Author's Accepted Manuscript
Sarcoidosis – the greatest mimic
Varut Vardhanabhuti MBBS, FRCR, Chun-Lap PangMBBS, Tishi Ninan MBBS, FRCR, Will M. AdamsFRCR, Vikram Raju MBBS, FRCR, Priya Suresh MBBS,MRCP, FRCR
PII: S0887-2171(13)00148-0DOI: http://dx.doi.org/10.1053/j.sult.2013.12.003Reference: YSULT571
To appear in:Semin Ultrasound CT MRI
Cite this article as: Varut Vardhanabhuti MBBS, FRCR, Chun-Lap Pang MBBS, TishiNinan MBBS, FRCR, Will M. Adams FRCR, Vikram Raju MBBS, FRCR, Priya SureshMBBS, MRCP, FRCR, Sarcoidosis – the greatest mimic,Semin Ultrasound CT MRI , http://dx.doi.org/10.1053/j.sult.2013.12.003
This is a PDF file of an unedited manuscript that has been accepted for publication. As aservice to our customers we are providing this early version of the manuscript. Themanuscript will undergo copyediting, typesetting, and review of the resulting galley proofbefore it is published in its final citable form. Please note that during the production processerrors may be discovered which could affect the content, and all legal disclaimers that applyto the journal pertain.
www.elsevier.com/locate/enganabound
Full Title Page (a) Manuscript Title: Sarcoidosis – the greatest mimic. (b) Authors/Affiliations: Dr Varut Vardhanabhuti MBBS FRCR Dr Chun-Lap Pang, MBBS Dr Tishi Ninan MBBS FRCR Dr Will M Adams FRCR Dr Vikram Raju MBBS FRCR Dr Priya Suresh MBBS MRCP FRCR Department of Radiology, Derriford Hospital, Derriford Road, Plymouth, Devon, PL6 8DH, United Kingdom (c) Corresponding Author/Address Priya Suresh Department of Radiology Derriford Hospital Derriford Road Plymouth Devon PL6 8DH United Kingdom Telephone: (+44) 01752432508 Fax: (+44) 01752315300 Email: [email protected] Abstract Little is known about the exact pathogenesis of sarcoidosis but it is widely recognized that it affects multiple organs. The presentation and imaging features can be non‐specific and this is the reason why it is a great mimic of other diseases. Diagnosis of sarcoidosis is often prompted initially by clinical suspicion. Imaging plays a crucial role both in detection and monitoring of disease process. This review is a case‐based systemic approach looking at various systemic manifestation of the disease presenting real clinical encounters using various imaging modalities.
Introduction:
Sarcoidosis is a systemic disease characterized by noncaseating granuloma. Although
many factors and agents have been implicated (microbial, environmental or familial),
the precise aetiological triggers, and exact pathogenesis of the disease are as of yet
unknown. There is a very varied spectrum of clinical manifestations. However most
will have well recognized pulmonary involvement although systemic involvement is
not uncommon. The diagnosis is made often with a combination of clinical,
radiological and histological testing. Imaging plays a crucial role in supporting the
diagnosis, directing treatment as well as localising sites for further testing such as
biopsy to confirm the diagnosis. All this is made whilst excluding other possible
diseases. This is no easy task since manifestations of systemic sarcoidosis have
variable radiological presentations often mimicking other disease processes. The aim
of this article is therefore to present a comprehensive system‐based approach of
various systemic presentations using multi‐modality imaging techniques.
Pulmonary:
Case#1:
45 year old female presented with 3 months history cough and dyspnea. Initial work‐
up chest radiograph reveals bilateral hilar and right paratracheal
lympahadenopathy. A chest CT was subsequently performed (Figs. 1a, 1b).
Constellation of bilateral hilar and right paratracheal lymphadenopathy is known as
Garland triad (also known as 1‐2‐3 sign) and is said to be suggestive of sarcoidosis. A
differential diagnosis remains of lymphoma, TB, and malignancy. Following the chest
CT, the typical pattern of micronodules in the perilymphatic taking in conjunction
with history makes sarcoidosis the most likely diagnosis in this case.
