DR Syed Aqeel Gilani Department of Pulmonology Ayub Teaching Hospital Abbottabad

Dr Syed Aqeel Gilani Department Of Pulmonology Ayub Teaching Hospital Abbottabad
Defining ILD
Classification Focus on IPF Clinical Presentation
Confirming the Diagnosis Management Medical Surgical Palliative Prognosis
Brief review of other common subtypes

Important Role of the PCP.
http://inspire.org.pk/
The interstitial lung diseases (ILDs) are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium leading to impaired oxygen transfer and ultimately scarring within the lung. In 2002, the American Thoracic Society (ATS) and European Respiratory Society (ERS) revised the original classification scheme and introduced the term diffuse parenchymal lung disease (DPLD) in place of the term ILD.
UIP is NOT a clinical entity but a specific radiological
and pathological pattern.

IPF (Idiopathic Pulmonary Fibrosis) is its clinical
counterpart; defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of UIP.
Epithelial fibroblastic disease Normal healing
reestablishing epithelium
IPF
Injury ....activation,migration,prolifer mesenchymal cells.fibroblast/myofibroblast..accumulation matrix.irreversible damage lung parenchyma

Chronic exertional dyspnea, dry cough,
bibasilar inspiratory crackles, and finger clubbing. Sixth and seventh decades. Men>Women History of cigarette smoking Prevalence estimate of between 14.0 and 42.7 per 100,000
CXR
Spirometry Bronchoscopy(BAL)
HRCT Carefull Hx,Exam to exclude secondary cause

Lung biopsy 6MWT
Subpleural, basal predominance
Reticular abnormality
Honeycombing with or without traction
bronchiectasis
Absence of features inconsistent with UIP pattern Upper/mid lung predominance Extensive groundglass opacities Micronodules/cysts/consolidations.
Exclusion of other known causes of ILD (e.g., domestic
and occupational environmental exposures, connective tissue disease, and drug toxicity).
The presence of a UIP pattern on HRCT in patients not
subjected to surgical lung biopsy.

Specific combinations of HRCT and surgical lung
biopsy pattern in patients subjected to surgical lung biopsy.

The committee did not find sufficient evidence to
support the use of any specific pharmacologic therapy for patients with IPF.
An Official ATS/ERS/JRS/ALAT Statement 2010: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management
Corticosteroid monotherapy. Except in acute exacerbations given high mortality

Colchicine.
Cyclosporine A.
Combined corticosteroid and immune-modulator
therapy. Interferon g 1b. Bosentan. Etanercept.

Acetylcysteine monotherapy.
Pirfenidone.
Treatment of asymptomatic GERD.
Acetylcysteine is a precursor to the antioxidant
glutathione,which may be reduced in the lungs of patients with IPF.

RCTs showing lower 12-month decline in vital capacity
and diffusing capacity.

No observed difference in mortality or other secondary
endpoints including dyspnea, quality of life, exercise physiology, or radiographic appearance.

Pirfenidone is a pyridone compound with pleiotropic, anti
inflammatory, antifibrotic, and antioxidant properties, with antagonism of TGF-b1 effects.
Mixed results from RCTs Two big studies suggesting possible reduced rate of lung
function decline, improved oxygenation and progression free survival.
Pirfenidone was associated with significant gastrointestinal
adverse events, liver laboratory abnormalities,photosensitivity, and rash. Recommended in japan

Abnormal acid gastroesophageal reflux is highly
prevalent in patients with IPF, and up to one half of patients are asymptomatic.
Two retrospective case series describe stabilization of
pulmonary function and oxygen requirements with medical and surgical management of GERD.
Asymptomatic gastroesophageal reflux disease should
be medically treated in the majority of patients with IPF.

