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S I N G A P O R E M E D I
C A L J O U R N A L
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Clinics in Diagnostic Imaging (27)
A N Leung
CASE REPORT
A 44-year-old Caucasian man presented with symptoms of weakness and
decreasing exercise tolerance. An electrocardiogram demonstrated a third-degree
heart block which was treated with placement of a dual chamber pacemaker.
Posteroanterior chest radiograph (Fig
1) and computed tomography (CT) of the thorax (Fig
2) were performed. What do these demonstrate? What further investigations
should be performed for confirmation of the diagnosis?
IMAGE INTERPRETATION
Chest radiograph (Fig 1) showed
right paratracheal, aortopulmonary window, and subtle bilateral hilar lymphadenopathy
associated with a diffuse reticulonodular parenchymal pattern. CT confirmed
the presence of mediastinal (Fig 2A)
and bilateral hilar lymphadenopathy (not shown); the diffuse micronodular
pattern predominated in the middle and upper lung zones (Fig
2B).
DIAGNOSIS
Sarcoidosis
CLINICAL COURSE
After placement of the pacemaker, all of the patient’s symptoms resolved.
Specifically, he denied fevers or any residual cardiopulmonary symptoms.
His measured angiotensin converting enzyme (ACE) level was normal. Transbronchial
biopsies were non-diagnostic. In order to justify treatment with corticosteroids,
definitive diagnosis was obtained via mediastinoscopy. Histopathologic
examination of biopsied lymph nodes revealed the presence of non-caseating
granulomas consistent with sarcoidosis.
DISCUSSION
Sarcoidosis is a multisystemic granulomatous disorder of unknown aetiology
that involves the lungs in 90% of patients(1).
Although thoracic manifestations are most common, virtually any organ system
may be affected(2).
Ocular, neurologic, and cardiac involvement usually require prolonged and
high dose of corticosteroid treatment(3).
Thoracic sarcoidosis may present with a myriad of radiographic findings
ranging from normal (Stage 0) to parenchymal fibrosis (Stage IV)(4).
The most characteristic radiographic finding is lymphadenopathy which is
present in approximately 50% of patients(4).
Radiologic accuracy and confidence in the diagnosis of thoracic sarcoidosis
has been improved by evaluation using high-resolution computed tomography
(HRCT)(5).
On HRCT, nodules are the predominant parenchymal lesion in sarcoidosis(6).
Nodules may range in size from a few mm to several cm; are typically irregular
in margination, and have a very distinctive distribution – along bronchovascular
bundles, interlobular septa, and subpleural regions – reflecting the pathologic
distribution of sarcoidosis granulomas along lymphatic pathways(7).
Sarcoidosis nodules are found predominantly in the upper and middle lung
zones. Intralobular reticular opacities, architectural distortion, and
traction bronchiolectasis/bronchiectasis are seen in patients who have
progressed to fibrosis.
Occasionally, the predominant parenchymal finding in sarcoidosis may
consist of single or multiple large nodules (> 1 cm), masses, or mass-like
areas of consolidation with cavitation or air-bronchograms (Fig
3). Histologically, these findings represent aggregations of sarcoidosis
granulomas sometimes present in association with surrounding fibrosis(8).
In the differential diagnosis, sarcoidosis (Fig
4) must be distinguished from two other nodular diseases – lymphangitic
carcinomatosis (LC) (Fig 5) and
lymphoma (Fig 6) – that also have
a perilymphatic distribution and are often associated with intrathoracic
lymphadenopathy. Often the clinical context, particularly if a previous
history of malignancy can be elicited, can help prioritise the differential
possibilities. Sarcoidosis is one of few pulmonary disorders in which the
radiologic extent of disease may be much more impressive than the patient’s
symptomatology. On HRCT, although all three of the previously mentioned
diseases may cause interlobular septal thickening, formation of well-defined
“polygonal arcades” occurs more frequently in the neoplastic diseases as
does focal disease restricted to one lobe or one side. Peribronchovascular
consolidation (Fig 6) is a characteristic
feature of lymphoma and may be so extensive as to mimic a pneumonia. HRCT
findings of fibrosis (architectural distortion, traction bronchiectasis,
intralobular reticular opacities, honeycombing) would favour sarcoidosis
as it does not occur in either LC or lymphoma.
In some patients, as exemplified by the reported case, the perilymphatic
distribution of sarcoid nodules may not be apparent on HRCT. The differential
diagnosis of disseminated micronodules (< 7 mm in size) with associated
lymphadenopathy includes pneumoconioses, miliary dissemination of infectious
granulomatous diseases including tuberculosis, lymphoma, and metastatic
disease from thyroid carcinoma, renal cell carcinoma, or poorly differentiated
adenocarcinoma from any site. Again the clinical context, particularly
with respect to exposure history or acuteness of illness, may be critical
in appropriately narrowing the differential possibilities.
The diagnostic criteria for sarcoidosis are well established and consist
of: 1) compatible clinical or radiologic evidence or both; 2) histologic
evidence of noncaseating granulomas, and 3) negative bacterial and fungal
studies of biopsied tissue or sputum(9).
Although radiologic findings may be consistent or highly suggestive of
the diagnosis, they are not pathognomonic and it is generally felt that
if treatment with corticosteroids is indicated, the diagnosis should be
confirmed with histology(10).
Additionally, tissue cultures should always be performed to exclude an
infectious granulomatous disease.
ABSTRACT
A 44-year-old Caucasian man presented with third-degree heart block.
Chest radiograph and high-resolution computed tomography (HRCT) of the
thorax showed mediastinal and bilateral hilar lymphadenopathy associated
with a diffuse, bilateral micronodular pattern. The HRCT findings and differential
diagnosis of sarcoidosis are reviewed.
Keywords: computed tomography, high-resolution; sarcoidosis
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