Pulmonary involvement occurs in 90% of patients with sarcoidosis (1). Respiratory
features at presentation include cough, dyspnea, wheeze and bronchial
hyperactivity. However as many as 50% are asymptomatic with abnormalities
detected initially at chest radiography (2). There are 4 stages of pulmonary
sarcoidosis as defined by Siltzbach et al (1967) (3). Stage I (bilateral hilar
lymphadenopathy, BHL), II (BHL and interstitial changes) and III (interstitial changes
without BHL). It is very important to characterize and stage disease because the
disease remains reversible and steroid‐responsive in the first three stages. Stage IV
consists of pulmonary fibrosis, and at this stage the disease is considered end‐stage
disease. These findings can be readily appreciated on chest radiography or
computed tomography. The distribution of lymph node is important with usual BHL
alone or in combination with mediastinal lymphadenopathy occurring in 95% of
cases. Anterior mediastinal involvement is seen in less than 10% of cases, and
posterior mediastinum is less commonly involved (4). Isolated adenopathy in the
anterior or posterior mediastinal compartments, should therefore prompt one to
consider diagnosis other than sarcoid.
The role of CT as well as being of particular importance in establishing diagnosis, also
allows the identification of the disease stage in order to distinguish active
inflammation (where potentially disease may be reversible) to irreversible end‐stage
fibrosis. Prognostically, this is of vital importance because asymptomatic
presentation with BHL alone are likely to resolve spontaneously within 2 years (5).
Pulmonary sarcoidosis classically present with lymphadenopathy (bilateral hilar and
right paratracheal) and micro‐nodules in the peri‐lymphatic distribution. This is seen
in up to 75‐90% of cases (6). Fibrotic changes occur at end‐stage in around 20% of
patients and imaging findings include reticular opacities, architectural distortion and
traction bronchiectasis (7). Classic distribution is described as predominantly upper
and middle zones. However, pulmonary sarcoidosis is also known to have a plethora
of varied imaging appearances. These include airspace consolidation, large nodules
(Figs. 2‐3), patchy ground glass opacities (Fig. 4), linear opacities, fibrocystic changes
(cysts, bullae, or honeycomb‐like opacities), military opacities, and occasionally
pleural disease (effusion, chylothorax, thickening, and calcifications). Imaging also
plays a role in identifying the presence of pulmonary artery hypertension, which is
an ominous sign and may warrant referral for lung transplantation (8). A
retrospective series indicate that although this may relate to the degree of
pulmonary fibrosis, pulmonary arterial hypertension can also be seen in patients in
stage I‐III disease suggesting a sarcoid‐specific vascuolopathy as possible aetiology
(9).
Cardiac:
Case #2:
56 year‐old with known pulmonary and orbital sarcoidosis presented with
palpitations and syncope. Cardiac MRI was requested (Fig. 5) which shows
mid/epicardial delayed gadolinium enhancement in the basal inferior and
inferolateral wall. The pattern of enhancement in this case is non‐specific and could
also be seen in myocarditis (although this is typically more epicardial), or Fabry’s
disease (although there is no left ventricular hypertrophy). Infarction is unlikely
because of the distribution, and also typically these extend from subendocardial
before involving the whole thickness of the myocardium.
Involvement of the heart occurs in 20‐30% but clinical manifestation may be as low
as 5% patients (10). This can involve any location including pericardium, myocardium
and valve leaflets but with a predilection for the base of the interventricular septum.
This results in histo‐pathological findings of inflammation, oedema, granulomatous
infiltration, fibrosis or scarring (11). Clinical findings, when present, include complete
heart block (23‐30%), bundle branch block (12‐32%), ventricular tachycardia (23%),
congestive heart failure (25‐75%) and sudden death (25‐65%) (12). Although, clinical
manifestation is not always evident, a high degree of suspicion is required especially
in the assessment of an otherwise healthy young or middle‐aged patient with cardiac
symptoms, or in a patient with known sarcoidosis who develops conduction
abnormality, arrhythmias, or heart failure. In terms of prognosis, unlike isolated
pulmonary disease, cardiac involvement has much poorer outlook with an estimated
of 5‐8% mortality, but in some parts of the world this can be as high as 80%, for
example in Japan (13).
