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Pulmonary Boards > Diffuse Parenchymal Lung Disease > Flashcards

Diffuse Parenchymal Lung Disease Flashcards

1
Q

How is small fiber neuropathy due to sarcoid treated?

A

Fells pain, paresthesias, numbness, and burning sensations. Can be resistant to standard immunosuppressive treatment including steroids and methotrexate. IVIG and anti-TNF can be beneficial. Can also try amitriptyline, gabapentin, or carbamazepine.

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2
Q

What are the CT findings of chronic hypersensitivity pneumonitis?

A

Mosaic attenuation, diffuse centrilobular nodular opacities, and GGOs with lung bases spared

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3
Q

What are the features of silicosis?

A

Histo: non-necrotizing granulomas, multinucleated giant cells with cytoplasmic inclusions (asteroid bodies), silicotic nodule with whorled hyalinized collagen, polarized microscopy shows faintly birefringent particles within fibrotic nodules
Features: drilling/sandblasting/rock quarry, can take decades to develop symptoms. Has increased risk of developing cancer
Imaging: innumerable upper lobe predominant small rounded nodules with perilymphatic distribution, hilar/mediastinal LAD, eggshell calcification of LNs, can be PET avid

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4
Q

What are some genes associated with lung diseases?

A

Familial pulmonary fibrosis- TERT/TERC, SFTPC, MUC5B, RTEL1, TOLLIP
Birt-Hogg-Dube-FLCN (chromosome 17p11.2) tumor suppressor gene folliculin
Tuberous sclerosis/LAM- TSC1 and TSC2

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5
Q

What are the features of silicone embolism syndrome?

A

Histo- clear vacuoles containing translucent refractile, nonbirefringement microdroplets within and adjacent to alveolar septal capillaries surrounded by histiocytes
Features- right heart failure, ARDS, ILD
Imaging- disruption of implant

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6
Q

How is scleroderma ILD best treated?

A

MMF to improve FVC, equal benefit from cyclophosphamide but more than 12m of treatment had serious SE. Only have data on nintedanib helping slow progression of FVC loss.

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7
Q

What are the features of pulmonary alveolar microlithiasis?

A

Histo- rarely needed
Features- Dx in early adulthood, autosomal recessive
Imaging- Sandstorm on CXR, CT with micronodular calcification along bronchovascular bundles and subpleural regions
Dx- Usually just imaging, BAL may show lamellar microliths
Tx- supportive, lung transplant

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8
Q

What are risk factors for increased in-hospital mortality for surgical lung biopsies?

A

Male, increasing age, high comorbidity scores, open vs VATS, “provisional” Dx IPF/CT-ILD. 6.4%. Cryobiopsy is now deemed an acceptable alternative to surgical lung biopsy

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9
Q

What are some definite histopathologic findings for UIP pattern?

A

dense fibrosis/architectural distortion +/1 honeycombing, patchy involvement, fibroblast foci, subpleural/paraseptal predominance, no features suggesting an alternative Dx such as granulomas

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10
Q

Remember chart for radiologic and histologic UIP patterns and their likelihood of being IPF

A
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11
Q

What is the benefit of inhaled treprostinil in CT-ILD related PH?

A

Increased 6MWT, no change in QOL

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12
Q

How is progressive pulmonary fibrosis defined?

A

ILD other than IPF AND 2/3 of the following within the past year without explanation: 1) worsening symptoms 2) physiologic progression (absolute 5% decline FVC or absolute 10% decline in DLCO), 3) radiologic progression

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13
Q

What are the indications for antifibrotics?

A

Nintedanib- IPF, SSc0ILD, chronic fibrosing ILD with progressive phenotype (PPF)

Pirfenidone- IPF

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14
Q

What is the common radiologic finding in CPFE?

A

Upper lobe emphysema and lower lobe fibrosis

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15
Q

What is the most common radiologic pattern of fibrosis in CT-ILD?

A

NSIP, except in RA-ILD (UIP pattern is most common) but will often get tested in seeing LIP in Sjogren’s despite NSIP still being more common

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16
Q

What type of lymphoma is most often seen in patient’s with Sjogren’s?

A

MALTomas

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17
Q

What are the features of antisynthetase syndrome?

A

Features: subset of PM/DM, “mechanics hands”, acute onset, Raynaulds, difficult to treat
Dx: anti-Jo, aminoacyl-tRNA synthetase

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18
Q

What are the treatments for CT-ILDs?

A
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19
Q

What are the features of sarcoidosis?

