From applied radiology

By Ugur Gonlugur, MD; Melih Kaptanoglu, MD; Tanseli Gonlugur, MD

Volume: 36 Number: 4 April 2007

Summary:

A 23-year-old housewife presented with a history of breathlessness, cough, night

sweats, weakness, anorexia, weight loss for 1 month, and fever of 2 days
duration. She had bacillus Calmette-Gurin (BCG) scars on her left upper
extremity but no smoking history. The patient had a temperature of 38.5 to 39C,
pallor, dyspnea, and general malaise. There were cervical, submandibular,
supraclavicular, and axillary lymphadenomegaly on physical examination,
crackles in both lungs on pulmonary examination, and hepatosplenomegaly on
abdominal examination. Erythrocyte sedimentation rate was 57 mm/h,
hemoglobin 7.3 g/dL, white blood cell count 7190/mm3, serum albumin: 22 g/L
(normal 35 to 55), alanine aminotransferase 50 U/L, aspartate aminotransferase
86 U/L, lactate dehydrogenase 805 U/L (normal 230 to 460), alkaline
phosphatase 304 U/L (normal 42 to 141), gamma-glutamyl transpeptidase 223
U/L (normal 8 to 35), total bilirubin: 1.1 (normal 0 to 1), and C-reactive protein 99
mg/L (normal 0 to 8). Electrocardiography showed sinus tachycardia. Oxygen
saturation was 76% in pulse oximetry. Serological tests for human
immunodeciency virus, cytomegalovirus, syphilis, and toxoplasmosis were
negative. Thyroid function tests were within normal ranges. Antinuclear antibody,
anti-ds-deoxyribonucleic acid, rheumatoid factor, and antithyroglobulin antibody
were negative. The parenchyma of the spleen was heterogeneous on abdominal
ultrasonography. Brain computed tomography (CT) was normal, and no
neurological decits were observed. Her chest X-ray and CT revealed
disseminated miliary shadows in both lung elds, which was highly suggestive of
miliary tuberculosis (Figure 1). There were no acid-fast bacilli in her sputum, but
the histopathologic examination of her cervical lymph node revealed caseating
granulomas.

Diagnosis:
Bilateral spontaneous pneumothorax in a patient with miliary pattern tuberculosis

Imaging Findings:
In addition to oxygen therapy, treatment with a combination of isoniazid,
rifampicin, ethambutol, and pyrazinamide was started on the third day of
hospitalization. On the fth day of antituberculous treatment, her fever had not
yet decreased, and methylprednisolone (1 mg/kg) was added to the therapy.
Multiple blood cultures were negative. No pathogens were identied in aerobic
cultures of sputum or urine. Wright and Rose Bengal agglutination tests for
brucellosis were negative. The serum levels of CA-125 and CA-15.3 were high,
but gynecological examination and thyroid and mammary ultrasonography
showed no signs of malignancy. No beroptic bronchoscopy or other invasive
procedures were performed because of respiratory failure. On the 38th day of
hospitalization, she complained of pleuritic chest pain, and chest CT revealed a
left-sided pneumothorax (Figure 2). A tube thoracostomy was placed, but after 1
week she developed a new pneumothorax on the other side (Figure 3). The
patient was managed with bilateral tube thoracostomy, and both lungs were
expanded. Her high temperature, which was occasionally subfebrile, continued
for 2 months in spite of systemic corticotherapy. In the fth month of therapy, the
tumor markers (CA-125, CA-15.3) returned to normal levels. The treatment
(antituberculosis drugs and corticosteroids) was continued for 12 months, but the
miliary shadows did not disappear. Her forced vital capacity (FVC) was 55%, her
forced expiratory volume in 1 second (FEV1) was 58%, and her FEV1/FVC was
93% in pulmonary function tests. Diffusion capacity was 65%, but the patient was
not a candidate for an invasive diagnostic procedure at this time.

Discussion:
Miliary pattern and pneumothorax are rare radiological features in pulmonary
tuberculosis. Their incidences are nearly 1.3% and 1.5%, respectively.1 However,
the coincidence of pneumothorax on miliary tuberculosis is a very rare event. We
found only 9 well-documented cases in the English literature.2-7 The age
distribution of these patients showed adult predominance (Table 1), which
supported the ndings of other studies.8,9 Table 1 also illustrates that the
male/female ratio was 4:6. However, this rate was 3:1 in all causes of secondary
spontaneous pneu-mothorax.10

The pathogenesis of pneumothorax in miliary tuberculosis is unclear, but the

following mechanisms can be considered: caseation or necrosis of subpleural
miliary nodules and their subsequent rupture can cause pneumothorax. On the
other hand, acute miliary dissemination may lead to emphysematous changes.
This mechanism may explain the bilateral, simultaneous, and/or recurrent
pneumothoraces.2,4 Despite this knowledge, it is not clear why a pneumothorax
complicating miliary tuberculosis is commonly left-sided.

It has been noted that surgical pleurectomy should be attempted early in

simultaneous bilateral secondary spontaneous pneumothorax.5 In miliary
tuberculosis, open thoracotomy should not be considered until the patient has
received antituberculous therapy for at least several weeks. The initial treatment
for nearly every patient with a secondary spontaneous pneumothorax should be
tube thoracostomy.

In this patient, at the end of the twelfth month of antituberculous therapy with
corticotherapy, the miliary pattern was unchanged and her diffusion capacity was
reduced, despite the presence of good performance status. These ndings
clearly suggested an interstitial lung disease, such as histiocytosis X or
Langerhans cell his-tiocytosis. Pulmonary histiocytosis X is an uncommon,
smoking-related, interstitial lung disease that primarily affects young adults.
Spontaneous pneumothorax, which may be recurrent, is a recognized feature of
this disease and likely results from the destruction of lung parenchyma with
associated cysts or nodules (with or without cavitation). Prior to a biopsy
procedure, a high-resolution chest CT can be helpful in the diagnostic evaluation.
The natural history is variable, with some patients experiencing spontaneous
remission of symptoms and others progressing to end-stage brotic lung disease.
There is no clear benet for either corticosteroids or cytotoxic agents.11,12

CONCLUSION

The possibility of a histiocytosis X should be considered in a young adult with a

miliary pattern that is unresponsive to a long duration of antituberculosis drugs
and corticosteroids, particularly in the absence of neurologic ndings.
Tuberculosis can accompany an interstitial lung disease that is characterized by
a miliary pattern. In such a condition, fever may not respond to corticosteroid
therapy. Finally, tumor markers may be used in the follow-up of tuberculosis.

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