Imaging has a crucial role to play but current diagnostic imaging techniques such as
echocardiography, 18FFDG PET and MPI‐SPECT have variable sensitivity and specificity
and the most appropriate screening strategy is as of yet unknown. Rest and stressed
radionuclide study has an important role to play. Thallium 201 or technetium 99m
can display a phenomenon known as “reverse distribution” which is characterised by
improvement or resolution of defect following dipyridamole (14). However, this is
not specific and may also occur in other cardiomyopathies. Despite this,
radionucleotide scintigraphy remains a useful tool in demonstrating both cardiac and
extra‐cardiac disease. MRI is emerging as the modality of choice in identifying
cardiac involvement. In the acute phase, oedema associated with acute
inflammation can be readily appreciated on T2‐weighted images and early
gadolinium enhanced images. Fibrosis or scarring can also be demonstrated with
delayed enhancement images following gadolinium injection. The location of the
delayed enhancement is typically mid‐wall and usually occurs in the basal septum
(Fig. 6), although it is recognized that this can be present in any location, and
frequently patchy involvement is seen(15). In addition, MRI can be used to assess
regional wall abnormality. Because, dyskinetic or akinetic segments are interspersed
with normokinetic segments, this results in an uneven wall motion abnormality,
which can be differentiated, from idiopathic dilated cardiomyopathy in which
uniform regional dyskinesia is seen. The patchy mid‐wall delayed enhancement can
also be seen in myocarditis as well as Fabry’s disease which remain important
differential diagnoses to consider given the right clinical context. CMR can also
provide an accurate guide for obtaining endomyocardial biopsies. Serial CMR studies
can also be used for the assessment of disease activity and demonstrate
normalization of myocardial enhancement following treatment (16). In severe
cardiac involvement in which diffuse infiltration leading to global contraction
abnormalities, features of restrictive cardiomyopathy can also be shown on CMR. In
advanced, post‐inflammatory/chronic phase, localised wall thinning and wall motion
abnormality is seen with persistence of delayed enhancement of post gadolinium
images. These typically spare the subendocardium which differentiate it from
pattern of myocardial infarction (17).
Neurological
Case #3
45 year‐old man presented with confusion and non‐specific generalised weakness.
Initial CT brain was unremarkable. Follow‐up Brain MRI reveals florid leptomeningeal
disease (Figs. 7a, 7b). Chest radiograph was performed which revealed bilateral and
right paratracheal lymphadenopathy. The differential diagnoses lie between sarcoid
and TB in this case.
Brain:
Central nervous system sarcoidosis is first recognised by Winker et al in 1905 (18).
Neurosarcoidosis can be defined as when sarcoidosis manifest specifically in the
nervous system. Neurological complications occur in about 5% of all patients with
sarcoidosis (19). The most common presentations include cranial mononeuropathy
(Fig. 8), neuroendocrine dysfunction and peripheral neuropathy. Other clinical
features include myelopathy, radiculopathy, communicating or non‐communication
hydrocephalus and meningeal involvement. Isolated neurosarcodosis, without
systematic evidence of sarcoidosis is very rare, and occurs in less than 1% of
sarcoidosis patients (20). There are not many well‐designed study to look at the
prognosis of patients with neurosarcoidosis. In general, neurosarcodosis is a very
rare complication in existing sarcoid patients or occurring in isolation. It can range
from mild involvement, e.g. isolated facial nerve palsy whom responds well to
corticosteroids, and life threatening involvement, e.g. progressive relapsing
encephalitis and obstructive hydrocephalus. Corticosteroids is the mainstay of
treatment and other immunomodulators may be considered. Surgical excision of a
mass and radiotherapy are considered in very extreme circumstances. In terms of
central nervous system imaging, neurosarcoidosis does not have specific features
that lead to definitive diagnosis. The most frequent choice of investigation is
contrast enhanced MRI (21). Leptomeningeal disease is a common pattern of
involvement, which can either be localised or widespread. Meningeal or
parenchymal enhancement suggests active inflammation of the blood brain barrier.
MRI also helps to identify any parenchymal masses (Fig. 9) or hydrocephalus.
Neuroimaging is often used in combination with clinical features, cerebrospinal fluid
analysis, biopsy and others. Electroencephalography, evoked potentials,
electromyography, nerve conduction studies and angiography are occasionally used
to exclude other conditions or to localise isolated lesion. Subjects can be tested
false‐postive for Kveium‐Siltzbach test, but this is by no means pathognomonic for
neurosarcodosis (22). Overall, a presumptive diagnosis of neurosarcodosis is only
made in the right clinical setting.
