Pulmonary tuberculosis presenting with aortic pseudoaneurysm, diffuse alveolar hemorrhage, and cardiomyopathy: Is there an intriguing relationship?

doi:10.4236/crcm.2013.29129

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Vol.2, No.9, 495-498 (2013) Case Reports in Clinical Medicine

http://dx.doi.org/10.4236/crcm.2013.29129

Pulmonary tuberculosis presenting with aortic

pseudoaneurysm, diffuse alveolar hemorrhage, and

cardiomyopathy: Is there an intriguing relationship?*

Min Shen1, Xuejun Zeng2#, Xingrong Liu3, Bao Liu4, Baotong Zhou5, Xue Lin6, Ran Tian6

1Department of Rheumatology, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences (CAMS)

& Peking Union Medical College (PUMC), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education,

Beijing, China

2Department of General Internal Medicine, PUMCH, CAMS & PUMC, Beijing, China;

#Corresponding Author: [email protected]

3Department of Cardiac Surgery, PUMCH, CAMS & PUMC, Beijing, China

4Department of Vascular Surgery, PUMCH, CAMS & PUMC, Beijing, China

5Department of Infectious Diseases, PUMCH, CAMS & PUMC, Beijing, China

6Department of Cardiology, PUMCH, CAMS & PUMC, Beijing, China

Received 29 October 2013; revised 27 November 2013; accepted 10 December 2013

permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In accordance of

ABSTRACT

A 22-year-old Ch inese man presented with fever,

cough, hoarseness, neck pain, acute heart fail-

ure and hemoptysis. Pulmonary tuberculosis

was proved by sputum culture. Chest imaging

showed an aortic arch pseudoaneurysm and

bilateral ground glass opacities. Echocardi-

ography confirmed cardiomyopathy. With anti-

TB drugs, high-dose prednisone and surgery,

the life of this patient was successfully pro-

longed for more than four months. The con-

comitant disorders of aortic pseudoaneurysm,

alveolar hemorrhage and cardiomyopathy in

pulmonary tuberculosis are intriguing. We pos-

tulate that immune-mediated small vessel vas-

culitis triggered by pathogens plays an impor-

tant role in the pathogenesis of this disease,

rather than a direct TB infection.

Keywords: Pseudoaneurysm; Vasculitis; Thoracic

Aorta

1. INTRODUCTION

Tuberculous pseudoaneurysm is a rare but fatal condi-

tion that is invariably indicative of disseminated tuber-

culosis (TB), which is usually diagnosed with evidence

of TB infection at other positions and fever and persis-

tent pain, besides para-aortic mass [1]. TB is rarely asso-

ciated to diffuse alveolar hemorrhage (DAH), and to our

knowledge, pulmonary TB has been reported only twice

as the cause of DAH [2].

Here, we report a culture-proven pulmonary TB patient

concomitantly presenting with aortic pseudoaneurysm,

DAH and cardiomyopathy, which has not been previ-

ously reported. The intriguing relationship among pseu-

doaneurysm, DAH, cardiomyopathy and TB will be dis-

cussed in this report.

2. CASE PRESENTATION

A 22-year-old Chinese man was admitted to our hos-

pital, complaining of fever and cough for more than one

month, aggravated in the recent 10 days. He also had

fatigue, anorexia, and weight loss, without night sweat,

rash, or arthralgia. Ten days before admission, he began

to suffer from lower limb pain, and was unable to walk.

He experienced progressive shortness of breath, hoarse-

ness, cough productive of blood-tinged sputum, palpita-

tion, oppressive pain of left side of the neck, and left

upper extremity weakness. He was healthy before this

admission. One of his classmates was diagnosed with

pulmonary TB previous year. His mother had a history of

pulmonary TB when she was five. He denied smoking

and drug abuse.

*Competing interests: The authors declare that they have no competing

interests.

M. Shen et al. / Case Reports in Clinical Medicine 2 (2013) 495-498

496

On admission he looked distressed, with a heart rate of

128 bpm, bilateral blood pressure of 140/82 mm Hg, and

oxygen saturation of 92% under normal conditions.

