T H E  S O U T H E R N  A F R I C A N  J O U R N A L  O F  H I V  M E D I C I N E                                    A P R I L  2 0 1 1

HIV AND BULLOUS LUNG DISEASE
Liat Malek, MB ChB

Grace Rubin, MB ChB, DA (SA), FCRad (D) SA
Susan Lucas, MB ChB

Department of Radiology, University of the Witwatersrand, and Helen Joseph Hospital, Johannesburg

The mechanisms behind accelerated emphysema in 
adults with HIV infection and the HIV-infected smoking 
population are both multifactorial and unclear. However, 
the association of HIV and emphysematous lung disease 
is recognised. We describe a patient with HIV infection 
and accelerated emphysema, highlighting the facts that 
no other background disease predisposed him to these 
lung changes, and that smoking in conjunction with HIV 
infection acted synergistically to produce the changes.

CASE REPORT
A 38-year-old man with a 3-day history of shortness of 
breath, productive cough and pleuritic chest pain presented 
to the medical department at Helen Joseph Hospital, 
Johannesburg. There was no history of tuberculosis or 
other lung disease, but the patient had been a smoker for  
10 years. He was on no medication. On examination he 
presented with a respiratory rate of 20/min, tachycardia 
(164 beats/min), and a blood pressure of 98/66 mmHg. 
He had decreased air entry on auscultation of the left 
chest. An initial chest radiograph revealed a differential 
transradiancy of the hemithoraces, and a diagnosis of 
spontaneous left pneumothorax was made. The patient 
was placed on oxygen and an intercostal drain (ICD) 
inserted. Blood tests revealed that he was HIV infected, 
with a CD4 count of 469 cells/µl and a white cell count 
of 469 x 109/l. He was not on antiretroviral therapy. Once 
his condition had stabilised, a high-resolution computed 
tomography scan of the chest was done (Figs 1 and 
2). This revealed large bilateral, diffuse, predominantly 
apical, medial and lateral bullous lung disease associated 
with a small residual left pneumothorax. The underlying 
lung parenchyma deep to the paraseptal bullae was 
not affected. No radiological features of pre-existing 
consolidation, cavities or cysts were present, and there 
were no nodules, reticules or bronchiectasis. There 
was no lymphadenopathy or effusions. The diagnosis of 
spontaneous pneumothorax secondary to bullous lung 
disease in an HIV-positive male smoker was made. 

Near-total re-expansion of the left lung occurred. The 
ICD was removed and the patient was discharged to an 
outpatient clinic.

DISCUSSION
Emphysematous lung disease has become a known 
pulmonary complication of HIV and AIDS. HIV-related 
bullous lung disease was first reported in the late 1980s. 
Subsequently numerous studies have indicated that the 
HI virus itself is a predisposing factor in the pathogenesis 
of bullous lung disease.1-3

In 1989 a publication by Kuhlman et al. compared 
the incidence of bullous lung damage in a group of 
HIV-positive patients with that in a similar group of 
immunocompromised patients with acute leukaemia.4 
Bullous lung damage was found in 42% of the HIV-
positive group, as opposed to 16% of the acute leukaemia 
group. The average age of the HIV-positive group was 
also significantly lower than that of the leukaemia 
group. The study documented the distribution of bullous 

Fig. 1. Coronal reconstruction of the lung parenchyma on 
lung windows. Large bilateral apical and medial paraseptal 
bullae are present. Note the absence of any parenchymal 

pathology deep to the paraseptal bullae. An intercostal drain 
tip is seen in the left lateral pleural space.

Fig. 2. Axial computed tomography scan on lung windows.  
Large bilateral paraseptal bullae are  demonstrated with 

residual antero-medial pneumothorax.

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A P R I L  2 0 1 1                                    T H E  S O U T H E R N  A F R I C A N  J O U R N A L  O F  H I V  M E D I C I N E

changes to be predominantly apical and peripheral. Of 
the patients 70% had a history of previous documented 
pulmonary infections (in particular Pneumocystis jirovecii 
pneumonia) and 13% did not, emphasising the direct 
effect of HIV itself on the lung parenchyma. Spontaneous 
pneumothoraces were a common complication in 
patients with bullous lung disease.

Other studies also emphasise the role of HIV in premature 
emphysema and bullous lung disease. HIV-infected 
subjects have significantly more emphysematous lung 
damage than HIV-negative smokers.1,5 HIV-associated 
emphysema also occurs over a much shorter period of 
time than smoking-related emphysema in HIV-negative 
patients. This is thought to reflect an increase in and a 
susceptibility to damage caused by cigarette smoking in 
HIV-positive patients.1

In 2000 Diaz and co-workers compared the incidence 
of emphysema in an HIV-positive group and an HIV-
negative group matched for age and smoking history.5 
The incidence of emphysema was 15% for the HIV-
positive and 2% for the HIV-negative group. Smoking 
was the single most important risk factor in the HIV-
positive group, contributing to 37% of the patients with 
emphysema in this group, compared with 0% in the HIV-
negative group.

The differential diagnosis of bullous lung disease includes 
tobacco smoking, intravenous drug use (methyphenidate, 
heroin, cocaine), marijuana and cocaine smoking, and a 
long list of diseases including α1-antitrypsin deficiency, 
HIV infection, auto-immune and connective tissue 
disorders, bullous sarcoidosis, idiopathic giant bullous 
emphysema and neurofibromatosis.2

A higher percentage of cytotoxic lymphocytes has been 
demonstrated in broncho-alveolar lavage specimens of 

HIV-infected patients than in uninfected subjects.1,5 In 
the 1990s reports of accumulation of CD8 cytotoxic T 
lymphocytes in the lungs of patients with severe chronic 
obstructive pulmonary disease (COPD) appeared. This 
may explain the accelerated emphysema in HIV-infected 
patients, whose lung response to the HIV infection is 
characterised by the accumulation of CD8 lymphocytes 
in alveolar spaces. Lymphocytic alveolitis, defined as 
more than 15% lymphocytes in broncho-alveolar lavage, 
is a common finding in HIV-infected subjects.1

More recently, highly active retroviral therapy (HAART) 
has been associated with a significant decrease in the 
number of CD8 cells in broncho-alveolar lavage. This 
raises the interesting possibility that the incidence 
of COPD in HIV-infected subjects may decrease 
significantly in the HAART era.1

CONCLUSION
The spectrum of HIV-associated accelerated 
emphysematous lung changes carries significant 
morbidity. It is therefore important to recognise early lung 
changes as well as understand the predisposing factors 
associated with accelerated bullous lung disease in the 
hope that prevention, early detection and treatment will 
decrease morbidity and mortality in these patients. 

REFERENCES
1. Petrache I, Diab K, Knox KS, et al. HIV associated pulmonary emphysema: a 

review of literature and inquiry into its mechanism. Thorax 2008;63:463-469.
2. Mireles-Cabodevila E, Sahi H, Farver C, Mohammed TL, Culver DA. A young 

patient with a minimal smoking history presents with bullous emphysema and 
recurrent pneumothorax. Chest 2000;132:338-343.

3. Crothers K. Chronic obstructive pulmonary disease in patients who have HIV 
infection. Clin Chest Med 2007;28(3):575-587. 

4. Kuhlman JE, Knowels MC, Fishman EK, Siegelman SS. Premature bullous 
pulmonary damage in AIDS: CT diagnosis. Radiology 1989;173:23-26.

5. Diaz PT, King MA, Pacht ER, et al. Increased susceptibility to pulmonary 
emphysema among HIV-seropositive smokers. Intern Med 2000;32:369-372.

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