Spine:
Case #4:
39 year old male presented with right arm weakness of 4 weeks duration. There was
some improvement in symptoms initially but this persists. Full neurological imaging
was performed with a normal MRI brain but isolated high T2 lesion seen in the upper
thoracic cord and lower lumbar level. Initially this was thought to be multiple
sclerosis but absence of brain lesion make this diagnosis less likely. The appearance
could therefore represent granulomatous disease.
Spinal cord sarcoidosis can be part the central nervous system manifestation of
neurosarcoidosis. Like neurosarcoidosis in the cerebrum, granulomatous change can
exhibit as either diffuse leptomeningeal enhancement, for example, as multiple
nodules on the meninges covering the spinal cord (Fig. 10a), or as focal lesions (Fig.
10b), often associated with cord swelling, such as, focused increase nodularity in the
cauda equina. Isolated spinal cord sarcoidosis is exceedingly rare.
Musculo‐skeletal
Sarcoidosis has been reported to affect the musculoskeletal system in 10‐40% of
cases (23,24). Manifestations of sarcoidosis in the musculo‐skeletal system can
broadly be classified into osseous changes, joint involvement and muscular changes.
Plain radiographic findings of bony sarcoidosis has been well documented in the
literature but recently MR has been used to image the occult lesions. The MR
findings are not specific for sarcoidosis, and should be used in conjunction with the
clinical history, other available modalities and the histology in the doubtful cases.
Case #5
A 55 year old male was referred to by is GP with extensive pain and swelling of his
third toe in his left foot. Clinically due to the erythema and swelling, osteomyelitis
was suspected. However, plain radiograph of the foot demostarted lace‐like
trabecular pattern of phalanges of the third toe of the left foot with acro‐osteolysis
(Fig. 11). The inflammatory markers were not elevated and review of PA chest
radiograph showed bilateral hilar lymphadenopathy. A diagnosis of osseous sarcoid
was made.
Osseous changes: Sarcoidosis of the skeletal system typically involves the small
bones of the hands and feet. However involvement of other sites like long bones of
the limbs, pelvis, vertebrae, vault of the skull and long bones are well documented
(25). On plain radiography, the classic lesions are described as lucent, punched out
lesions or coarsening of the trabeculae. The classical lace‐like pattern is due to
osteolysis which can lead to cortical destruction and acro‐osteolysis. As described in
the above case sausage‐like dactylitis of the fingers or toes can occur. The
radiographic finding of the lacy destructive pattern in small bones of the hand and
joint are pathognomonic in the clinical setting of sarcoidosis as in the above
mentioned case.
Case #6
GP requested a MR scan of the spine for a patient who had back pain which did not
respond to conservative treatment. The MR showed multiple focal lesions in the
vertebral bodies which were well defined and had a target like appearance. They
were predominantly low signal intensity on the T1 weighted images with a rim of
high signal intensity suggesting presence of fat within the lesion. The T2 images
showed clearly marginated focal lesions with high signal intensity. These were
thought to represent metastases although the presence of fat within the lesions on
T1 weighted images suggested a more benign process. A CT scan of the cheat
abdomen and pelvis was performed to look for a primary lesion. This revealed typical
lung lesions and hilar lymphadenopathy inkeeping with sarcoidosis.
Sarcoidosis of the axial skeleton usually do not cause cortical destruction unlike the
lesions affecting the small bone (26) Although sclerotic lesions can be seen in the
pelvis and the vertebrae, osseous sarcoid may be occult on plain radiography. On
MRI, the lesions can have a variety of appearances. They can be poorly marginated
lesions which are confluent and show irregular marrow infiltration. Less well‐defined
discrete lesions have also been described. They may be well defined round,
cannonball‐like intramedullary lesions (Fig. 12). The lesions as described above are
commonly hyperintense on T2‐weighted and short‐tau inversion recovery images,
and has low signal intensity on T1‐weighted sequences.
As in the case described above focal areas with signal intensity characteristics
corresponding to fat and may be useful in differentiating these lesions from osseous
metastases (26).Bone scintigraphy shows increased uptake in areas of bone lesions.
Case reports have also mentioned increased uptake of FDG in osseous sarcoidosis. In
short, the radiolographically occult bony lesions have varied appearance on MR
imaging, which can be non‐specific and can mimic other graulomatous disease,
infection or metastatic disease. Correlation of the MR imaging findings with other
modalities, laboratory data, and clinical symptoms is necessary to aid diagnosis.
