Pulmonary involvement in HIV-Pulmonary involvement in HIV-associated Kaposi's sarcomaassociated Kaposi's sarcoma

基隆長庚醫院基隆長庚醫院呼吸胸腔內科呼吸胸腔內科姜伯穎 醫師姜伯穎 醫師

26 October, 200726 October, 2007

Kaposi's sarcoma (KS)Kaposi's sarcoma (KS)

the most common malignancythe most common malignancy associated with HIV infection associated with HIV infection

occurs in approximately occurs in approximately 6 to 20 percent6 to 20 percent of HIV-infected homo of HIV-infected homosexual or bisexual men and a small number of HIV-infected pasexual or bisexual men and a small number of HIV-infected patients from other risk groupstients from other risk groups

Kaposi's sarcoma-associated herpesvirusKaposi's sarcoma-associated herpesvirus or or human herpesvirus human herpesvirus 88, is responsible for the development of Kaposi's sarcoma, is responsible for the development of Kaposi's sarcoma

With prevention and control of opportunistic infections, patienWith prevention and control of opportunistic infections, patients with AIDS live longer, and ts with AIDS live longer, and disseminated malignancydisseminated malignancy has be has become one of the major causes of morbidity and mortality.come one of the major causes of morbidity and mortality.

Clinical Presentation of KSClinical Presentation of KS

varies from indolent skin lesions to extensive visceral varies from indolent skin lesions to extensive visceral diseasedisease

The The faceface and and mucous membranes of the oral cavitymucous membranes of the oral cavity ar are common sites of initial involvement.e common sites of initial involvement.

Major clinical problems encountered with KS are Major clinical problems encountered with KS are – local disfigurement, local disfigurement,

– disabling lymphedema, and disabling lymphedema, and

– extensive visceral involvement, extensive visceral involvement, with the with the gastrointestinal tractgastrointestinal tract and and lungslungs being the most common sit being the most common sites of diseasees of disease

Pulmonary KSPulmonary KSApproximately Approximately one-thirdone-third of KS patients will have of KS patients will have clinclinically evident pulmonary diseaseically evident pulmonary disease and and one-halfone-half have have ppulmonary involvement at autopsyulmonary involvement at autopsy. . Affected patients can present with Affected patients can present with shortness of breathshortness of breath,, feverfever, , coughcough, , hemoptysishemoptysis, or , or chest painchest pain, or , or with no sywith no symptoms but an abnormal chest radiographmptoms but an abnormal chest radiograph. . In patients with known KS who present with a respiraIn patients with known KS who present with a respiratory problem, up to tory problem, up to 50 percent50 percent are due to are due to parenchymaparenchymal involvement by KSl involvement by KS. . KS can involve the KS can involve the lung parenchymalung parenchyma, the , the airwaysairways, the , the pleurapleura, and , and intrathoracic lymph nodesintrathoracic lymph nodes..

Pulmonary KS – 1Pulmonary KS – 1Parenchyma Parenchyma – KS involves the lung typically in KS involves the lung typically in either an interstitial or noeither an interstitial or no

dular patterndular pattern. . – Parenchymal involvement is usually manifested clinically bParenchymal involvement is usually manifested clinically b

y y dyspneadyspnea, , hypoxemiahypoxemia, and , and dry coughdry cough. . HemoptysisHemoptysis, , feverfever, , and occasionally and occasionally respiratory failurerespiratory failure can also occur. can also occur.

Endobronchial lesionsEndobronchial lesions– Violaceous or bright red lesionsViolaceous or bright red lesions are found in some patients are found in some patients

on the mucosa of on the mucosa of the lower airwaysthe lower airways, especially at branchin, especially at branching points, or, much less commonly, in the upper airways. g points, or, much less commonly, in the upper airways.

– These lesions usually cause These lesions usually cause no symptomsno symptoms, although , although intractaintractable coughble cough, , hemoptysishemoptysis, , wheezingwheezing, , upper airway obstructionupper airway obstruction,, and and atelectasisatelectasis are occasionally seen. are occasionally seen.

Pulmonary KS – 2Pulmonary KS – 2PleuraPleura– Pleural effusions are radiographically visualized in Pleural effusions are radiographically visualized in up to two thirds of up to two thirds of

patients with parenchymal KSpatients with parenchymal KS and occasionally as an and occasionally as an isolated phenomeisolated phenomenonnon. .

– The effusion may be The effusion may be unilateral or bilateralunilateral or bilateral and and range from small to largrange from small to largee. They are . They are typically clear or serosanguineoustypically clear or serosanguineous and are and are almost always exalmost always exudatesudates. .

– Transudates and chylous effusions are rare.Transudates and chylous effusions are rare.– Many effusions are Many effusions are asymptomaticasymptomatic. . – However, However, chest pain can occurchest pain can occur and, when the effusions are large, respir and, when the effusions are large, respir

atory distress can be a serious problem. atory distress can be a serious problem.

