Diagnoses

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130

pulmonary hypertension. The marked proximal vessel nar-

rowing and the diminutive vessels at every level, associated

with discrete narrowings and post-stenotic dilatation are not

angiographic features commonly associated with chronic

thromboembolic disease (Case 1, Chap. 1). This is especially

the case when eccentric lining thrombus and intravascular,

recanalized clot are not evident on CT.Proceeding with con-

firmatory imaging of the aorta and the major vessels, even

without historical or exam evidence for vascular compromise

was appropriate and confirmed the diagnosis of major vessel

arteritis.

Points ofEmphasis

Historical evidence for peripheral arterial events, the pres-

ence of arterial bruits or diminished pulses, and atypical CT

or catheter-based angiographic findings, especially in young

female patients with pulmonary hypertension, should alert

the clinician to the possibility of pulmonary arteritis.

Diagnoses

1. Pulmonary arteritis and aorto-arteritis (Takayasu

arteritis).

2. WHO group V pulmonary hypertension.

Case Presentation

Fifty-two-year-old female with a vague medical history of a

nonspecific mixed connective tissue disease, and poorly char-

acterized lung nodules, experiencing years of exertional dys-

pnea. Progressing more rapidly over 6 months, she was

Chapter 14

Case 14: Sarcoidosis

andLarge Vessel

Pulmonary Vascular

Disease—Another

CTEPH Mimic

RobertSchilz, DeepaGopalan, andWilliamR.Auger

R. Schilz (*)

CWRU School of Medicine, Cleveland, OH, USA

Lung Transplantation and Pulmonary Vascular Disease, University

Hospitals of Cleveland, Cleveland, OH, USA

e-mail: Robert.Schilz@UHhospitals.org

D. Gopalan

Department of Radiology, Imperial College Hospitals, London, UK

e-mail: d.gopalan@nhs.net

W. R. Auger

Lewis Katz School of Medicine, Temple University,

Philadelphia, PA, USA

e-mail: Bill.Auger@tuhs.temple.edu

134

hospitalized for the treatment of a pericarditis and pleural

effusions. A month later, an echocardiogram revealed normal

left ventricular size and function; right ventricular size and

function were also assessed as normal, with an RV systolic

pressure estimated at 24mmHg. The pericardial effusion had

resolved.

With the development of sharp chest wall pains and persis-

tent dyspnea 4months later, a follow up chest radiograph was

obtained (Fig.14.1) and was without demonstrable cardio-

megaly, parenchymal infiltrates or pleural effusions.

F . PA chest radiograph demonstrating normal heart size,

symmetric hilar fullness, in the absence of significant parenchymal

abnormalities

R. Schilz et al.

135

CT angiogram of chest was then requested demonstrating

diffuse mediastinal and hilar “soft tissue fullness” consistent

with matted adenopathy was described; multifocal parenchy-

mal opacities were again demonstrated and there was no

evidence for acute pulmonary emboli (Fig.14.2).

Pulmonary function tests in April 2018: FVC 2.65L (78%),

FEV-1 2.20 L (81%), FEV-1/FVC ratio 83% with DLCO

18.65 (82% predicted).

Ongoing investigation of the patient’s symptoms prompted

a right heart catheterization in May 2018. Resting pulmonary

hemodynamics showed RA mean 3, PAp 39/12mmHg with a

mean pressure of 22mmHg. Fick cardiac output 5.2 L/min

with a cardiac index 3.0L/min/m

; PVR was 3.25Wood units.

A level 3 cardiopulmonary stress test later that same month

included rest and exercise pulmonary hemodynamics and full

gas exchange. A borderline elevated PA pressure at rest

(40/18mmHg, mean pressure of 25mmHg) showing a dra-

matic rise with exertion to 90/35 mmHg, 53 mmHg mean

(Table14.1).

In further evaluation of the abnormal pulmonary vascular

response to exercise, lung perfusion imaging was obtained

(Figs.14.3 and 14.4).

F . Extensive soft tissue in the subcarinal space and sur-

rounding the proximal pulmonary vessels

Chapter 14. Case 14: Sarcoidosis andLarge Vessel...

136

Given the concern for the possibility of chronic thrombo-

embolic disease, catheter-based pulmonary angiography was

obtained (Fig.14.5).

With concerns over the persistent perfusion defects on

VQ scan, significant functional limitations, and the atypi-

cal presentation, a referral was initiated for a second

opinion.