Case #7
50 year old man presented with history of a swelling in the left distal forearm.
Clinically a malignant lesion was suspected. Magnetic resonance scan of the forearm
was performed. This showed increased signal in pronator quadratus muscle on T2
weighted images (Fig. 13). MRI also showed mature periosteal reaction (Fig. 14). This
has been postulated to be most likely secondary to an inflammatory reaction of
Sharpey’s fibres at the site of muscle insertion. (27) The diagnosis of sarcoidosis was
confirmed on histology. Review of his clinical history revealed that he had stage III
sarcoidosis.
Muscular changes: Muscular involvement of sarcoidosis was first described by
Licharew in 1908 (28). The muscular changes are very rare and are seen in about 6%
of patients with sarcoidosis (29). Involvement of the muscles is asymptomatic in
many cases and can be under‐diagnosed. When symptomatic, there are three main
clinical subtypes described – palpable nodules, acute myositic and chronic
myopathic. The palpable nodular type was the first type described by Licharew but
this is the least common variety. The sarcoid nodules have typically been described
to consist of a star shaped area of low signal intensity on T1 and T2 weighted images
with a surrounding area of higher signal intensity that is more marked on the T2
weighted images. The surrounding area also demonstrates enhancement on the post
gadolinium images. CT scans of these nodules show a central area of low density
with slight post contrast enhancement of the periphery. The acute myositic type is
usually seen in the early stages of sarcoidosis. The typical appearance is described as
‘three stripes’ appearance where hypo intense granulomatous tissue is sandwiched
between oedematous tissue (28). The chronic myositic variety usually presents as
non specific muscle atrophy. Electromyographic correlation is required to
differentiate this from other causes of muscle atrophy.
Case #8
Patient presented to the plastic surgeon with multiple, slightly painful cutaneous
lumps, which were slowly growing. A clinical diagnosis of neurofibromatosis was
made. The patient was sent for US imaging which revealed multiple mildly vascular
hypoechoic plaque like lesion in the upper lumbar region (Fig. 15). This was not
typical for a neurofibroma. This was further characterized by MR imaging (Fig. 16)
which demonstrated increased signal intensity on water‐sensitive images, low signal
intensity on T1‐weighted images, and enhancement on contrast‐enhanced images. A
biopsy of the lesion confirmed sarcoidosis.
Cutaneous sarcoidosis:
It occurs in up to one third of patients with systemic sarcoidosis and is known as one
of the “great imitators” in dermatology with varied presentation including papules,
plaques, erythema nodosum and lupus pernio. Lesions of lupus pernio, in particular,
are associated with more severe systemic involvement, while erythema nodosum
often indicates acute benign disease. About 30 percent of patients with cutaneous
lesions develop systemic involvement (30). On MR images the skin nodules, and soft‐
tissue masses associated with lymphadenopathy may be detected. The soft‐tissue
lesions may be discrete or indistinct and have nonspecific signal intensity
characteristics that correspond to solid mesenchymal masses with some nonspecific
inflammatory changes (Fig. 17). Differential diagnosis includes other benign and
malignant mesenchymal masses including tophus, pannus, and xanthoma.
Gastrointestinal (including liver and spleen)
Involvement of liver and spleen are common findings post‐mortem with up to 80%
has been reported (31) although clinically the patient are only symptomatic in
roughly 5‐15% of cases(32). Liver involvement is often incidentally diagnosed upon
investigation for abnormal liver function tests. If symptomatic, presentations include
abdominal pain and pruritus. Ultrasound, CT and MRI are the commonest imaging
modalities. Findings include generalized visceromegaly (such as hepatomegaly or
splenomegaly) or more focally as hepatic or splenic nodules representing coalescent
granulomas (Figs. 18a ‐18b)(33,34). It is uncommon for gastrointestinal tracts to be
involved, with only 10% found on autopsy, but when present stomach is the
commonest site with manifestation characterized by localized granuloma associated
with ulceration or diffuse involvement similar to that of linitis plastica(33).
Genito‐Urinary Systems
Case #9
32 year old man presented with chronic testicular pain associated with pulling
sensation around the groin. US testes was performed which shows multiple
hypoechoic lesions within both testicles (Fig. 19a). US of the region superior to the
testes also revealed thickened spermatic cord (Fig. 19b). Differentials include
lymphoma, metastases, and sarcoidosis (which was subsequently proven).