Physical examination revealed diminished breath sounds

and rales in the right lower lung. Neurological examina-

tion was normal. Laboratory results showed a WBC

count of 5.3 × 109/L (4.0 - 10.0 × 109/L), hemoglobin

126 g/L (120 - 160 g/L), and platelet count of 374 ×

109/L (100 – 300 × 109/L). Urinalysis and renal, hepatic,

and coagulation profiles were normal. Serum investiga-

tions found a raised erythrocyte sedimentation rate (ESR)

and C-reactive protein (CRP). Antinuclear antibodies,

antiphospholipid antibodies and antineutrophil cytoplas-

mic antibodies were negative. He tested negative for the

purified protein derivative of tuberculin (PPD), but his

results of Mycobacterium tuberculosis (MTB)-specific

IFN-γ release assays were as high as 3620 SFCs/106

PBMC. Bronchoscopy was conducted with normal find-

ings. Multiple sputum sample smears found no evidence

for bacterial, mycobacterial, or fungal organisms.

His condition worsened at night on the admission day,

and he presented with severe cough, and orthopnea with

hemoptysis. His pulse rate was 170/min, blood pressure

150/100 mm Hg, respiratory rate 35/min, and his oxygen

saturation was only 90% under reservoir oxygen mask

with velocity of flow 10 L/min. Chest examination re-

vealed widespread rales of bilateral lungs. Laboratory

testing showed an obvious drop of hemoglobin level

from 126 g/L to 101 g/L, and elevation of brain natri-

uretic peptide (BNP) (1441 ng/L; reference value 0 - 100

ng/L), while cardiac enzymes were normal. Chest CT

scan (Figure 1) showed an aortic arch pseudoaneurysm

with a 1.2 × 3.0 cm defect in the lateral wall and mural

thrombus. Meanwhile, high-resolution CT revealed bi-

lateral areas of ground glass opacities. Echocardiography

showed a decreased cardiac systolic function, suggestive

of cardiomyopathy with an ejection fraction of only 48%.

Sputum cultures grew Mycobacterium tuberculosis

after his admission. The patient was treated with a stan-

dard therapy with rifampicin, isoniazid, ethambutol,

pyrazinamide, and moxifloxacin. In addition, corticos-

teroids were given on the same day because vasculitis

was suspected. His symptoms resolved in the first week

while chest CT cleared in about 10 days (Figure 1). His

left ventricular ejection fraction increased to 60%. Two

weeks later, he suddenly developed severe pain in the

chest and lower extremities with high blood pressure,

while the blood pressure of lower limbs could not be

measured. Repeat CT revealed a further increase in the

size of the pseudoaneurysm (Figure 1). The patient un-

derwent an emergency surgery, with exclusion of the

pseudoaneurysm. The pathology of the tissue of the aor-

tic pseudoaneurysm revealed a creeping rupture of the

media of the wall, and focal inflammatory cell infiltra-

(a)(b)

(c)(d)

Figure 1. (a) High-resolution CT reveals bilateral ground glass

opacities and pleural effusion. (b) Longitudinal contrast-en-

hanced CT scan shows a pseudoaneurysm arising from the

aortic arch, with mural thrombus. CT scan also shows a 1.2 ×

3.0 cm defect in the lateral wall of the aortic arch (arrow), and

the pseudoaneurysm compresses the adjacent aortic arch, left

pulmonary artery, and left main bronchus. (c) High-resolution

CT reveals that the lungs get clear after 10 days therapy of anti-

TB drugs and high-dose steroids. (d) Longitudinal contrast-

enhanced CT scan performed three weeks later (after image

shown in A) reveals a further increase in the size of the pseu-

doaneurysm, with the defect in the lateral wall of the aortic

arch increasing to 1.5 × 3.7 cm (arrow).

tion at the adventitia, without caseous necrosis. In addi-

tion, the culture of the tissue was negative for any organ-

isms. The anti-tubercular chemotherapy was continued,

with corticosteroids tapered gradually. After 19 weeks of

the operation, the patient had sudden onset hematemesis

and hypovolemic shock, and died soon thereafter. The

postmortem could not be performed.

3. DISCUSSION

A patient who developed aortic pseudoaneurysm,

DAH, cardiomyopathy, and pulmonary TB is described

in this report. Other infectious, malignant, or immu-

nological etiologies for the development of aortic pseu-

doaneurysm, DAH, and cardiomyopathy were excluded

after extensive studies. We therefore think that these dis-

orders were the complications of TB infection. The con-

comitant appearance of all the unusual complications in

one patient has not been reported in literature.