PANTHER-IPF is a multi-center trial being conducted by The
National Heart, Lung, and Blood Institute (NHLBI), that tested prednisone, azathioprine, and N-acetylcysteine (NAC) triple therapy vs. placebo, and NAC alone vs. placebo.
NHLBI stopped the triple therapy study arm in October 2011
since participants treated with triple therapy had more mortality (11% v/s 1%), more serious adverse events, and more drug discontinuations, without evidence of benefit.
The results of PANTHER-IPF prednisone/azathioprine/NAC
combination therapy arm are expected to be available in spring 2012. The final PANTHER-IPF study results are expected to be published in 2013
Long Term Oxygen Therapy

Recommended but no disease specific survival data.
Pulmonary rehabilitation Mechanical Ventilation

Not recommended in majority.
Lung Transplant
Pulmonary rehabilitation programs involve aerobic
conditioning, strength and flexibility training, educational lectures, nutritional interventions, and psychosocial support.
Two controlled trials of pulmonary rehabilitation in
IPF have demonstrated an improvement in walk distance and symptoms or quality of life.
Five-year survival rates after lung transplantation in
IPF are estimated at 50 to 56%.

A single-center study of 46 patients referred for lung
transplantation with IPF demonstrated a reduced risk of death at 5 years in patients receiving lung transplantation.
Patients with pulmonary fibrosis undergoing lung
transplantation have favorable long-term survival compared with other disease indications.
DLCO < 39% predicted

A 10% or greater decrement in forced vital capacity
(FVC) during 6 months' follow-up FVC < 60-65% predicted Decrease in oxygen saturations < 88% during 6-minute walk test
Limited data suggest that corticosteroids and thalidomide
may be beneficial for chronic cough in IPF.

Chronic opioids may be used for severe dyspnea and cough. Advanced directives and end-of-life care issues should be
addressed in the ambulatory setting in all patients with IPF, particularly those with severe physiologic impairment and comorbid conditions.
In patients who are bedbound due to IPF, hospice care
should be considered.
Pulmonary Hypertension
Pulmonary Embolism Lung Cancer
CAD OSA
YOU MIGHT PREFER ONE OF THESE INSTEAD..
Similar clinical presentation as IPF
with cough and dyspnea for months to years.

HRCT shows diffuse lung
involvement consisting of peripherally located irregular linear opacities with ground-glass opacities and small cystic lesions (honeycombing is rare).
Steroid responsive. Five year survival >80%.
BEFORE
AFTER
Key imaging features for differentiation between UIP and NSIP
UIP
NSIP
DIP
RBILD
Respiratory Desquamative interstitial pneumonia http://inspire.org.pk/ bronchiolitis-associated interstitial lung disease
Both seen in current or former smokers. Typical presentation 4th to 5th decade. Sub acute (weeks-months) DIP is middle to lower lung pre dominant/RB-ILD is upper
lung pre dominant.

lesions.
Smoking cessation is important in the resolution of these
Questionable role of steroids and other
immunosuppressive agents.
Prognosis is good with 10year survival of >70%.

Idiopathic
Fifth to sixth decade
Men=Women
Sub acute 1-2m Flulike illness,cough, fever,
malaise, fatigue, and weight loss. Peripheral, basal predominant consolidation or ground glass opacities. Corticosteroid therapy results in clinical recovery in twothirds of the patients.
BEFORE
AFTER
Falls in the spectrum of
lymphoproliferative disorders that can involve the lung. Seen in association with AIDs,hypergammaglobulinemia, Sjgren's syndrome, other autoimmune conditions. Sub acute, over months. HRCT shows ground-glass attenuation, centrilobular nodules, cysts and interstitial thickening. Steroid responsive. May progress to pulmonary lymphoma.
BEFORE
AFTER
ILD AND THE CENTRAL ROLE OF PRIMARY CARE PHYSICIANS
Suspect
Chronic cough/SOB
Investigate
Pulmonary Function Tests (Spirometry/Lung Volumes and DLCO) CXR HRCT
Counsel
Smoking cessation Weight gain/loss (BMI requirement for lung transplant < 30 and > 17) Realistic expectations
Refer Remain Involved

