L13 Interstitial Lung disease Flashcards
Diffuse Parenchymal Lung Disease aka
Interstitial lung disease
honeycombing
clustered cystic air spaces
Interstitial lung disease pathophysiology
Extension distortion of the airway and alveolar compartment along with the interstitium causing progressive scarring of lung tissue surrounding the alveoli
Areas of fibrosis alternate with areas of normal lung
Honeycombing
Causes of Interstitial lung disease
Idiopathic
Autoimmune
Exposure
Drugs
exposure causes
asbestos, silica, bat/bird droppings, radiation, hot tubs
drug causes
amiodarone propranolol nitrofurantoin methotrexate rituximab
autoimmune causes
dermatomyositis,RA, scleroderma, sarcoidosis, sjogrens
Interstitial lung disease is ______ so the treatment goal is to ______
IRREVERSIBLE
prevent progression/alleviate sx
what symptoms are uncommon in Interstitial lung disease
wheezing, chest pain
main sx of Interstitial lung disease
Progressive dyspnea on exertion
Persistent non-productive cough
extra-pulmonary symptoms may indicate
connective tissue disease→ musculoskeletal pain, weakness, joint pain/swelling, fevers, dry eyes/mouth
heard on lung exam of a patient with Interstitial lung disease
velcro crackles at bases
inspiratory squeaks
late stage Interstitial lung disease signs
cyanosis, digital clubbing cor pulmonale (middle/late)
Extrapulmonary manifestations: erythema nodosum
Sarcoidosis
Extrapulmonary manifestations: gottron’s papules
Dermatomyositis
highest yield for noninvasive test to diagnose Interstitial lung disease
High resolution chest CT (HRCT)
Gold standard to diagnose Interstitial lung disease
Tissue biopsy
findings on CXR that could indicate Interstitial lung disease
Ground glass appearance early, nonspecific
Reticular “netlike” (most common), nodular, or mixed pattern of opacities
Honeycombing: small cystic spaces: poor prognosis
reticular opacities are
small irregular opacities
Micronodular pattern is
small rounded opacities
HRCT: reticular opacities
IPF
Asbestosis
HRCT: ground glass attenuation
Drug toxicity
Respiratory bronchiolitis
HRCT: upper/central lobes
Sarcoidosis
Serologic studies to rule out subclinical autoimmune disease
ANA, RF
when to evaluate for vasculitis and how
pulmonary hemorrhage or suspicious systemic symptoms
Antineutrophil cytoplasmic antibodies (ANCA)
signs of restrictive disease on PFTs
Decreased Total lung capacity
Decreased FEV1, FVC
Normal/increased FEV1/FVC
PFTs obstructive disease
Increased TLCC
Normal FVC
Decreased FEV1
Decreased FEV1/FVC ratio
If PFTs indicate restrictive disease, obtain
DLCO: low=ILD
ILD with obstructive pattern
sarcoidosis
Extension of bronchoscopy that allows sampling→ cell counts, culture (atypical/typical), cytology (identify maligancy)
Bronchoalveolar lavage
Is bronchoalveolar lavage typically performed with HRCT findings consistent with IPF
no
gold standard for diagnosis
lung biopsy
contraindication for lung biopsy
honeycombing
Transbronchial biopsy is a broncoscope + biopsy used to assess ____
central locations
NOT periphery
2 types of surgical lung biopsies
- Video assisted thoracoscopic surgery: 2 incisions in lateral chest wall
- Thoracotomy: 5-6 cm incision
Bronchoscope used to evaluated hilar and mediastinal lymph nodes
useful if sarcoid suspected
Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)
2 biopsy methods that can be done in conjunction if a different scope is passed
Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)
-and-
Transbronchial lung biopsy
UIP=“Usual interstitial pneumonia”
Idiopathic pulmonary fibrosis
Abestosis
most common interstitial lung disease
Idiopathic pulmonary fibrosis
what is HRCT used for in idiopathic pulmonary fibrosis
Determine if: probable/indeterminate UIP pattern, alternative diagnosis→ Bronchoalveolar lavage/lung biopsy
treatment for idiopathic pulmonary fibrosis
Treatment for GERD
Nintedanib: tyrosine kinase inhibitor
Nintedanib is a _____ and works by
tyrosine kinase inhibitor
downregulation of production of growth factors and procollagens
sarcoidosis extrapulmonary findings
erthema nodosum
lupus pernio
granulomatous uveitis
arthralgias
Non-caseating granulomas
sarcoidosis
→ secrete 1,25 dihydroxyvitamin D→ serum Ca++ elevated
→ secrete ACE
labs of sarcoidosis
Elevated serum ACE +/- elevated serum calcium, hypercalciuria
Elevated alkaline phosphatase
lupus pernio
idk some weird nose nodule
sarcoidosis
CXR staging of sarcoidosis
- Normal
- Hilar adenopathy
- Hilar adenopathy + diffuse infiltrates
- Only diffuses parenchymal infiltrates
- Pulmonary fibrosis
when to treat sarcoidosis
stage 2 and symptomatic
Sarcoidosis tx
high dose corticosteroids + PJP prophylaxis
methotrexate (alternative)
chloroquine/hydroxychloroquine: cutaneous lesions, neuro sx, hypercalcemia
topical corticosteroids for ocular disease
lung transplant: stage IV
most common CXR pattern of sarcoidosis LAD
