DIFFUSE LUNG DISEASE - Nusrum



DIFFUSE LUNG DISEASE

Interstitial Lung Disease

It is the result of injury to structures in the alveolar space, interstitial space, or both

PATHOLOGY

Alveolar cell injury

Altered permeability in the alveolocapillary inter phase

Alveolar exudation

Infiltration of interstitial spaces by mononuclear cells, neutrophils and other cells

Increase in the thickness of the pulmonary interstitium

Capillary damage causes increased permeability and edema formation

Interstitial fibrosis

PHYSIOLOGY “Restrictive Defect”

Lung Volumes - decreased

TLC, VC, and RV decreased

Airflow rates - near normal

Lung compliance is decreased and elastic recoil is increased

DLCO is decreased early in the course

Exercise induced hypoxemia

Hypocarbia in early stages

Hypercarbia in late disease

(Respiratory Exhaustion)

ROENTGENOLOGY

HRCT Scan:

Shows characteristics findings

Pulmonary Langerhans Cells Granulloma:

Nodular cystic spaces in the upper lung field

Idiopathic Pulmonary Fibrosis:

Subpleural honey combing

Lymphangioleiomyomatosis

Well defined cystic spaces in the lung parenchyma

CXR:

Alveolar pattern

Interstitial pattern

combination of alveolar - interstitial patterns

CLASSIFICATION OF DIFFUSE

Pulmonary Diseases

IDIOPATHIC PULMONARY FIBROSIS

Cryptogenic Fibrosing Alveolitis

Rare Disorder

Diffuse Fibrosis throughout the Lung fields

Late middle age

Most common cause of chronic diffuse interstial Lung disease

Some studies indicate that Hepatitis C virus infection is an etiologic factor

PATHOGENESIS

Complex

Macrophages activated by several factors

Production of growth factors e.g. Fibronectin, platelet-derived growth factor (PDGF), TGT-B, IGF-I

Deposition of collagens type I and III

CLINICAL FEATURES

Progressive Breathlessness

Frequent Coughing

Fatigue

Anorexia

Weight loss

Arthralgias

PHYSICAL FINDINGS

Cyanosis

Tachypnea at rest

Clubbing of the fingers and toes

(without hypertrophic osteoarthropathy)

Dry crackles, or coarse crackles on inspiration (Auscultation), heard at the Lung bases

Accentuated pulmonic second sound

Signs of RHF

IMAGING

CXR- Diffuse reticular or reticulonodular markings (lower Lung zones)

High Resolution CT- “Ground Glass” appearance of the lower Lung fields (stage of alveolitis)

Nodular Infiltrates

Honey Combed or Swiss Cheese appearance (end stage disease)

Iinear opacities

Ring shaped opacities

Lung field contracted

LABORATORY EXAMINATION

ESR elevated

Circulating immune - complex titers 50%

Serum immunoglobins

Cryoimmunoglobulins

RF (Rhematoid factors) 30%

ANA (Antinuclear antibody) 35%

LUNG FUNCTION TESTS

Advanced Disease

TLC

VC

RV

FEV1/FVC Ratio-Normal or increased

DLCO - Reduced by 30% - 50%

Bronchoscopy

Sarcoidosis - Yield 80%

BAL Useful information about cells+proteins

LUNG BIOPSY

Histologic Evaluation

Good Microbial Cultures

Immunofluorescence and eleetnn-microscope studies

Analysis of inorganic substances

DIAGNOSTIC APPROACH

Occupational and environmental history

Prior chest films review

check for multi system disease process, new medication, neurologic status (aspiration and infection)

Chest X-ray / HRCT chest scan

Pulmonary function test / DLCO

Arterial Blood gases (desatuiration with exercise)

Fiberoptic bronchoscopy (1st invasive procedure)

Transbronchial biopsy / BAL

Imaged thorascopic or open lung biopsy

TREATMENT

Oral prednisone 1mg / kg / d * 8 week maintenance level 0.25 mg / kg / day * 6 months

Immosuppression with cyclophosphamide if disease is progressive (Addition) dose 1mg / kg / day

Pulsed doses of cyclophosphamide given biweekly

Azathioprine

Colchicine 0.6 mg / day (inhibit macrophage produced fibroblast growth factors)

Discontinue cigarette smoking

Supplemental oxygen therapy

Diureties

Bronchodilator’s

Narcotic containing antitussive medication

Management of infection during immno suppression

Prophylactic use of pneumococcal and influenza vaccines

Lung transplantation

IDIVIDUAL FORMS OF ILD

ILD associated with collagen vascular disorders

Systemic Lupus Erythematosus

Half of the patients with SLE ultimately develop overt lung disease

Pleuritis, pleural effusion, Acute pneumouitis (most frequent)

Chromic progressive ILD (uncommon)

abnormal DLCO

Lymphocytic alveolitis (better response to treatment)

End

RHEUMATOID ARTHRITS

Pleural disease (effusion and subpleural noduls)

Parenchymal nodular infiltrates

Diffuse interstitial fibrosis

ILD can develop before joint disease becomes evident (in men)

High titers of rheumatoid factor

Broncholitis obliterans with organizing puemonia have been reported

Patient who receives methotrexate or gold must be differentiated

Penicillamine therapy broncholitis obliterans

ANKYLOSING SPONDYLITIS

Bilateral upper lobe fibrosis

Fibrocavitary disease, may develop late in the course

SYSTEMIC SCLEROSIS

Radiographic evidence of lung involvement

Cutaneous scleroderma can involve the anterior chest wall and abdomen restrictive lung disease

SJOGREN’S SYNDROME

SYNDROMES OF ILD WITH PULMONARY HEMORRIAGE

Recurrent hemoptysis, dysprea and hypoxemia with diffuse alveolar opacities on chest radiography

SLE, wegener’s granulomatosis, behcet’s disease, allergic churg- strauss granulomatosis, Henoch- schonlein purpura, mixed cryoglobinemia

Serologic test ANA, Anti GBM antibody and complement

Renal biopsy and lung biopsy may be repaired for definite diagnosis

e-g

GOOD PASTUR’S SYNDROME

Pulmonary hemorrhage and glumerulonephritis are the features

Anti bodies to renal glomeraular and lung alveolar basement membranes

IDIOPATHIC PULMONARY HEMOSIDEROSIS

Diagnosis of exclusion

Lung biopsy is necessary (rule out) inflammatory injury)

Children and young adults are usually affected

Clinical course fulminant mild

Glucocorticoid treatment to control bleeding acutely

PULMONARY ALVEOLAR PROTEINOSIS

Alveoli are filed with grammar material that stains with periodic acid schiff reagent (PAS)

(Primary VS secondary)

secondary can be associated with inhaled dust exposure (silica and aluminium) malignancy, and chronic infection

intraalveolar material surfactant phospholid, LDH, proteins and Igs

whole lung lavage provides long term benefits

LYMPHOCYTIC INFILTRATIVE DISORDERS

Behave as low grade lymphoma

diffuse interstitial infiltration with lymphocytes and plasma cells

Autoimmune disease or dysproteinemia exists

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