A

Features: most common in AA, women, familial clusters, 20-60 on presentation with 90% of patients with lung involvement, wheezing if airway involvement, rare clubbing, some extrapulmonary involvement including eyes, cardiac, skin, calcium, nervous system, LNs
Imaging: hilar/mediastonal LAD, nodules along bronchovascular bundle, upper lobe traction broncheictasis and fibrosis, perilymphatic nodules
Dx: noncaseating granulomas in Bx, lymphocytic BAL (15+%), high CD4/CD8 ratio
Staging: 0= no lung involvement, 1= bilateral hilar LAD, 2= bilat hilar LAD + parenchymal, 3= parenchymal without hilar LAD, 4= progressive fibrosis +/- cavity/cysts
Tx: treat symptomatic stage II/III with PFT impairment or progression or with extrapulmonary involvement with pred 20-40mg/d (40-60 in cardiac) for 4-6w then taper over 6-12 mo, alt. MTX, AZA, Leflunomide, TNF antagonist, transplant. For cough in mild I/II, try ICS. Can have spontaneous remission in all but stage IV

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20
Q

What are the features of granulomatous lymphocytic ILD?

A

Features: ILD with non-necrotizing granulomas, LIP, and follicular bronchiolitis. In patients with immunodeficiency, often CVID, 20-50yo, DOE/cough, or asymptomatic
Imaging: GGO, nodules, LAD
Dx: usually sugrical lung Bx
Tx: IVIG, steroids, immunosuppression/RTX

21
Q

What are the features of follicular bronchiolitis?

A

Histo: reactive lymphoid follicles with germinal centers around bronchi/bronchioles
Features: can occur independently or with autoimmune disorders, middle aged, gradual onset, fever and weight loss
Imaging: bilat centrilobular nodules and patchy GGOs
Dx: surgical lung biopsy as TBBx cannot provide enough tissue, distinguish from lymphoma as can occur frequently in Sjogrens
Tx: immunosuppressive drugs, some case reports with macrolides

Of note: differentiated from LIP as it has diffuse interstitial and alveolar septal infiltration of lymphocytes

22
Q

What are the features of cryptogenic organizing pneumonia?

A

Histo: endoluminal granulation tissue polyps (Masson bodies), type II pneumocyte metaplasia
Features: idiopathic or associated with infection, autoimmune, IBD, transplant, drug, environment. Cough, fever, malaise, weight loss
Imaging: patchy lower lung predominant consolidation and GGO with subpleural/peribrocnhovascular distribution. Bilateral, peripheral, and migratory. can also have atoll sign.
Dx: biopsy
Tx: prednisone .75-1mg/kg/day with gradual tapering over 6-12 months

23
Q

What are the features of pulmonary benign metastasizing leiomyoma?

A

Histo: uterine well-differentiated spindle shaped cells
Features: rare, women of late childbearing age with Hx uterine leiomyomas, cough, dyspnea, PTX/hemothorax, hemoptysis
Imaging: numerous random, well-circumscribed nodules with miliary pattern, can cavitate, low/no avidity on PETCT
Dx: biopsy

24
Q

What is the most important factor in considering daptomyosin associated AEP?

A

Duration of therapy- mean onset being 2.8 weeks. Dramatic response to steroids (2-4w). Avoid future exposures

25
Q

What are the features of Kartagener syndrome?

A

Triad of situs inversus, chronic sinusitis, and bronchiectasis, as subgroup of PCD

26
Q

Describe the diagnostic guidelines for hypersensitivity pneumonitis

A

BAL lymphocytes more than 30%, positive antigens, three density pattern on CT, centrilobular GGO, head cheese sign on imaging for fibrosing HP, cryobiopsy has greater yield but may not always be necessary, low CD4/CD8 ratio

27
Q

What are the features of idiopathic inflammatory myositis?

A

Histo:
Features: fever, rash, proximal muscle weakness, dysphagia
Imaging: patchy GGOs
Dx: elevated CRP, aldolase, PL-7 antisynthetase antibody, Ro antibody, ANA often negative
Tx: steroids, immunosuppression

28
Q

Why are there so few patients with advanced pulmonary sarcoidosis receiving lung transplant?

A

Likely due to the higher mortality. Will also exhibit higher PGD, 30 day mortality, prolonged ventilation/LOS but with similar long-term outcomes and median survival

29
Q

What treatment options are there for PAP?

A

Previously only whole lung lavage, but now nebulized GM-CSF augmentation has proved to improve DLCO, CT findings, and dyspnea. Testing for GM-CSF antibodies are both sensitive and specific, and serum GM-CSF is usually normal

30
Q

Match CT findings of ILD with histology

A
31
Q

What are the features of bleomyocin toxicity?

A

Risks: older age, cumulative dose, renal dysfunction, previous/synchronous radiation, high supplemental oxygen, GCSF.

1-6 months after treatment, bilateral reticular/interstitial opacities with GGOs, fibrosis over time

Treat with 1mg/kg of prednisone and taper over several months

32
Q

What are the features of dermatomyositis associated ILD?

A

Features: hyperkeratosis, periungual erythema with dilated nail fold capillaries, fever, minimal myopathy
Dx: anti-MDA5

33
Q

What are the features of pulmonary lymphomatoid granulomatosis?