bilateral symmetrical hilar and right paratracheal mediastinal adenopathy
inhalation and deposition of mineral dust
pneumoconiosis: asbestosis, silicosis, coal worker’s
Chronic simple silicosis
10-12 years exposure
+/- asymptomatic
Nonprogressive once exposure eliminated
Hilar node calcification: eggshell pattern
Chronic complicated silicosis
> 20 years exposure
Progression even after exposure eliminated
Tachypnea, prolonged expiration, rhonchi, wheezing, rales
Advanced disease: cyanosis, cor pulmonale
silicosis classic CXR finding
eggshell calcifications
asbestosis presentation
15-20 years after exposure, dose dependent
40-75 year old males
Smoking increases risk of bronchogenic cancer
Presentation: Insidious onset, dyspnea, reduced exercise tolerance, chest discomfort, end inspiratory rales
Digital clubbing which does not correlate with severity
asbestosis imaging
HRCT: reticular opacities
Restrictive pattern
CXR: opacities in lower lungs, thickened pleura, pleural plaques
Open-lung biopsy not required but is definitive diagnosis: fibrosis, asbestos bodies through light microscopy
reliable indicator of asbestosis on CXR, not always present
calcified pleural plaques in diaphragmatic pleura of the 6th-9th ribs
asbestosis treatment
NO immunotherapy drugs or steroids alter disease course
basically prevent exposure and treat symptoms
asbestosis has a high risk of
Cancer
risk increased by smoking→ bronchogenic carcinoma
→ cancers of upper respiratory tract, esophagus, biliary system, kidneys
Mesothelioma
form of cancer almost always associated with asbestos exposure, even short term (1-2) years
Cancer develops in mesothelium: protective lining covering organs: pleura (most common), peritoneum, pericardium
Not caused by smoking, poor prognosis
granulomatosis with polyangiitis aka
“ANCA-associated vasculitis”
granulomatosis with polyangiitis
Multisystem autoimmune disease
Systemic vasculitis of small/medium vessels
Necrotizing granulomas of upper and lower respiratory tracts
RARE, 35-55 year old northern europeans
Relapse common
granulomatosis with polyangiitis presentation
recurrent respiratory infections, constitutional symptoms (fever, weight loss, night sweats, anorexia, fatigue/lethargy)
Ocular: conjunctivitis, episcleritis, uveitis, RAO
ENT: chronic sinusitis, rhinitis, epistaxis, saddle nose deformity
Pulmonary: infiltrates, cough, hemoptysis, dyspnea, stridor
Renal: failure, erythrocyte casts
Peripheral nervous system: cranial nerve palsies, sensorimotor polyneuropathy
Skin: palpable purpura, skin ulcers, necrotic, blistering purpura
Cardiac: pericarditis, coronary vasculitis
Polyarticular arthralgias
specific granulomatosis with polyangiitis findings that distinguish it
saddle nose deformity
infiltrates of CXR
a million systemic symptoms of vasculitis
(+) C-ANCA
CT: stellate shaped peripheral pulmonary arteries= “vasculitis sign”
granulomatosis with polyangiitis lab findings
(+) C-ANCA +/- RF, ANA
Elevated ESR/CRP Normocytic anemia Thrombocytosis Leukocytosis Increased BUN/Cr UA: proteinuria, RBC cast
granulomatosis with polyangiitis treatment
Cyclophosphamide: immunosuppressant which improves prognosis
Toxicities: CHF, thrombocytopenia, acute hemorrhagic cystitis, acute tubular necrosis, N/V/D
Corticosteroids
Hypersensitivity Pneumonitis
Repetitive inhalation of antigens in a susceptible host → inflammation
Antigens: bacteria, fungi, mold, proteins, chemicals, environments: bird/bat droppings, animal fur/feathers, hot tubs, hardwood dusts
4th-6th decade
Hypersensitivity Pneumonitis acute disease
remits without treatment
subtypes of Hypersensitivity Pneumonitis
Acute: flu-like syndrome within hours of exposure
Remits without therapy
Sub-acute: insidious onset of cough, dyspnea, fatigue over weeks
Chronic: progressive dyspnea, cough, fatigue, malaise
Hypersensitivity Pneumonitis symptoms
Diffuse, fine bibasilar crackles, fever, tachypnea, muscle wasting, clubbing, weight loss
Hypersensitivity Pneumonitis imaging
Interstitial inflammation
Honeycombing
Centrilobular fibrosis
Peribronchiolar fibrosis
Hypersensitivity Pneumonitis tx
Avoid antigen
Corticosteroids: speed recovery, chronic
adjunctive therapy: Bronchodilators, antihistamines, inhaled corticosteroids
Pulmonary hypertension/Cor pulmonale/CV disease
complication of interstitial lung disease
Lungs stiffen→ pulmonary arteries become dilated→ right ventricular dysfunction and enlargement
complications of interstitial lung disease
Pulmonary hypertension/Cor pulmonale/CV disease Pneumothorax PE Pulmonary infection Elevated cancer risk Progressive respiratory insufficiency
Granulomatosis with polyangitis imaging
CXR: variable, nodules which may cavitate
CT chest:
→ stellate shaped peripheral pulmonary arteries= “vasculitis sign”
→ Feeding vessels to nodules/cavities
→ Diffuse alveolar hemorrhage
Tissue biopsy: histopathologic evidence of vasculitis, granulomatous inflammation
Labs:
