Interstitial Lung Diseases (ILD)

8/12/2019 Interstitial Lung Diseases (ILD)

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Interstitial Lung Diseases

Introduction

• Also known asDiffuse Parenchymal Lung Disease(DPLD).

• Heterogenous group of conditions causedby diffuse thickening of the alveolar walls

with inflammatory cells & exudate (ARDS),granulomas (Sarcoidosis), haemorrhage(Goodpasture’s syndrome) and / orfibrosis.

• Restrictive ventilatory defect.

• Slowly progressive loss of alveolarcapillary exchange units which ultimately

will lead to impairment of pulmonary gastransfer & arterial hypoxemia.

• In many cases eventually progresses torespiratory failure , pulmonary HTN &cor pulmonale.

Aetiology

1. Sarcoidosis.

2. Cryptogenic fibrosing alveolitis (CFA).

3. Exposure to organic dusts :Farmer’s lung.

4. Exposure to inorganic dusts : Asbestosis.

5. Pulmonary eosinophilia .

6. Collagen vascular diseases :SS, SLE, RA, Vasculitis.

7. Exposure to irradiation & drugs .

8. Miscellaneous : Alveolar proteinosis, Histiocytosis .

Conditions mimicking ILD

• Malignancies :Leukemia, lymphoma,Carcinomatosis lymphangitis,alveolar cell carcinoma, metastasis.

• Infections :Viral, miliary tuberculosis,histoplasmosis, P. carinii

Diagnosis• History :

Occupational / pets / smoking / drugs /family history.Symptoms- general, respiratory,

extrapulmonary.• Physical signs :

General, pulmonary, extrapulmonary.• Investigations :

Blood, Radiology (CXR, HRCT), PFT,

Histology.

• Also known as Idiopathic PulmonaryFibrosis (IPF).

• A common ILD of unknown cause.

• May be associated with systemicdiseases.

• About twice as common among cigarettesmokers than in non-smokers.

• Progressively downhill course withdevelopment of respiratory failure & deathwithin 5 years of diagnosis.

• Rapidly progressive form :Hamman – Rich syndrome .

• Disease of late middle age;M:F= 2:1.

Pathology

• Alveolitis :inflammatory cells in alveoli & interstitium.

• Fibrosis : variable involvement.- Initially patchy involvement,

- Late stage, lung is converted into cysticspaces of varying size.

Clinical features• Symptoms :

Progressive exertional dyspnea, dry coughGeneral - malaise, fever, wt loss, myalgia,

arthralgia.• Signs :

Majority - finger clubbing, cyanosis,

tachycardia, tachycardia. Almost all- superficial fine end inspiratory

crepitations at lung bases.

Diaphragm may be raised.

Investigations• Blood:

ANA & RF may be positive in low titers.ESR raised.

Hypergammaglobulinemia.• Arterial desaturation (hypoxemia withouthypercapnea) after exercise- may be anearly indication.

• PFT : Restrictive pattern- decreased TLC, VC, TV

- normal / increased FEV1/ VC.

Pulse oximeter

SPIROMETER

• CXR : Loss of lung volumeEarly – ground glass appearance;

Late – reticulonodular pattern ; Advanced – multiple cystic spaces

(honeycomb)

• HRCT- more sensitive method todemonstrate early disease, but lacksspecificity.

• BAL : increased cells; PMNL predominant.

• Open lung biopsy : Definitive diagnosis.

CFA- CXR

CFA- CT scan

Diagnosis

• Clinical• Investigations.

• Exclude- Pneumoconiosis.

- Collagen vascular disease.

Treatment

A. Drug therapy :1. Corticosteroids

Prednisolone:1 mg/kg body wt. for 6-8 wksgradually tapered to maintenancedose (10-12.5 mg daily).

Advanced disease/ elderly- poor response .Subjective improvement in majorityObjective improvement in minority.

• Second line drugs :1. Azathioprine- monitor blood count & LFT.2. Cyclophosphamide - monitor blood count.