A

Histo: polymorphic lymphocytic infiltrate, angiitis, central necrosis
Features: mid age, male predominance, cough, dyspnea, fevers, malaise, weight loss, hemoptysis with cavitation, skin papules in glutes and extremities, low level EBV viremia in immunosuppressed patients
Imaging: peribronchovascular nodules, coarse irregular opacities, small cysts, cavitary masslike opacities that wax and wane over time
Dx: surgical lung biopsy, grade 1-3 based on amount of lymphocytes
Tx: stop triggering immunosuppressive medication, RTX with grade 3 disease

34
Q

Other than lung transplant, what conditions are associated with constrictive/obliterative bronchiolitis?

A

Inhalational injury, infections, systemic disease, hematopoietic transplant

35
Q

How can refractory chronic eosinophilic pneumonia be treated?

A

High dose ICS, omalizumab, mepolizumab, benralizumab, and dupilumab have been tried. Unfortunately relapse is common

36
Q

Why would you choose pirfenidone over nintedanib?

A

Nintedanib is metabolized by CYP3A4, can’t use with other CYP3A4 inducers (carbamazepine, phenytoin, St John’s wart)

37
Q

Describe testicular nodules in GU sarcoidosis

A

Nontender, discrete and palpable. Can have coexisting sarcoid and cancer so still need testicular biopsy.

38
Q

What are some additional therapies for ICI pneumonitis that is not responding to steroids?

A

Infliximab, cyclophosphamide, MMF

39
Q

What are the stages of ABPA?

A

Stage I- acute (elevated IgE, asthma, eosinophilia, opacities, antibodies), Stage II- remission, stage III- exacerbation, Stage IV- steroid dependent asthma, stage V-fibrosis

40
Q

How is ABPA treated?

A

Steroids, +/- itraconazole for 16 weeks (vori has been used), limited evidence for omalizumab, standard asthma treatment

41
Q

What are some of the more common triggers od drug induced AEP?

A

Daptomycin, NSAIDs, amiodarone, MTX, cocaine/heroin inhaled (over 140+ medications reported)

42
Q

What are the features of idiopathic AEP?

A

Histo: diffuse alveolar damage, eosinophils
Features: young and healthy, recent smoking, WTC, vaping, febrile illness of 5-7d can rapidly progress
Imaging: diffuse alveolar or mixed alveolar and interstitial opacities
Dx: BAL eos >25%, peripheral eos not present early on
elevated BAL IL5/VEGF/IL18, serum IgE
Tx: steroids 2-8w, relapse is rare

43
Q

What are the features of chronic eosinophilic pneumonia?

A

Histo: eos, BO, microabscesses, or noncaseating granulomas
Features: middle aged women, 1/2 with asthma, subacute to chronic presentation with fever, night sweats, weight loss, cough and wheezing, high peripheral eos above 30%
Imaging: peripheral based opacities, “photographic negative of pulmonary edema” but seen in less than 1/2 patients
Dx: BAL high, elevated IgE
Tx: steroid for 3-6 months with frequent relapse

44
Q

What are the features of idiopathic hypereosinophilic syndrome?

A

Features: young to middle aged males, multisystem syndrome with also cardiac/skin/GI/neuro involvement
Dx: unexplained Eos 1500 for more than 6 months or 2 exams more than 1 month apart PLUS some sign of end organ dysfunction
Tx: steroids, IFN-alpha, chemo, hydroxyurea, imatinib

45
Q

What are the features of EGPA?

A

Histo: necrotizing eosinophilic vasculitis, granulomas
Features: middle age, subacute (3 phases- asthmatic–>eosinophilic–>vasculitic), mononeuritis multiplex, sinus disease, skin involvement in 1/2, DAH is rare (less than 5%), cardiomyopathy in 12%, P-ANCA in 40-60% and may correlate with vasculitis, controversy if LTRAs cause versus unmask
Dx: 4/6 criteria: asthma, eosinophilia, paranasal sinus abnormalities, mononeuropathy, migratory pulm opacities, extravascular eos on biopsy
Tx: steroids, maintenance with RTX, AZA, MTX, leflunomide, mepo. Relapse is uncommon, refractory can try cyclophosphamide, plasmaphoresis

46
Q

What are the features of pulmonary Langerhans cell histiocytosis?

A

Histo: bronchocentric destructive Langerhans cells (CD1a, langerin, S100)
Features: young adult smokers, can have PTX, bone lesions in 10%, rarely DI
Imaging: irregular cysts, basilar sparing, nodules +/- cavitation, reticular opacities
Dx: imaging, BAL >5% CD1a cells, TBBx
Tx: smoking cessation, BRAF inhibitors if patient has BRAF-V600E mutation, cobimetinib, cladribine, cytarabine, MTX, can be used, screen for PH, lung Tx. Has increased risk of malignancy

47
Q

Which tumors should patients with Birt-Hogg-Dube syndrome be screened for?

A

Renal tumors. Will also have fibrofolliculomas which are benign

48
Q

What are the characteristics of the cysts seen in Birt-Hogg-Dube syndrome?

A

Varying sizes and shapes, favoring medial and lower lung fields

Pulmonary Boards (14 decks)

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