Diseases of the Pleura

Christopher N. Faber, M.D.

Outline

Physiology of the pleura Pleural effusions Neoplastic disease of the pleura Pneumothorax Chylothorax, pseudochylothorax, and hemothorax

Key Points

Differentiation between transudates and exudates. The characteristic pleural findings for specific diseases (i.e. CHF, SLE, RA, tuberculosis,) Differentiation and management of parapneumonic effusions (there are 3 types). Causes and diagnosis of neoplastic disease of the pleura Causes and management of the various types of pneumothorax Causes and management of chylothorax Causes and management of hemothorax

Some important numbers

Size of pleural effusion to be seen on the chest film 200cc Preferred size of effusion before thoracentesis - > 10 mm wide in the lateral decubitus view

Pleural Histology

From: Cretien, J, Bignon, J., Hirsch, A, eds: The Pleura in Health and Disease. New York: Marcel Dekker, 1985, p174-175.

Physiology of the Pleural Space

From: Cretien, J, Bignon, J., Hirsch, A, eds: The Pleura in Health and Disease. New York: Marcel Dekker, 1985, p182.

Causes of Pleural Effusion

Congestive heart failure Pneumonia Malignancy Pulmonary embolism Viral Cirrhosis with ascites GI disease Collagen-vascular disease Tuberculosis Asbestos Mesothelioma

500,000 300,000 200,000 150,000 100,000 50,000 25,000 6,000 2,500 2,000 1,500

Light,RW: Pleural Diseases (3rd) edition, Philadelphia: Lea & Febiger, 1995, p 76

Diagnostic evaluation of the pleura

Radiography Thoracentesis Video-assisted thoracic surgery (thoracoscopy) Thoracotomy

Differentiation of transudates and exudates

Transudates < 0.5
Pleural Fluid

Exudates > 0.5

Pleural/serum Protein
Pleural/serum LDH Pleural LDH

< 0.6 < 2/3 the upper limit for serum

> 0.6 >2/3 the upper limit for serum

Transudative Pleural Effusions

Congestive heart failure Pericardial disease Hepatic hydrothorax Nephrotic syndrome Urinothorax Myxedema Pulmonary embolism (sometimes)

Exudative Pleural Effusions

Parapneumonic effusions Tuberculous Fungal Viral Parasitic Pulmonary embolism Abdominal disease Collagen vascular disease Post cardiac injury Post CABG Asbestos

Parapneumonic Effusions
Parapneumonic effusions

Complex

Simple

Empyema

Characteristics of a Complicated Parapneumonic Effusion

Glucose < 60 mg/dL pH < 7.2 Positive culture Pleural LDH > 3x the upper limit for serum Pleural fluid is loculated

Empyema

Pus in pleura space Positive gram stain

Management of Parapneumonic Effusions

Simple Antibiotics Complicated Antibiotics plus tube thoracostomy Empyema Tube thoracostomy and possible decortication

Tuberculous Pleuritis

Acute illness 2/3 of cases; chronic illness in 1/3 Unilateral effusion 1/3 will have parenchymal disease Exudative, lymphocyte predominant effusion

Diagnosis of Tuberculous Pleuritis

PPD may be negative in up to 30% Culture Pleural fluid for

Adenosine deaminase Interferon-gamma Polymerase chain reaction (PCR) for

tuberculous DNA

Biopsy

Treatment for Possible Tuberculous Pleuritis

Treat for tuberculosis

If lymphocyte-predominant exudate and: The adensoine deaminase, polymerase chain reaction, or interferon gamma is positive in the absence of rheumatoid arthritis or empyema ( Light, RW, Broaddus, VC. Pleural Effusion. In Murray & Nadel: Textbook of Respiratory Medicine, 3rd ed. W.B. Saunders Company, 2000. PPD is positive (Ansari, T, Clin Chest Med, 1998)

Actinomyces

Gram positive, filamentous bacteria Characteristically spreads through anatomic barriers and forms fistulae More that 50% have pleural involvement Can extend to the chest wall and form a pleural-cutaneous fistula Characteristic sulfur granules Treat with penicillin

Nocardia

Gram-positive filamentous rods, weakly acid fast Pleural involvement in 50% of cases Patients are frequently immunocompromised (but not necessarily) Diagnosis by culture Treatment with sulfonamides

