ILD

Question 1

What is the hallmark of ILD?

Answer 1

Alveolar epithelial cell injury is a hallmark of ILD. The source of injury may be extrinsic, as in cases of HP, pneumoconiosis, or radiation pneumonitis.

Question 2

What are frequent complaints with interstitial pulmonary fibrosis?

Answer 2

Dyspnea, nonproductive cough, most common.
Uncommon chest pain.

Question 3

What is Collagen vascular disease?

Answer 3

Collagen vascular disease refers to a group of conditions that cause chronic inflammation in your connective tissues. Many of these diseases are also autoimmune diseases ( RA, scleroderma, lupus, sejogren)

Question 6

Medical term for velcro crackles?

Answer 6

Inspiratory crackles

Question 7

Testing for collagen vascular diseases

Answer 7

Antinuclear antibodies (ANA) and extractable nuclear antigens (ENA)

Rheumatoid factor (RF) and anticyclic citrullinated peptide antibodies (anti-CCP3)
Creatine kinase (CK), aldolase, and anti-Jo1 antibodies

Scl-70 and anticentromere antibodies

Double-stranded DNA antibodies

Myositis panel

Question 9

Testing for Hypersensitivity Pneumonitis

Answer 9

Serum precipitin testing in HP may be used to confirm the presence of serum antibodies against a specific antigen implicated as a causative agent of disease.

Question 10

ILD testing

Answer 10

PFT (Restrictive pattern specifically IPF)
However, the finding of restriction on PFTs is nonspecific and may be due to a number of causes including chest wall disease, obesity, neuromuscular disease, etc.

In reality, a significant number of ILDs show a mixed obstructive–restrictive pattern on pulmonary function testing.

A predominantly obstructive pattern may be seen in ILDs with small airways involvement including sarcoidosis, HP, and the smoking-related ILDs like PLCH, RB-ILD, and DIP.

Combined pulmonary fibrosis and emphysema (CPFE) may present with normal appearing PFTs, however, the diffusion capacity (DLCO) is universally decreased in these patients.

Question 11

CT findings

Answer 11

Honeycombing, GGO, linear or reticular markings, nodular marking,

Question 12

HRCT SCAN TO DIAGNOSE IPF it should show?

Answer 12

reticular infiltrates in a predominantly basilar and subpleural distribution

honeycombing with/without traction bronchiectasis

an absence of features inconsistent with the diagnosis of UIP (e.g., ground glass, upper lung predominance, cysts, mosaic attenuation, and micronodules).

Question 13

Ild exclusevly in smokers

Answer 13

PLCH, RB-ILD, and DIP occur almost exclusively in smokers

Question 15

Treatment

Answer 15

In general collagen vascular, hypersensitivity, and autoimmune diseases are treated with glucocorticoids and/or immunosuppressive agents with varying degrees of success.

Targeted therapies, such as granulocyte macrophage colony–stimulating factor (GM-CSF) for PAP may be considered.

There have been promising advances in the field of LAM, which is now treated with sirolimus in selected cases. Other drugs for the treatment of LAM are currently in clinical trials.

Pirfenidone is an antifibrotic agent that reduces fibroblast proliferation, inhibits collagen production, and reduces production of fibrogenic mediators. Primary side effects include photosensitivity and GI upset.

Nintedanib is a tyrosine–kinase inhibitor that targets vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), and platelet-derived growth factor receptor (PDGFR). Primary side effects include diarrhea, nausea, and other GI effects.