ILD

Question 1

Lung biosy finding of Hermansky Pudlak Syndrome

Answer 1

Fibrotic pattern

Alveolar macrophages may contain cytoplasmic ceroid-like inclusions

Question 2

Granulomatosis with polyangiitis

Answer 2

Wegener syndrome

Question 3

Eosinophilic granulomatosis with polyangiitis

Answer 3

Churg-Strauss

Question 4

Rare form of ILD
Some have autoimmune disease or dysproteinemia
Reported in Sjögren syndrome and HIV infection

Answer 4

Lymphocytic Interstitial Pneumonitis

Question 5

Scattered late inspiratory high pitched rhonchi heard in bronchiolitis

Answer 5

Inspiratory squeaks

Question 6

Lab findings in ILD

Answer 6

Identification of ANA and anti- Ig Ab (rheumatoid factors)

Raised LDH

Elevation of serum levels of ACE: sarcoidosis

Serum precipitins: hypersensitivity pneumonitis

Antineutrophil cytoplasmic or anti-BM Ab: vasculitides

Question 7

Spirometry done in ILD usually produce a restrictive defect with reduced TLC, FRC, RV, FVC.

What spirometry finding is usually normal or increased?

Answer 7

FEV1/FVC

Question 8

Resting ABG in ILD

Answer 8

Normal
Hypoxemia (secondary to mismatching of ventilation and perfusion)
Respiratory alkalosis

Normal arterial O2 tension (or saturation by oximetry) at rest does not rule out hypoxemia during exercise or sleep

Question 9

Cardiopulmonary exercise findings in ILD

Answer 9

Arterial oxygen desaturation

A failure to decrease dead space appropriately with exercise (i.e. a high VD/VT or dead space/tidal volume ratio)

Excessive increase in RR with a lower than expected recruitment of TV

6-min walk test is used to obtain a global evaluation of submaximal exercise capacity in patients with ILD

Question 10

Most effective method for confirming the diagnosis and assessing disease activity

Answer 10

Lung biopsy

Question 11

Initial procedure of choice

Answer 11

FOB with multiple transbronchial lung biopsies (4-8 biopsies)

Question 12

Mainstay of therapy for suppression of the inflammation present in ILD

Answer 12

Glucocorticoids

But success rate is low

Optimal dose and proper length of therapy is not known

Common starting dose: prednisone 0.5-1 mg/kg OD based on patient’s lean body weight x 4-12 weeks after which patient is reassessed.
If the patient is stable or improved, the dose is tapered to 0.25-0.5 mg/kg and is maintained at this level for an additional 4-12weeks

Question 13

Autosomal recessive disorder
Granulomatous colitis and ILD may occur
Oculocutaneous albinism
Bleeding diathesis secondary to platelet dysfunction
Accumulation of chromolipid, lipofuscin material in cells of the reticuloendothelial system

Answer 13

Hermansky Pudlak Syndrome

Question 14

Radiograph of pulmonary alveolar proteinosis

Answer 14

Bilateral symmetric alveolar opacities located centrally in middle and lower lung zones resulting in a “bat wing” distribution

Question 15

HRCT of PAP

Answer 15

Ground-glass opacification and thickened intralobular structures and interlobular septa