Hypercalcemic states often accompany sarcoidosis, and this accounts for
nephrocalcinosis being the commonest manifestations in the kidneys. Direct
involvement to the kidneys is rare with non‐specific appearances from striated
nephrograms on contrast‐enhanced CT caused by interstitial nephritis or multiple
focal low attenuation lesions giving rise to a pseudo‐tumour appearance (35,36). The
epididymis is the most commonly involved intrascrotal structure but testicular
involvement can also occur (37), although rare with up to 5% on autopsy series. The
appearances on US are single or multiple hypoechoic lesion(s) (Fig. 19a). It is often
difficult to differentiate from tumour but multiplicity and epididymal involvement is
suggestive of sarcoidosis in patients with a history of the disease (Fig. 19b).
Conclusion
It is important to always bear in mind that sarcoidosis can affect various organs and
is a great mimic of other diseases. Plethora of imaging features have been discussed
and illustrated in this review with relevant clinical presentations. Familiarity with the
various locations and manifestation on multi‐modality imaging platform will aid in
the diagnosis and further management of the patient.
Reference: 1. Lazarus A. Sarcoidosis: epidemiology, etiology, pathogenesis, and genetics. Dis
Mon. 2009 Nov 1;55(11):649–60.
2. Lynch JP, Kazerooni EA, Gay SE. Pulmonary sarcoidosis. Clin Chest Med. 1997 Dec;18(4):755–85.
3. Siltzbach LE. Sarcoidosis: clinical features and management. Med Clin North Am. 1967 Mar;51(2):483–502.
4. Miller BH, Rosado‐de‐Christenson ML, McAdams HP, Fishback NF. Thoracic sarcoidosis: radiologic‐pathologic correlation. Radiographics. 1995 Mar;15(2):421–37.
5. King CS, Kelly W. Treatment of sarcoidosis. Dis Mon. 2009 Nov 1;55(11):704–18.
6. Criado E, Sanchez M, Ramirez J, Arguis P, de Caralt TM, Perea RJ, et al. Pulmonary Sarcoidosis: Typical and Atypical Manifestations at High‐Resolution CT with Pathologic Correlation. Radiographics. 2010 Oct 12;30(6):1567–86.
7. Abehsera M, Valeyre D, Grenier P, Jaillet H, Battesti J‐P, Brauner MW. Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function. AJR Am J Roentgenol. 2000 Jun;174(6):1751–7.
8. Baughman RP. Pulmonary hypertension associated with sarcoidosis. Arthritis Res Ther. 2007;9 Suppl 2:S8.
9. Nunes H, Humbert M, Capron F, Brauner M, Sitbon O, Battesti J‐P, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006 Jan;61(1):68–74.
10. Kim JS, Judson MA, Donnino R, MD MG, Cooper LT, Prystowsky EN, et al. Cardiac sarcoidosis. American Heart Journal. 2009 Jan 8;157(1):9–21.
11. Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978 Dec;58(6):1204–11.
12. Sekhri V, Sanal S, DeLorenzo LJ, Aronow WS, Maguire GP. Cardiac sarcoidosis: a comprehensive review. aoms. 2011;4:546–54.
13. Iwai K, Sekiguti M, Hosoda Y, DeRemee RA, Tazelaar HD, Sharma OP, et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis. 1994 Mar;11(1):26–31.
14. Haywood LJ, Sharma OP, Siegel ME, Siegel RJ, Gottlieb SL, Caldwell J, et al.
Detection of myocardial sarcoidosis by thallium 201 imaging. J Natl Med Assoc. 1982 Oct;74(10):959–64.
15. Youssef G, Beanlands RSB, Birnie DH, Nery PB. Cardiac sarcoidosis: applications of imaging in diagnosis and directing treatment. Heart. 2011 Dec;97(24):2078–87.
16. Matoh F, Satoh H, Shiraki K, Odagiri K, Saitoh T, Urushida T, et al. The usefulness of delayed enhancement magnetic resonance imaging for diagnosis and evaluation of cardiac function in patients with cardiac sarcoidosis. J Cardiol. 2008 Jun;51(3):179–88.