Regimens :1. Azathioprine 100 mg daily +

Prednisolone 20mg OD on alternate days

2. Cyclophosphamide 2-3mg/ kg daily +Prednisolone

• B. Supportive therapy : Oxygenof cor pulmonale.

• C. Single lung transplantation :For young pts with advanced disease.

Prognosis

• Median survival time of untreated cases is4 years.

• In majority of pts the disease isprogressive, even in those who haveresponded to treatment.

• Approximately 50% survive for 5 years &

25% for 10 years.• Tenfold increase in the incidence ofbronchogenic carcinoma.

SARCOIDOSIS

• It is a multisystem granulomatous diseaseof unknown etiology, which proceed eitherto resolution or to fibrosis.

• Lungs are usually involved.

Prevalence & incidence

• Prevalent in West & Japan.

• Worldwide prevalence: 20 / 100000.

• Inversely related to TB.

• Blacks > Whites.

• Genetic- twins/ siblings.

Aetiology

• Unknown.

• Immunological response to someunidentified antigen in geneticallysusceptible individual.

Pathology

• Alveolitis – predominantly lymphocytic.• Non caseating granuloma.

• In 70% cases inflammatory changes stopearly & resolution occurs spontaneouslywithout sequelae;fibrosis occurs in 5-10% cases.

Sarcoidosis- Histology

Clinical features

• Asymptomatic: 20-30% cases.• Constitutional disturbances- fever,

malaise, fatigue, wt. loss, cough.

• Can affect virtually all organs & tissues ofthe body , except adrenal glands.• Lungs, eyes, skin & liver are most

frequently affected.• Alarming CXR findings, scanty physical

signs on auscultation of respiratorysystem.

Sarcoidosis- skin lesions

Sarcoid- nose

LUPUS PERNIO

Dactylitis

Investigations

• Blood :WBC count – N/ Increased (lymphopenia)ESR- moderate raise.

S. calcium – increased in 20-40% cases.Hypergammaglobulinemia with reversal of A:G.S. ACE- elevated in pts with active disease;

useful in f/up.

• Tuberculin test : 60% show negative result.

Strongly positive reaction excludes sarcoidosis.

• Biopsy- transbronchial lung / skin / LN.confirms the diagnosis.

• Kveim test : in cases where biopsy isdifficult / negative.

Intradermal injection of 0.1-0.2 ml of human

sarcoid tissue; positive test after 4-6 wks in theform of purplish red nodule at the site ofinjection.

• BAL : Increased lymphocytes (normal <10%)

• PFT : Restrictive pattern

• CXR : Thoracic sarcoidosis.Stage I : Bilateral Hilar Lymphadenopathy

with or without right paratrachealadenopathy; may be associatedwith erythema nodosum,

arthralgia , low grade fever;

Stage II : Pulmonary infiltrates coexist(often diffuse) with adenopathy .Gradual onset of exertional

dyspnea, cough.

STAGE III : Diffuse pulmonary infiltrates

without adenopathy;development of pulmonaryfibrosis (mid & upper zone).

Pts become breathless, hypoxic,ultimately develop cor pulmonale

• CT scan :early LN , parenchymal infiltration.

Stage I

SARCOIDOSIS- FIBROSIS

Treatment

• Symptomatic ; spontaneous resolution inmost cases.• Corticosteroids

Prednisolone: 0.5-1 mg / kg single dailydose for 4 wks; graduallytapered to maintenance

dose of 7.5-10 mg daily.Indications : Acute/ severe form, CNS,

eye, vital organ involvement.

Prognosis• Stage I : Spontaneous recovery within 1 yr

in majority.• Stage II : 50% resolve spontaneously within

2 yrs; 30-40% require steroid therapy;

small number develop progressivepulmonary fibrosis.

• Stage III: 30% show improvement with steroid

therapy; may eventually developpulmonary fibrosis & respiratory failure.

Terminally – Respiratory/ Renal/ Cardiac failure.

THANK YOU

Interstitial Lung Diseases (ILD)

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