ILD And Vacular Dz Flashcards
What is the definition of ILD
A group of respiratory disorders with many potential causes featuring variable degrees of pulmonary inflammation and fibrosis
What are the common S/s of ILD
Cough, dyspnea, restrictive pattern on PFTS
Can be with a co existing airflow obstruction (mixed) pattern
Decreased DLCO
INCREASED alveolar-arterial oxygen difference at rest or during exertion
What is the CXR finding in ILD
Dyspnea, late inspiratory crackles and CXR with septal thickening and reticulonodular changes.
Does ILD effect the airways proximal to the bronchioles
NO!
Leads to obliteration of capillaries & fibrosis
What is the pathophy of ILD
injury leading to attempted repair causing fibrosis and a honeycomb pattern on CXR and secondary pulm hypertension
What is the general etiology of ILD
No specific cause found in most patients
Most common known causes are medications & inorganic/organic dusts, radiation
Strong correlation with connective tissue disease
—Rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, dermatomyositis, Sjogren syndrome
What are the common CXR findings of ILD
Diffuse ground glass pattern
Progression to Reticular/linear infiltrates
Nodules (reticulonodular infiltrates)
Ill-defined nodules with air bronochograms
(Acinar rosettes)
What is the common CXR finding in sarcoidosis
hilar and mediastinal adenopathy
What is the common CXR finding in Berylliosis
hilar and mediastinal adenopathy
What is the common CXR finding in Silicosis
hilar and mediastinal adenopathy
What is the common CXR finding in chronic eosinophilic pneumonia
peripherally located pulmonary infiltrates in the upper and middle lung zones with relatively clear perihilar and central zones
You see peripherally located pulmonary infiltrates in the upper and middle lung zones with relatively clear perihilar and central zones CXR think ?
Chronic eosinophilic pneumonia
What are the adv/ disadvantages of CXR
can be helpful but often not diagnostic, low radiation
What are a the adv/ disadv of CT
can be much more diagnostic than CXR , higher radiation (but still low risk)
What are the procedural complications of Tissue Bx
Bleeding, pneumothorax, truama
You see decreased lung volumes, honeycombing, perihilar reticular changes with a lower lobe predominance
Think
ILD- Idiopathic pulm fibrosis
What type of infiltrates are seen in crypogenic organizing pneumonia
Migratory infiltrates
What type of infiltrates are seen in chronic eosinophilic pneumonia
Migratory infiltrates
What is the most common type of ILD
Idiopathic pul fibrosis
What is the appraoch to idiopathic interstitium pneumonias
1st step—identify pts whose disease is truly idiopathic
i.e., not infectious, med-related, environmental, occupational or connective tissue related
Careful medical hx
CXR & high-resolution CT may be diagnostic
Lung biopsy often required for definitive dx
What is “usual” interstitium pneumonia
lung injury characterized by patchy collagen fibrosis with associated scarring distributed in a peripheral, sub-pleural fashion with honeycomb changes.
You see lung injury characterized by patchy collagen fibrosis with associated scarring distributed in a peripheral, sub-pleural fashion with honeycomb changes.
What is this?
UIP, usually interstitium pneumonia
A pt comes in recently diagnosed with asthma, on your workup you hear bibasilar inspiratory crackles and note clubbing of the fingers
The pt has a NML SPO2 at rest with exertional desat
There is a restrictive pattern on PFTS
And the CXR shows bilateral reticualr opacities.
What is this? And what would you expect to see on CT
Idio pulm fibrosis
CT: Patchy reticular opacities and honeycombing with a peripheral, bibasilar predominance
And
Minimal ground-glass opacities
What is the dx standard for IPF and its tx
Dx: Lung Bx
(May not be needed if classis s/s and Ct are high index)
Tx: Prednisone is often ineffective
Nintedanib and pirfenidone are more effective but very very $$$
Tx the pt with supplemental O2 if sat is below 89
Only definite Tx is Transplant
What is the general prognosis of IPF
Progressive decline in pulmonary function ultimately resulting in death
5 year survival 50%
Average survival from time of dx: 2-5 yrs
What is the MOA of nintedanib
Tyrosine kinase inhibitor
This is the second most common ILD
Presents with granulomatous disease of unknown etiology
Can often involve the skin, eyes, lymph, neuro, liver, kidney, and heart
Highest incidence is in African America women
S/s Cough, dyspnea, chest pain, fatigue, fever, and wt loss
With erythema nodosum, lupus perino , iritis, arthritis, peripheral neuropathy, parotid enlargement, hepatosplenomegaly, and muscle weakness
Think? Dx? CXR? Tx?