Palliative care measures Discussions regarding mechanical ventilation/code status/end of life
care/hospice. Clinical awareness of co-morbidities such as CAD and pulmonary embolism.
DPLDs are a heterogeneous group of disorders characterized by inflammation
and/or fibrosis of the pulmonary interstitium.
Idiopathic interstitial pneumonias are a subgroup of this entity and include IPF, NSIP, RB-ILD, DIP, LIP, AIP and COP/BOOP.
IPF has a dismal prognosis and no approved pharmacological treatment to
date.
NAC and Perfinidone are used in selected cases. Early referral for lung transplant improved survival in progressive cases of IPF.
Smoking cessation is essential in management of DIP and RB-ILD. Steroids are beneficial in the management of NSIP, LIP and COP.
QUESTIONS?

DR Syed Aqeel Gilani Department of Pulmonology Ayub Teaching Hospital Abbottabad

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Dr Syed Aqeel Gilani Department Of Pulmonology Ayub Teaching Hospital Abbottabad

Brief review of other common subtypes

UIP is NOT a clinical entity but a specific radiological

and pathological pattern.

Epithelial fibroblastic disease Normal healing

Injury ....activation,migration,prolifer mesenchymal cells.fibroblast/myofibroblast..accumulation matrix.irreversible damage lung parenchyma

Chronic exertional dyspnea, dry cough,

HRCT Carefull Hx,Exam to exclude secondary cause

Subpleural, basal predominance

Exclusion of other known causes of ILD (e.g., domestic

subjected to surgical lung biopsy.

biopsy pattern in patients subjected to surgical lung biopsy.

The committee did not find sufficient evidence to

Corticosteroid monotherapy. Except in acute exacerbations given high mortality

therapy. Interferon g 1b. Bosentan. Etanercept.

Treatment of asymptomatic GERD.

Acetylcysteine is a precursor to the antioxidant

glutathione,which may be reduced in the lungs of patients with IPF.

and diffusing capacity.

endpoints including dyspnea, quality of life, exercise physiology, or radiographic appearance.

Pirfenidone is a pyridone compound with pleiotropic, anti

inflammatory, antifibrotic, and antioxidant properties, with antagonism of TGF-b1 effects.

function decline, improved oxygenation and progression free survival.

Pirfenidone was associated with significant gastrointestinal

adverse events, liver laboratory abnormalities,photosensitivity, and rash. Recommended in japan

Abnormal acid gastroesophageal reflux is highly

be medically treated in the majority of patients with IPF.

PANTHER-IPF is a multi-center trial being conducted by The

Long Term Oxygen Therapy

Pulmonary rehabilitation Mechanical Ventilation

Pulmonary rehabilitation programs involve aerobic

Five-year survival rates after lung transplantation in

IPF are estimated at 50 to 56%.

DLCO < 39% predicted

Limited data suggest that corticosteroids and thalidomide

may be beneficial for chronic cough in IPF.

YOU MIGHT PREFER ONE OF THESE INSTEAD..

Similar clinical presentation as IPF

with cough and dyspnea for months to years.

Key imaging features for differentiation between UIP and NSIP

Respiratory Desquamative interstitial pneumonia http://inspire.org.pk/ bronchiolitis-associated interstitial lung disease

lung pre dominant.

Smoking cessation is important in the resolution of these

Questionable role of steroids and other

Prognosis is good with 10year survival of >70%.

Falls in the spectrum of

ILD AND THE CENTRAL ROLE OF PRIMARY CARE PHYSICIANS

Refer Remain Involved

care/hospice. Clinical awareness of co-morbidities such as CAD and pulmonary embolism.

DPLDs are a heterogeneous group of disorders characterized by inflammation

and/or fibrosis of the pulmonary interstitium.

IPF has a dismal prognosis and no approved pharmacological treatment to

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