17. Dubrey SW, Grocott‐Mason R, Mittal TK. Images in cardiology: Cardiac sarcoidosis with delayed enhanced MRI. Heart. 2005 Sep;91(9):1185.
18. Vinas FC, Rengachary S. Diagnosis and management of neurosarcoidosis. J Clin Neurosci. 2001 Nov;8(6):505–13.
19. Joseph FG, Scolding NJ. Sarcoidosis of the nervous system. Pract Neurol. 2007 Aug;7(4):234–44.
20. Smith JK, Matheus MG, Castillo M. Imaging Manifestations of Neurosarcoidosis. American Journal of Roentgenology. 2004 Feb;182(2):289–95.
21. Pawate S, Moses H, Sriram S. Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM. 2009 Jul;102(7):449–60.
22. Joseph FG, Scolding NJ. Neurosarcoidosis: a study of 30 new cases. J Neurol Neurosurg Psychiatr. 2009 Mar;80(3):297–304.
23. Bonakdarpour A, LEVY W, Aegerter EE. Osteosclerotic changes in sarcoidosis. Am J Roentgenol Radium Ther Nucl Med. 1971 Dec;113(4):646–9.
24. Prabhakar HB, Rabinowitz CB, Gibbons FK, O'Donnell WJ, Shepard J‐AO, Aquino SL. Imaging Features of Sarcoidosis on MDCT, FDG PET, and PET/CT. American Journal of Roentgenology. American Roentgen Ray Society; 2008 Mar;190(3_supplement):S1–S6.
25. Bloch S, Movson IJ, Seedat YK. Unusual skeletal manifestations in a case of sarcoidosis. Clin Radiol. 1968 Apr;19(2):226–8.
26. Moore SL, Teirstein A, Golimbu C. MRI of sarcoidosis patients with musculoskeletal symptoms. AJR Am J Roentgenol. 2005 Jul;185(1):154–9.
27. Suresh S, Tirabosco R, Saifuddin A, O'Donnell P. An unusual presentation of muscular sarcoidosis. Skeletal Radiol. 2007 Oct;36(10):995–8.
28. Otake S, Banno T, Ohba S, Noda M, Yamamoto M. Muscular sarcoidosis: findings at MR imaging. Radiology. American Public Health Association;
1990;176(1):145–8.
29. Heckmann JG, Stefan H, Heuss D, Hopp P, Neundorfer B. Isolated muscular sarcoidosis. Eur J Neurol. 2001 Jul 25;8(4):365–6.
30. Mañá J, Marcoval J, Graells J, Salazar A, Peyrí J, Pujol R. Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol. 1997 Jul;133(7):882–8.
31. Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M, Nagai S. Radiologic manifestations of sarcoidosis in various organs. Radiographics. 2004 Jan;24(1):87–104.
32. Afshar K, BoydKing A, Sharma OP, Shigemitsu H. Gastric sarcoidosis and review of the literature. J Natl Med Assoc. 2010 May;102(5):419–22.
33. Warshauer DM, Lee JKT. Imaging manifestations of abdominal sarcoidosis. AJR Am J Roentgenol. 2004 Jan;182(1):15–28.
34. Ebert EC, Kierson M, Hagspiel KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol. 2008 Dec;103(12):3184–92–quiz3193.
35. Heldmann M, Behm W, Reddy MP, Bozeman C, Welman G, Abreo F, et al. Pseudotumoral renal sarcoid: MRI, PET, and MDCT appearance with pathologic correlation. AJR Am J Roentgenol. 2005 Sep 1;185(3):697–9.
36. Montagnac R, Schillinger F, Achab A, Ciupea A. Pseudotumeur musculaire avec hypercalcémie et insuffisance rénale aiguë révélant une sarcoïdose. Néphrologie & Thérapeutique. 2005 Oct;1(4):241–6.
37. Stewart VR, Sidhu PS. The testis: the unusual, the rare and the bizarre. Clin Radiol. 2007 Apr 1;62(4):289–302.
Figure Legends Figure 1a. Contrast‐enhanced chest CT (mediastinum window) showing enlarged hilar lymphadenopathy (white arrows). Figure 1b. Contrast‐enhanced chest CT (lung window) showing typical micronodules in the peri‐lymphatic distribution. (white arrows) Figure 2. Contrast‐enhanced chest CT viewed in mediastinum window( Fig 2a) and lung window (Fig 2b) showing bilateral enlarged hilar lymphadenopathy White arrow and multiple large nodules (black arrow) consistent with ‘nodular‐type’ sarcoid. Figure 3.