So far, TB as the cause of pseudoaneurysm has been

only occasionally reported, with less than 50 cases since

1945 in the literature in English [1,3-7]. Typical clinical

scenarios of tuberculous aortic pseudoaneurysm include

evidence of TB infection elsewhere in the body, with 1 or

M. Shen et al. / Case Reports in Clinical Medicine 2 (2013) 495-498 497

more of 3 presentations: fever and persistent pain related

to the location of the pseudoaneurysm, dysphagia, or

hoarseness; evidence of massive bleeding; or pulsative,

rapidly expanding para-aortic mass [1]. In our patient,

pseudoaneurysm with pulmonary TB should raise the

suspicion of tuberculous aortic pseudoaneurysm. But our

case was unusual because neither anti-fast bacilli nor

caseous necrosis was detected from the tissue of the aor-

tic pseudoaneurysm. Therefore, the cause of pseudoaneu-

rysm in our patient might have been secondary to vascu-

litis which was strongly related to the TB infection, as

could be seen in the relation between TB and Takayasu

arteritis [8], or that between TB and Behcet disease [9].

In our patient, the diagnosis of DAH relied on clinical

(hemoptysis and dyspnea), laboratory (rapid drop of he-

moglobin), and typical radiological findings. The possi-

ble mechanisms for TB associated DAH include infec-

tion related diffuse alveolar damage, immune-mediated

process, and antiphospholipid antibodies [2,10]. Coinci-

dentally, cardiomyopathy was also confirmed by echo-

cardiography. The causal link between TB, DAH and

cardiomyopathy was strongly supported by the prompt

clinical response to anti-TB therapy and corticosteroids.

Indeed, the clinical outcomes claimed that immune-me-

diated damage caused by TB infection might have played

an important role in the pathogenesis of DAH and car-

diac involvement in this patient.

Considering the above findings, aortic pseudoaneu-

rysm, DAH and cardiomyopathy in this TB patient were

most likely caused by immune-mediated vasculitis rather

than direct TB infection, though we did not find a first-

hand evidence of vasculitis. The patient should have

been treated with anti-TB drugs and steroids, coupled

with surgery as soon as possible. Unfortunately, the case

was complicated with DAH and cardiomyopathy at the

time of admission, because of which definitive surgical

treatment could not be instituted on time. Emergency

operation thereafter helped extend his life for more than

four months, but he eventually expired, most probably

owing to the perforation contained in the exclusion

pseudoaneurysm into the gastrointestinal tract.

To our knowledge, this is the first reported case of ac-

tive pulmonary TB complicated with aortic aneurysm,

DAH, and cardiomyopathy in an immune-competent

patient. However, because of a lack of previously pub-

lished experience on diagnosis and therapy, we were

confronted with enormous difficulties. With prompt com-

mencement of anti-TB therapy, high-dose prednisone,

and surgical treatment, it is possible to offer the best

chance for a cure and constitute the only way to salvage

patients struck with this disease. Medical management

alone or surgical management alone is not adequate. As

the prevalence of TB is rising worldwide, we hope this

report will increase alertness in physicians to the com-

plications of TB infection.

4. CONSENT

Written informed consent was obtained from the fami-

lies of the patient for publication of this case report and

accompanying images. A copy of the written consent is

available for review by the Editor-in-Chief of this journal.

5. AUTHORS’ CONTRIBUTIONS

MS was involved in the conception of the report, lit-

erature review, manuscript writing and submission. XZ

was involved in the conception of the report, manuscript

critique and review. XL and BL performed the surgery

and involved in the manuscript critique and review. BZ,

XL and RT were involved in the diagnosis and treatment

course, manuscript critique and review. All authors read

and approved the final manuscript.

6. ACKNOWLEDGEMENTS

This work was supported by the Clinical Research Funds of Chinese

Medical Association (grant number 12040760376), and the Youth Re-

search Funds of Peking Union Medical College Hospital (grant number

2010103).

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LIST OF ABBREVIATIONS

DAH: diffuse alveolar hemorrhage;

TB: tuberculosis;

ESR: erythrocyte sedimentation rate;

CRP: C-reactive protein;

BNP: brain natriuretic peptide.