Interstitial Lung Diseases

Sevda Özdoğan MD, Prof.

Chest Diseases

Definition Interstitial lung diseases covers

a group of diffuse lung disorders that involve infiltration of alveolar air spaces or thickening of pulmonary interstitial structures

This heterogenious group of diseases have similar clinical, radiological and physiological characteristics

Alveolus

Interstitium

Type I pneumocyt

Type II pneumocyt

Capillair

Endotheel cel

AlveolusAlveolus

Interstitium

Type I pneumocyt

Type II pneumocyt

Capillair

Endotheel cel

Alveolus

Classification

Alveolar proteinosis,microlithiasis

Berilliosis, hypersensitivity pn, histiocytosisOccupational

radiation

40-50%

pneumoconiosis

Hypersensitivity pneumonitis

Collagen tissue diseases associated with ILD

SLE RA PSS Sjögren sydrome Polymyositis-dermatomyositis Mixed connective tissue disease Ankylosing spondylitis

Other Systemic Diseases

Sarcoidosis Vasculites (Wegener, Churg Strauss) Hemorragic Syndromes (Goodpasture,

Idiopathic pulmonary hemosiderosis) Hystiositosis X Chronic Gastric aspiration Lymphangitis Carcinomatosa Chronic pulmonary edema Chronic uremia Alveolary proteinosis

Epidemiology

The known insidance and prevalance rate of ILD depens on some reports which may not represent the population

Estimated prevalance in man is 80.9/100 000 population

Estimated prevalance in woman is 67.2/100 000 population

SYMPTOMS Dyspnea: Progressive, prominent in

exercise. Sudden worsening of dyspnea, particularly

if associated with pleural pain, may indicate a spontaneous pneumothorax.

Cough: A dry cough

Hemoptysis: Diffuse alveolar hemorrhage syndromes, Vasculitides

Churg strauss, Wegener Granulomatosis Behçet’s disease

Can be due to other complications Lung cancer Pulmonary tuberculosis

Symptoms of Right Hearth Failure

HISTORY

The cause of the illness is often recognized from the patient's history.

Entire lifelong employment Prior medication use Symptoms of a systemic disease

Musculoskeletal pain, weakness, fatigue, fever, joint pains or swelling, photosensitivity, dry mouth or eye

Gender:

Female

Lymphangioleiomyomatosis (Premenopausal)

pulmonary involvement in tuberous sclerosis

Connective tissue diseases

Male

ILD associated with rheumatoid arthritis

occupational exposures, pneumoconiosis.

Smoking history

Current or former smokers Hystiocytosis X DIP, IPF, RBILD

Never or exsmokers Sarcoidosis Hypersensitivity

pneumonitis.

Active smoking can lead to complications in Goodpasture's syndrome, in which pulmonary hemorrhage is far more frequent in current smokers.

Family history

Tuberous sclerosis, Neurofibromatosis

Idiopathic pulmonary fibrosis, Sarcoidosis

PHYSICAL EXAMINATION

Physical examination is usually nonspecific

Crackles or "velcro rales" are common in most forms of ILD, although they are less likely to be heard in the granulomatous lung diseases, especially sarcoidosis.

Scattered late inspiratory high-pitched rhonchi (inspiratory squeaks) are frequently heard on chest examination in patients with bronchiolitis.

Cyanosis Tachypnea Tachicardia Cor pulmonale (edema, hepatomegali) Extrapulmonary manifestations of

systemic diseases Erythema nodosum (sarcoidosis, tb) Telenjiectasis (scleroderma) Joint deformities (RA)

Clubbing: Common in IPF, Asbestosis

Diagnosis History

Chest radiography Chest x-ray (can be normal in 10%) HRCT

The routine laboratory evaluation is not much helpfull but should be done (biochemical and hematologic tests)

Chest x-ray

All available previous chest radiograms should be reviewed to ascertain the onset, progression, chronicity and stability of the disease Diffuse alveolar (aciner) pattern, lineer, nodular or reticulonodular infiltrations Cystic changes (honeycombing) can be

present

5-10 mm nodules aciner pattern

Cystic changes (Honeycombing)

Reticular patternNormal

The distribution of the lesions (Upper or lover zone distribution) can be usefull in narroving the differential diagnosis

The presence and the pattern of adenopathy may provide additional clues

HRCT More sensitive and specific than chest

radiographs Graund grass attenuation Lung consolidation Reticular opacities Nodules Cysts Fibrotic changes

Honey combing

Pulmonary Function Testing

A restrictive defect with reductions in total lung capacity (TLC), functional residual capacity (FRC), and residual volume (RV)

Flow rates are decreased (FEV1 and FVC), but the changes are in proportion to the decreased lung volumes; thus, the FEV1/FVC ratio is usually normal or increased

A reduction in the DLCO is a commonly found

Arterial Blood Gas analysis

The resting arterial blood gases may be normal or may reveal hypoxemia

A normal resting PaO2 does not rule out significant hypoxemia during exercise or sleep, which is common in ILD.