Contrast‐enhanced chest CT coronal section (lung window) demonstrating multiple large nodules in ‘nodular‐type’ sarcoid. Figure 4. HRCT demonstrating upper lobe predominant ground glass opacity occasionally seen with sarcoidosis. Figure 5. Cardiac MRI short‐axis view acquired using spoiled gradient echo (Turbo Flash) post gadolinium contrast showing mid and subepicardial delayed enhancement in the inferolateral wall. (white arrows) Figure 6. Cardiac MRI short‐axis view acquired using spoiled gradient echo (Turbo Flash) post gadolinium contrast showing delayed enhancement in the mid‐wall of the basal septal typical of cardiac sarcoidosis. (white arrows) Figure 7a. MRI brain (axial) post‐contrast showing florid leptomeningeal enhancement which is typical involvement of neurosarcoidosis. (white arrows) Figure 7b. MRI brain (coronal) post‐contrast showing florid leptomeningeal enhancement which is typical involvement of neurosarcoidosis. (white arrows) Figure 8. MRI brain T1 pre (a) and post‐contrast (b) illustrating enhancement along the optic nerve sheaths more conspicuous on the right. (white arrow) Figure 9. MRI brain T2‐weighted showing an abnormal area of high signal intensity in the region of the pons. (white arrow) Figure 10a. MRI spine T1 (a) and T2 (b) showing focal areas of high signal intensity on T2 in the spinal cord (white arrows). Figure 10b. MRI spine T1 post contrast showing enhancing focal lesions in the spinal cord. (white arrow) Figure 11. Foot radiograph demonstrating lace‐like bony changes affecting the 3rd proximal and middle phalanges and acro‐osteolyisis affecting the 3rd distal phalanx (white arrows). Figure 12.
MRI spine T1 and T2 weighted showing multiple focal lesions within the vertebrae (white arrows) with a convex margin with the marrow fat. A” target” lesion (grey arrows) with fat within the lesion on T1 weighted images. Image courtesy of Dr Kausik Mukherjee, University Hospital of Wales, Cardiff, United Kingdom. Figure 13. T2 weighted image of the distal forearm demonstrates hyperintense signal involving the pronator quadratus muscle. (white arrow) Figure 14. Coronal, T1‐weighted image of the distal forearm demonstrates a mass within the pronator quadrates muscle, which is mildly hyperintense (arrows). There is also low signal intensity periosteal reaction (black arrow) Figure 15. US image over upper lumbar region demonstrating predominantly hypoechoic lesion adjacent to skin surface with mild vascularity. (white arrow) Features were not suggestive of the clinical diagnosis of neurofibroma. Figure 16. MRI lumbar spine demonstrating a plaque like (white arrows)low signal intensity lesion in the subcutaneous tissue on T1 weighted images. This showed high signal intensity on STIR images. Note a simple haemangioma at L3 vertebra Figure 17. MRI STIR in another patient presenting with soft tissue lumps demonstrates hyperintense lesions within the subcutaneous planes overlying the lateral thigh muscles (white arrows). Also note the non‐specific inflammatory reticulated pattern in the adjacent subcutaneous tissue. Figure 18a Axial MRA demonstrating multiple low attenuation lesions (yellow arrows) in the spleen. Figure 18b. These multiple round lesions demonstrate enhancement (yellow arrow) even on late gadolinium sequences, and therefore are not cysts, but rather represent multiple granulomas. Note also midwall myocardial delayed enhancement in the basal anterio‐lateral wall (white arrow) consistent with cardiac sarcoidosis. Figure 19a. US image of the left testis showing ill‐defined hypoechoic lesion subsequently proven to be sarcoidosis. (white arrows) Figure 19b. US image with Doppler follow demonstrating thickened spermatic cord which was proven to be sarcoid infiltration.
Fig1a, b
Fig 2
Fig 3
Fig 4
Fig 5
Fig 6
Fig 7a, b
Fig 8
Fig 9
Fig 10 a, b
Fig 11
Fig 12
Fig 13
Fig 14
Fig 15
Fig 16
Fig 17
Fig 18 a, b
Fig 19a, b