Sarcoidosis
CXR: Often asymptomatic with abnormal CXR
—Bilateral hilar adenopathy common
—Right paratracheal lymphadenopathy
Dx: biopsy on noncaseating granulomas
Tx: oral corticosteroids or methotrexate if unable to handle steroids
You are treating a pt with sarcoidosis and they develop myopathy, difficult to control DM, excessive wt gain, osteoporosis from corticosteroid tx
What should you do
Switch them to methotrexate
What is stage 1 sarcoidosis on CXR
Bilateral hilar adenopathy
What is stage II sarcoidosis on CXR
Hilar adenopathy w/ parenchymal involvement mainly in middle or upper lobes (stage II)
What is stage III sarcoidosis on CXR
Parenchymal involvement alone mainly in upper lobes (stage III)
—Diffuse reticular infiltrates
What is stage IV sarcoidosis on CXR
Advanced fibrotic upper lobe changes (stage IV)
A young black woman with erythema nodosum should raise high index of suspicion for what dz
Sarcoidosis
How can sarcoidosis effect the heart
Can lead to restrictive cardiomyopathy and dys-rhythmias
What major lung complications can sarcoidosis cause
Lung complications: fibrosis, pneumothorax, hemoptysis, respiratory failure
What is the minim follow up criteria for a pt with sarcoidosis
At minimum– annual physical exam, PFTs, CMP, ophthalmologic exam, CXR, ECG
A pt presents with bilateral hilar lymphadenopathy, erythema nodosum and migratory poly arthralgias
What is this the triad for and what is the tx
Lofgren syndrome- Sacroidosis
No need to perform Bx
treat with NSAIDS, low dose glucocorticoids, colchicine and hydroxychlorquine
A pt present with fever uveitis and parotisis
Think?
Heerfordt Waldenstrom Sydome
Rare form of sarcoidosis that can lead to sicca that can mimic shorten syndrome
What pattern should sarcoidosis have on PFT
Restrictive with a decreased diffusing capacity
A pt lanes present with leukopenia, elevated ESR, hypercalcemia, and hypercaliuria, and elevated ACE levels
Think?
Sarcoidosis
You find hemosiderin-laden macrophages on bronch lavage
What dz is this the Dx for
How would you treat this Dz
Idiopathic pulmonary hemosiderosis
Common in children and y/a
Causes recurrent pulm hemorrhage
Iron deficieny is typical
Treatment: acute hemorrhage episodes–corticosteroids may be useful
What CXR findings would you expect to see in a pt with Idio pulm hemosiderosis
CXR reveals diffuse, bilateral alveolar infiltrates
. A chronic interstitial infiltrate may develop after repeated episodes, infrequently with hilar and mediastinal adenopathy
How can you tell the difference between Goodpasture and Idiopathic pulm hemosiderosis
Good pasture has postive anti GBMs and renal involvement with microscopic hematauria
Hemosiderosis does not
A 30-40 year old man presents with hemoptysis, anemia. Dyspnea, cough and fever, with hypoxia
CXR show diffuse bile alveolar infiltrates
Labs reveal iron def anemia, and microscopic hematauria
What is the Dx for this condition and Tx ?