Asbestos-related Pleural Disease

Benign asbestos pleural effusion (10-20 year latency) Pleural plaques (20-30 year after latency) Mesothelioma (30-40 year latency) Diffuse pleural fibrosis Rounded atelectasis

Asbestos-related Pleural Plaques

Asbestos-related Pleural Plaques

Collagen-Vascular Disease of the Pleura

Rheumatoid Arthritis Systemic Lupus Erythematosis Sarcoidosis Wegeners Granulomatosis Sjogrens syndrome

RA and SLE
Characteristics
Incidence Sex

RA

SLE

Effusion Glucose
C4 Pleural immunology Treatment Response

3%-7% 15%-44% 80% male Female 80% with SQ nodules Exudate Exudate < 20 mg/dl 63% > 70 mg/dl < 50 mg/dl 83% Low Low RF + LE cells or + ANA NSAID/Steroids Steroids Variable response Excellent

Neoplastic disease of the pleura

Lung Breast Lymphoma Ovary Stomach Unknown

36% 25% 10% 5% 2% 7%

Sahn, SA: In Fishman, JA 9ed): Fishmans Pulmonary Diseases and Disorders, 3rd ed. McGraw Hill, NY, 1998

Clinical Manifestations of Plural Metastasis

Symptom

Dyspnea Cough Weight loss Chest pain Malaise Fever Chills Asymptomatic

Patients with symptom (%) 57 43 32 26 22 8 5 23

Chernow, B., Sahn, SA., Am J Med, 1977

Mechanism of malignancyassociated plural effusion

Direct metastasis Lymphatic obstruction Bronchial obstruction with atelectasis Post obstructive pneumonia Thoracic duct involvement Pericardial disease Hypoproteinemia Pulmonary embolism Radiation therapy Chemotherapy (methotrexate, procarbazine, cyclophophamide, mitomycin, bleomycin)
Sahn, SA, Clin Chest Med, 1998 Light, RW, Pleural Disease, Philadelphia, Lea&Febiger, 1983

Characteristics of Malignant Pleural Effusion

Usually exudative (though occasionally transudative) Mononuclear cell predominant (lymphocytes, macrophages, and mesothelial cells) 1/3 will have low pH (less than 7.3)
Sahn, SA, Clin Chest Med, 1998 Good, TJ, et al: American Review of Respiratory Disease, 1985

Diagnosis of Malignant Pleural Effusion

Pleural fluid cytology Pleural biopsy Thoracoscopy

Treatment of Malignant and Paramalignent Pleural Effusions

Serial thoracentesis Chest tube with pleurodesis Thoracoscopy with talc poudrage Pleuroperitoneal shunt Pleurectomy

Survival with Malignant Pleural Effusion*

Primary Breast Lymphoma Lung GI Other Total
*time in months
Sahn, SA, Annals Internal Medicine, 1988

Total 14 7 5.3 3.8 6.3 7.3

Low pH 3.5 1.7 2.4 1.2 1.8 2.1

Normal pH 16.6 8.8 6.8 5.2 17.5 9.8

Mesotheolioma

Associated with asbestos exposure (even very modest exposures)

Latency of 35-40 years

No association with smoking Difficult diagnosis by cytology. Therefore, usually a biopsy is recommended Three histological subtypes
Epithelial Sarcomatous Mixed

Treatment of Mesothelioma

Extrapleural pneumonectomy
5% surgical mortality Median survival of 21 months (best with

epithelial histology) 5 year survival 22%

There may be a role for multimodality therapy using chemotherapy and radiation therapy

Benign Mesothelioma

Localized pleural tumors of mesenchymal origin Clinical manifestations

Asymptomatic in 50% Cough, chest pain, dyspnea in 40% of symptomatic

patients 2 paraneoplastic syndromes

Hypoglycemia caused by secretion of insulin-like growth factor II Hypertrophic pulmonary osteoarthropathy

Solitary mass Usually cured by surgical removal

Pneumothorax

Primary spontaneous pneumothorax Secondary pneumothorax Iatrogenic pneumothorax Traumatic Catamenial

Primary Spontaneous Pneumothorax

Felt to arise from sub pleural blebs Associated with smoking Patients tend to be taller and thinner Usually occurs when the patient is at rest Diagnosis confirmed by chest x ray
% pneumothorax= 100[1-lung3/hemithorax3)