Intrathoracic adenopathyIntrathoracic adenopathy– Enlarged mediastinal nodes have been reported in Enlarged mediastinal nodes have been reported in up to 46 percent of cup to 46 percent of c

asesases, although the origin of the nodes is seldom documented in these re, although the origin of the nodes is seldom documented in these reports. ports.

– The clinical significance of adenopathy in KS is related to the need to dThe clinical significance of adenopathy in KS is related to the need to distinguish KS from other diseases which can cause adenopathy, such as istinguish KS from other diseases which can cause adenopathy, such as infection.infection.

Chest Radiographic Features of Pulmonary KSChest Radiographic Features of Pulmonary KS

Variables Features

Parenchyma Reticulonodular infiltrates due to tumor nodules.

Diffuse interstitial infiltrates or linear/septal angiomatous infiltration.

Focal air space consolidation or collapse. Parenchymal lesions may appear normal, particularly in the early stages of disease.

Pleura Pleural effusions on one or both sides of the chest that may vary considerably in size.

Lymphadenopathy In advanced pulmonary KS, 10–20% of patients have enlarged hilar or mediastinal nodes.

Radiol Clin North Am 1997; 35:1029–1082Br J Hosp Med 1995; 53:344–350

PrognosisPrognosisThe median survival in HIV-infected patients with extensive pulmonary KThe median survival in HIV-infected patients with extensive pulmonary KS has been reported to be S has been reported to be two to ten monthstwo to ten monthsIn a retrospective review of patients treated with chemotherapy for extensiIn a retrospective review of patients treated with chemotherapy for extensive pulmonary KS, survival ve pulmonary KS, survival for responders was 10 monthsfor responders was 10 months versus versus six monthsix months for nonresponderss for nonresponders – Poor prognostic factors included:Poor prognostic factors included:

Presence of a pleural effusion Presence of a pleural effusion Severe shortness of breath Severe shortness of breath CD4 count below 100CD4 count below 100

Median survival in another study of 30 patients with symptomatic pulmonaMedian survival in another study of 30 patients with symptomatic pulmonary disease treated with chemotherapy was ry disease treated with chemotherapy was 6.5 months6.5 months..– Poor prognostic factors in this study were:Poor prognostic factors in this study were:

Absence of cutaneous KS Absence of cutaneous KS Presence of previous opportunistic infection Presence of previous opportunistic infection CD4 count below 100 CD4 count below 100 Hemoglobin below 10 g/dL Hemoglobin below 10 g/dL Lack of radiographic response to chemotherapyLack of radiographic response to chemotherapy

Am J Med 1989 Jul;87(1):57-61Am J Med 1989 Jul;87(1):57-61Thorax 1994 Oct;49(10):958-60Thorax 1994 Oct;49(10):958-60

TreatmentTreatmentTreatment is Treatment is palliativepalliative in almost all cases. in almost all cases. Although biological modifiers such as interferon alfa have a role early or laAlthough biological modifiers such as interferon alfa have a role early or late in the course of KS, te in the course of KS, pulmonary involvementpulmonary involvement usually requires usually requires chemotherchemotherapyapy..ChemotherapyChemotherapy– KS responds to a variety of chemotherapeutic agents, including vincristine, vinKS responds to a variety of chemotherapeutic agents, including vincristine, vin

blastine, bleomycin, doxorubicin, and paclitaxel.blastine, bleomycin, doxorubicin, and paclitaxel.– Highly active antiretroviral therapyHighly active antiretroviral therapy ( (HAARTHAART) is associated with both a ) is associated with both a decreasdecreas

ed proportion of new AIDS cases with KSed proportion of new AIDS cases with KS and a and a regression in size of existing regression in size of existing KS lesionsKS lesions..

Radiation therapyRadiation therapy– Radiation therapy has been successfully used to treat Radiation therapy has been successfully used to treat symptomatic airway obstrsymptomatic airway obstr

uctionuction in KS. in KS.– There is often significant improvement in dyspnea and hemoptysis but There is often significant improvement in dyspnea and hemoptysis but survival survival

is shortis short, , averaging 2.5 monthsaveraging 2.5 months in one series. in one series. Management of pleural effusionsManagement of pleural effusions– SclerosisSclerosis is usually tried if the effusions recur after thoracentesis, but it is is usually tried if the effusions recur after thoracentesis, but it is often often

ineffectiveineffective..– Thus, Thus, repeated thoracentesisrepeated thoracentesis is the modality most often used to is the modality most often used to relieve symptorelieve sympto

msms, frequently in combination with chemotherapy in an attempt to control the d, frequently in combination with chemotherapy in an attempt to control the disease.isease.