Carbon dioxide retention is rare and usually a manifestation of end-stage disease.

Invasive Diagnostic techniques FOB

Bronchoalveolar lavage (BAL) Transbronchial lung biopsy

VATS

Treatment

Avoid the exposed etiologic factor if there is any

Supportive care (Oxygen, pulmonary rehabilitation)

Treat the underlying disease if there is any

Corticosteroids Immunesuppressive treatment Lung transplantation

Sarcoidosis

Sarcoidosis is a multisystem granulomatous disorder of unknown cause(s). It commonly affects young and middle-aged adults and frequently presents with bilateral hilar lymph-adenopathy, pulmonary infiltration, ocular and skin lesions.

VII İnternational Sarcoidosis Meeting 1976

The histologic hallmark is noncaseating granulomas

Etiology

The cause of Sarcoidosis remains unknown

Despite the lack of specific infectious cause Tuberculosis Atipic mycobacteria Epstein Barr virus Fungal infections Mycoplasma are investigated

Organ involvement in sarcoidosis

OrganOrgan % % of patientsof patients

Mediastinal lymph nodesMediastinal lymph nodes 95-98%95-98%LungsLungs >90%>90%LiverLiver 50-80%50-80%SpleenSpleen 40-80%40-80%EyesEyes 20-50%20-50%Peripheral lymph nodesPeripheral lymph nodes 30%30%SkinSkin 25%25%Nervous systemNervous system 10%10%Heart (clinically)Heart (clinically) 5%5%

Chest x-ray

Bilateral hiler adenopathy

Bilateral hiler adenopathy+ paranchymal infiltrates

Chest radiographic stages of sarcoidosis

Stage Stage FrequencyFrequency

00 NormalNormal 5-10%5-10%

II BHLBHL 50%50%

IIIIBHL and parenchymal BHL and parenchymal infiltratesinfiltrates 25%25%

IIIIII Parenchymal infiltrates Parenchymal infiltrates without BHLwithout BHL 15%15%

IVIV Signs of fibrosisSigns of fibrosis 5-10%5-10%

HRCT

Hilar and mediastinal lymphadenopathy Beaded or irregular thickening of the bronchovascular

bundles Nodules along bronchi, vessels, and subpleural regions Ground glass opacification Parenchymal masses or consolidation Parenchymal bands Cysts Traction bronchiectasis Fibrosis with distortion of the lung architecture

Clinical Symptoms

2/3 of the patients are asymtomatic diagnosed in an incidental chest radiogram

Respiratory symptoms Dyspnea (marked on exertion)

Cough (nonproductive)

Chest discomfort (Chest pain often difficult to describe)

Chest tightness and Wheesing (In chronic fibrocystic sarcoidosis)

Systemic / constitutional symptoms

Fatigue (up to 70% of patients)

Fever (usually low-grade, but up to 40°C possible)

Weight loss(2-6 kg during 10-12 weeks)

Symptoms of involved system

Löfgren’s Syndrome

It is a well defined presentation of sarcoidosis that consists of Erythema nodosum Polyartritis Bilateral hiler lymphadenopathy

The unset is usually abrupt In most cases spontaneous remission

occurs

Heerfordt’s Syndrome

Also known as Uveoparotid fever Consists of Fever Anterior uveitis Parotid and lacrimal gland enlargement Bilateral hiler adenopathy Facial paralysis

Has an abrupt unset

Diagnostic Evaluation History (occupational and environmental exposure, symptoms) Physical examination Postero-anterior chest radiogaphy Pulmonary function tests: spirometry and CO diffusion capacity Serum chemistries: calcium, liver enzymes, creatinine, blood urea

nitrogen, ACE Urine analysis Electrocardiography Routine opthalmologic examination Tuberculin skin test Tissue biopsy

Bronchoscopic evaluation FOB can be usefull in tissue sampling by

Bronchial mucosal biopsy (there may be nodularity, mucosal edema, hypervascularity)

Transbronchial needle aspiration of the mediastinal lymph nodes

Transbronchial lung biopsy

BAL sampling Increased lymphocyts to 20-50% (<10% in normal

subjects)

The easiest accessible biopsy site should be preferred for tissue sampling A sklin nodule Superficial lymph node Nasal mucosa, salivary gland

Mediastinoscopy VATS or thoracotomy rarely needed

Treatment

>80% of the patients undergo spontaneous remission depending on the stage of the disease with the highest rate in the early stages

Which patients should be treated??

Treatment indications

Systemic symptoms (Fever, weight loss) Progressive symptomatic pulmonary

involvement (Dyspnea, cough, hypoxemia) Multipl vital organ involvement Myocardial involvement Eye involvement that is resistant to topical

treatment Santral Nervous system involvement Hypercalcemia

Corticosteroids minimum of 8-12 months

Supportive management of patients who have advanced disease and corpulmonale

Special topical treatments for extrapulmonary disease