Recurrence rate of 39% on ipsilateral side and 15% on contralateral side

Second recurrence rate of 50%

Treatment of Primary Spontaneous Pneumothorax

Observation Supplemental oxygen Simple aspiration Chest tube Thoracoscopy, bleb resection, and pleurodesis (usually reserved for recurrent disease)
Recurrence rates of 3-4 % after thoracoscopy

Secondary Spontaneous Pneumothorax

Etiology
COPD Cystic fibrosis

Interstitial lung disease such as sarcoidosis or

eosinophilic granuloma Pneumocystis

Recurrence rates higher that for primary spontaneous pneumothorax

Treatment of Secondary Pneumothorax

Chest tube Pleurodesis with first event with or without thoracoscopy

Iatrogenic Pneumothorax
Transthroacic needle aspiration 20% Mechanical ventilation Thoracentesis Central line placement Transbronchial lung biopsy

Treatment of Iatrogenic Pneumothorax

Minimal symptoms and less that 15% pneumothorax: observe Symptomatic or > 15 % : aspiration or chest tube For patients on mechanical ventilation: chest tube

Traumatic Pneumothorax

Penetrating or non-penetrating trauma 40% are occult to plain chest film and are discovered with CT Consider rare but catastrophic diagnoses that require immediate thoracotomy
Rupture of the trachea Rupture of the esophagus

Treatment is usually with a chest tube. If the pneumothorax is small and the patient is not in the ventilator, observation may be considered

Chylous Pleural Effusion

Defined by the presence of chyle (lymph) in the pleural space. Diagnosis

Appearance often milky. Must differentiate chylous from

chyliform effusion Chemical confirmation

Triglyceride > 110 mg/dL If triglyceride is between 50-110 mg/dL, send fluid for lipoprotein electrophoresis. Chylomicrons confirms a chylothorax If triglyceride is < 50, it is not chylous

Chyliform effusion has elevated cholesterol and occurs in long

standing effusions.

Causes of Chylous Effusion

Tumor
Lymphoma

54%
25%

Trauma
Surgical Other

Idiopathic 15% Miscellaneous 6%

Treatment of Traumatic Chylous Effusion

Pleuroperitineal shunts Chest tube: Caution that the patient may become malnourished. Therefore, chyle flow is reduced by GI rest and the use of parenteral nutrition Chemical pleurodesis Thoracotomy or thoracoscopy and ligation of the thoracic duct.

Treatment of Non-traumatic Chylous Effusions

Treat underlying lymphoma or carcinoma If ineffective, then insert a pleuroperitineal shunt

Lymphangioleiomyomatosis (LAM)

Rare disease of women of reproductive age A disease of proliferation of smooth muscle-like cells and affects the small airways, pulmonary microvasculature, and lymphatics Causes airway obstruction, cystic changes in the lung, and chylous effusions Typical patient is a young woman with interstitial disease on chest film, hyperinflated lungs (from air trapping) on pulmonary function, and a chylous effusion. Treatment with antiestrogen therapy (medroxyprogesterone, tamoxifen) or transplantation

Chyliform Effusions

Milky pleural fluid due to elevated cholesterol of lecithin-globulin complexes Most commonly associated with tuberculosis, rheumatoid arthritis, therapeutic pneumothorax

Hemothorax

Pleural fluid hematocrit greater that 50% that of peripheral blood Causes
Traumatic (penetrating or non-penetrating) Iatrogenic (thoracic surgery or line placement) Non traumatic (from metastatic pleural disease), spontaneous

rupture of an intrathoracic vessel, bleeding disorders Complication of anticoagulant therapy

Treatment is immediate chest tube (both to evacuate the fluid and monitor for additional bleeding)

Complications of Hemothorax

Retention of clotted blood in the thorax (causing restriction) Infection Effusion (usually self limited) Fibrothorax (occurs in less that 1% of hemothoraces. Decortication is necessary)

Decision Algorithm for Pleural Effusion

Type of effusion Type title here

Transudate

Exudate

CHF Hepatic failure

Renal failure

Infectious

Neoplastic

Bacterial

Tuberculous

Primary (Mesothelioma)

Secondary

Fungal

VIral
Lung Lymphoma Breast

Rheumatologic
Gastrointestional

Thromboembolic
Cardiac

Drug-related