This the Anti-glomerular basement membrane disease (Goodpasture Syndrome)
Dx: IgG on immunofluorescence & anti-glomerular basement membrane antibodies
Tx: combo of corticosteroids, cyclophosphamide, plasmapharesis to remove antibodies
DDX: idiopathic Pulm Hemosiderosis
However hemosiderosis doesnt have urine involvement or antiBM antibodies)
What is the dx for eosiphilic Granulamatosis with polyangitis
Lung, skin or nerve biopsy: histologic features of fibrinoid necrotizing epithelioid & eosinophilic granulomas
What is the Dx for granulomatic with polyangitis
Serologic testing (C-ANCA)
and biopsy of lung, sinus tissue, or kidney with demonstration of necrotizing granulomatous vasculitis
This is a rare dz where phospholipids accumulate witching the alveolar spaces
Can be idiopathic or 2/2 immunocomp, CA, inhalation of silica, titanium, or aluminum, or post infectious with TB
S/s progressive dyspnea, and cough
THink?
DX?
CXR?
Tx?
pulmonary alveolar proteinosis
Dx: Bronchoalveolar lavage
-Characteristic findings of milky appearance and PAS-positive lipoproteinaceous material
Some cases, will need transbronchial or surgical lung bx
—Amorphous intra-alveolar phospholipid
CXR: bilateral alveolar infiltrates suggestive of pulmonary edema
Tx: periodic whole lung lavage with warm saline, under general anesthesia with supplement O2 post treatment
What infection are pts with pulm alveolar proteinosis at an increased risk for
Increased risk for pulmonary infection with Nocardia or fungi
What medications increase the risk of Eosinophilic pulmonary syndromes
Exposure to medications (nitrofurantoin, phenytoin, ampicillin, acetaminophen, ranitidine)
infections with helmith s (Ascaris, hookworms, Strongyloides) or filariae (Wuchereria bancrofti, Brugia malayi)
Lead to what kind of pulmonary syndrome e
Eosinophilic pulm syndomre
A pt present with acute, febrile illness, cough, and dyspnea wit rapid progresision to resp failure
Often assoc with recent initiative or resumption of smoking
BAL reveals increased eosinophils without serum eosinophilia
Think>? CXR? Tx?
Acute Eosinophilic Pneumonia
CXR: abnormal, but nonspecific
Tx: Response to corticosteroids is usually dramatic
A woman presents with fever, night sweats, and wt loss, and Dyspnea
Is a non smoker
BAL reveals marked increased eospinophils
Think? CXR? Tx?
Chronic Eosinophilic Pneumonia
CXR: Infiltrates commonly involve upper lung fields
TX: oral prednisone and taper
This is an idiopathic disease characterized by
-Glomerulonephritis
-necrotizing granulomatous vasculitis of upper & lower resp. tracts (pulmonary infiltrates, nodules or cavitations),
and varying degree of small vessel vasculitis
S/s chronic sinusitis, chronic serous otitis (fluid in middle ear), with fever, and skin rash and wt loss
+/- hemoptysis, cough, CP and dyspnea
Think?
DX?
CXR? Tx?
Granulomatosis with polyangiitis
Aka Wegeners
Dx: serologic testing (C-ANCA) and biopsy of lung, sinus tissue, or kidney with demonstration of necrotizing granulomatous vasculitis
CXR: Characteristic sign of lung disease is nodular pulm. infiltrates w/ cavitation
Tx:
- Cyclophosphamide
- Oral corticosteroids (prednisone)
- Rituximab (Truxima) – antineoplastic, -Anti-CD20 (monoclonal antibody)
- Bactrim to prevent relapses in some cases
You are evaluating a pt and you find Tracheal stenosis, facial deformities (saddle nose), strawberry gums, petechiae/purpura
Think? TX?
Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
Tx: Cyclophosphamide and prednisone
RITUXIMAB can be used
And Bactrim for relapsing cases
This is a Idiopathic multisystem vasculitis of small and medium-sized arteries in pts w/ asthma
S/s chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia
Think? Dx? CXR? Tx?
Eosinophilic granulomatosis with polyangiitis
AKA Churg-Strauss syndomome
Dx: Serum eosinophilia
Lung, skin or nerve biopsy: histologic features of fibrinoid necrotizing epithelioid & eosinophilic granulomas
CXR: transient opacites to multiple nodules
TX; Corticosteroids and Medolizumab (interlukin 5 antagonist)
And cyclophosphamide for severe disease