F . Bilateral unmatched perfusion defects on lung scintig-

raphy

T . Summary of level 3 cardiopulmonary exercise testing

Rest Peak VO

RAP 3 8

PAP (systolic/diastolic/mean) 40/18/25 90/35/53

CO/CI 3.7/2.1 9.3/5.3

PAO P 9 14

HR 70 163

BP 116/64 150/90

SpO

100% 100%

R. Schilz et al.

137

Given the extent of mediastinal and hilar tissue, and the

absence of definable pulmonary intravascular findings to sug-

gest organized thrombus, biopsy of the subcarinal/mediasti-

nal tissue was recommended. This yielded non-caseating

F . Spectral imaging demonstrating the relationship

between compressive adenopathy and perfusion defects

F . Web-like narrowing of a right upper lobe artery (open

arrow), with an vessel narrowing over a considerable length of the

proximal descending pulmonary arteries, bilaterally (closed arrows)

Chapter 14. Case 14: Sarcoidosis andLarge Vessel...

138

granulomas; all microbiologic stains and titers were negative

consistent with the diagnosis of sarcoid.

Radiographic Interpretation

Chest radiograph demonstrates bilateral symmetrical hilar

enlargement with lobulated outline that favors lymph-nodal

enlargement as opposed to vascular dilatation. The lung vol-

umes are preserved. There is no cardiomegaly.

CTPA confirms mediastinal and hilar lymphadenopathy

with some nodes exhibiting calcification. There is extrinsic

compression of the pulmonary vasculature (for example, the

right lower lobe artery in the Fig.14.2, far right mediastinal

widow) with lobar stenoses. The resultant perfusion abnormali-

ties are elegantly demonstrated on the Spectral CT (Fig.14.4).

The main pulmonary artery is of normal dimension. The right

ventricle is not dilated or hypertrophied. The nature and distri-

bution of the lymph nodes are highly suspicious for sarcoidosis

although the lung windows did not reveal any bronchocentric

nodules, fissural nodularity or interstitial fibrosis.

There are matched and mismatched perfusion defects

bilaterally on VQ scintigraphy.

Catheter angiography elegantly depicts the proximal

smooth stenoses in the right upper and both lower lobe pul-

monary arteries with mild post-stenotic dilatation. The seg-

mental vessels are preserved with gradual tapering. There is

no evidence of eccentric thrombus, pouch defects or intravas-

cular webs. Taken in conjunction with the CT findings, the

location of the stenoses would be in keeping with fibrosing

mediastinitis and extrinsic compression by the mediastinal

nodes.

Multifactorial causes contribute to pulmonary hyperten-

sion in sarcoidosis. VQ appearances can be confusing as the

perfusion defects may be matched, mismatched or reverse

mismatched (ventilation worse than perfusion). CT is

extremely useful to delineate the extent and severity of the

lung fibrosis, mediastinal lymphadenopathy and extrinsic

R. Schilz et al.

139

compression of the pulmonary arteries and veins. Sarcoid

vasculopathy is however difficult to evaluate on CT and is an

important factor to remember if imaging features are under-

whelming in the presence of significant pulmonary hyperten-

sion in a patient with known sarcoidosis.

Clinical Comments

The presented case represents a reminder to the diagnostician

that perfusion abnormalities on lung scintigraphy can be

caused by a wide variety of disease states. Therefore, an abnor-

mal lung scan is insufficient to establish the diagnosis of

chronic thromboembolic disease. This is especially the case

when it comes to main PA and lobar level perfusion defects.

In these settings, it is imperative to rule out mediastinal and

hilar pathology that might be extrinsically compressing or

obstructing the pulmonary vessels. Though sarcoidosis was the

culprit in this case, mediastinal fibrosis such as from histoplas-

mosis, or mediastinal neoplasm can present with similar radio-

graphic findings on screening studies. And finally, when digital

subtraction angiography demonstrates vascular findings atypi-

cal for chronic thromboembolic disease—especially in the

proximal vessels—more advanced thoracic imaging needs to

be considered to better define mediastinal anatomy.

Point ofEmphasis

There are several mimics of chronic thromboembolic disease

that the astute clinician needs to differentiate. Persistent per-

fusion defects on lung scintigraphy, and the pulmonary vascu-

lar irregularities on catheter-based pulmonary angiography,

while suggestive of chronic thromboembolic disease, may

also result from intrinsic pulmonary vascular disease (as in

large vessel pulmonary arteritis) or from extrinsic compression

from mediastinal or hilar adenopathy as seen in this pre-

sented case of pulmonary sarcoidosis.

Chapter 14. Case 14: Sarcoidosis andLarge Vessel...

140

Diagnosis

1. Pulmonary sarcoidosis.

Diagnoses

Further Reading

Case Presentation

Radiographic Interpretation

Clinical Comments

Diagnosis

Further Reading