Smoking related ILD

Smoking Related ILD Amr Badreldin Hamdy MD, FCCP 10/16/11 amr badreldin hamdy

They represent a heterogeneous group of lung disorders, generally characterized by dyspnea, dry cough, diffuse interstitial infiltrates, restrictive lung function pattern, and impaired gas exchange.
10/16/11 amr badreldin hamdy

The majority of ILD are of unknown cause, and known causes include gases, fumes, drugs, radiation, infections, inorganic dusts…etc.

ILD positively associated with smoking:
1. UIP/idiopathic pulmonary fibrosis.
2. Desquamative interstitial pneumonia.
3. Respiratory bronchiolitis-associated interstitial lung disease.
4. Pulmonary Langerhan’s cell histiocytosis.

Respiratory bronchiolitis is extremely common in cigarette smokers (smoker’s bronchiolitis). Bronchiolitis is a generic term used clinically to describe various inflammatory diseases of small airways.

It usually occurs without symptoms or significant interstitial lung disease.
It may account for sub-clinical radiological changes in up to one fifth of smokers.

Although respiratory bronchiolitis occurs in virtually all smokers it is of little clinical significance in the vast majority of cases.

ILD negatively associated with smoking:
1. Hypersensitivity pneumonitis (exogenous allergic alveolitis).
2. Sarcoidosis.

1. Occupational/Environmental.
2. Granulomatous diseases.
3. Iatrogenic/drug induced.
4. Collagen-vascular diseases.
5. Inherited.
6. Unique entities.
7. Idiopathic interstitial pneumonia.

Without a complete thorough clinical evaluation, all ILD are of unknown cause.

Idiopathic Pulmonary Fibrosis 10/16/11 amr badreldin hamdy

The role of smoking in the pathogenesis of IPF is controversial. It appears to increase the risk of development of IPF, but there is no evidence that smoking per se directly leads to the development of IPF.

Usually sporadic or familial.
Male to female ratio 2 to one.
Bilateral reticular or reticular-nodular opacities with small lung volumes.
Typically lower zone and peripheral predominance in the distribution.

HRCT shows irregular linear opacities and honeycombing, predominantly in the base and sub-pleural lung.

PFT usually demonstrates a restrictive defect with reduced lung volumes and diffusing capacity.
Exercise-induced de-saturation is almost always seen.

In patients with pre-existing emphysema, the lung volumes and flow rates may be normal due to counteracting physiological effects of emphysema and fibrosis.
In such patients, PFT may only reveal a severely reduced diffusing capacity.

Desquamative Interstitial Pneumonia 10/16/11 amr badreldin hamdy

Ninety percent are smokers or ex-smokers.
May occasionally be seen in association with systemic disorders or infections, as well as exposure to occupational/environmental agents and drugs.

Average age of onset is 40 years.
Male predominance (2:1).
Inspiratory crackles are heard in 60%.
Digital clubbing in nearly 50%.

On chest X-ray lung volume appears reduced unless there is co-existent OAD such as smokers with emphysema.

Predominant finding by HRCT is the presence of areas of ground-glass attenuations, typically sub-pleural and lower lung zone predominance.
Honeycombing is usually not present.

R-BILD 10/16/11 amr badreldin hamdy

It is a clinicopathological entity seen almost exclusively in current or former smokers.

PFT may be normal, but more commonly show a mixed obstructive-restrictive pattern of a mild-to-moderate degree.
Reduced diffusing capacity is common.
TLC may be normal, mildly increased or mildly reduced.

Diffuse, fine reticular or reticular-nodular opacities are present in more than 2/3.
Ground-glass pattern may be the predominant abnormality.
There is no honeycombing (DD IPF).

Areas of ground-glass attenuation are the most common finding (smoker’s alveolitis).
Micro-nodules may be present ( = respiratory bronchiolitis).

Pulmonary Langerhan’s Cell Histiocytosis 10/16/11 amr badreldin hamdy

The isolated pulmonary form in adults occurs almost exclusively in cigarette smokers. Adult PLCH represents a polyclonal, reactive disorder triggered by cigarette smoking.

Most patients are young adults (30-40y).
Sex distribution is equal.
Ninety or more are current or previous cigarette smokers.
The bronchiolar distribution of pathological lesions is consistent with the possibility that an inhaled antigen is involved in the pathogenesis of this disorder.

Physical examination frequently normal.
Cystic bone lesions in 10% (skull, ribs, pelvis).
Diabetes insipidus in 10%.

PFT show both obstructive and restrictive changes (effects from cigarette smoking may be superimposed and difficult to distinguish from effects of PLCH itself).

Typical finding on CXR include nodular or reticular-nodular opacities most prominent in the middle and upper lung zones, usually sparing of the costo-phrenic angles
Lung volumes appear normal or increased.

HRCT show thin-walled cysts, nodules (with or without cavitation) or a combination of nodules and cysts.

Prognosis 10/16/11 amr badreldin hamdy

IPF
Response to steroids is poor.
Prognosis is poor with no possibility of complete recovery.

DIP
Most patients remain stable or improve with corticosteroid therapy and complete recovery is possible.

R-BILD
Good prognosis, particularly with smoking cessation.
Good response to corticosteroid therapy and complete recovery is possible.

PLCH
Smoking cessation may prevent progression of the disease.
Response to steroids is fair.
Complete recovery is possible.
Prognosis is good.

Conclusion 10/16/11 amr badreldin hamdy

Smoking history is important in ILD.
Quitting smoking is important in ILD.
ILD may accompany COPD.
HRCT may be of help in patients with COPD not responding to usual broncho-dilator therapy. They may need to add corticosteroids to their regimens.

This may explain why so many patients with COPD need corticosteroids in their treatment protocol.
The incidence of smoking in the smoking related ILD reaches 90%, the same incidence as for COPD.

THANK YOU 10/16/11 amr badreldin hamdy

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Smoking related ILD

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Smoking related ILD — Presentation Transcript