method for ILD that distinguishes airspace from interstitial disease, better assesses extent and distribution of disease. It is more likely to detect underlying disease.
Sarcoidosis Stage __: Nodular parenchymal infiltrates. Large pulmonary artery.
Cytotoxics have variable success for ILD. Cyclophosphamide and azathioprine are tried first. Then wait ____ weeks to reassess. If drug failure or intolerance, try methotrexate, colchicine, penicillamine, cyclosporine, but no consensus.
The most common form of idiopathic interstitial pneumonia. Male predominance.
Mineral fiber that was used for insulation of steam pipes, houses, brake pads from the early 20th century through late 1970s when it was outlawed and abatement began.
About ___% of the time, we see ILD in patients with occupational exposures to different kinds of dust, with connective tissue diseases or medication reactions.
Asbestosis latency is roughly ___ years from first exposure. Radiographic findings are interstitial disease and pleural plaques and/or calcifications. Early asbestosis has opacities in the lower lung fields. Advanced asbestosis are blunted costophrenic angles.

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