Multi-technique imaging of sarcMulti-technique imaging of sarcoidosisoidosis

A.Balan, E.T.D.Hoey, F.SheerinA.Balan, E.T.D.Hoey, F.Sheerin

Clinical Radiology 65(2010)750-760Clinical Radiology 65(2010)750-760

VocabularyVocabulary Sarcoidosis Sarcoidosis 肉状瘤病肉状瘤病 , , 类肉状瘤病；结节病类肉状瘤病；结节病 Idiopathic Idiopathic 先天的先天的 hall-mark hall-mark 标志标志 Caseate Caseate v.v. 干酪化干酪化 , , 发生干酪样变发生干酪样变 Collaret n.Collaret n. 女用围巾女用围巾 , , 女用领巾女用领巾 Insidious adj. Insidious adj. 阴险的阴险的 Corticosteroid n & adj.[Corticosteroid n & adj.[ 生化生化 ]] 皮质甾类皮质甾类 (( 的的 ),), 皮质类固醇皮质类固醇 (( 的的 )) Pathognomonic adj. Pathognomonic adj. （病征）能确定诊断的（病征）能确定诊断的 erythema n.erythema n. 红斑红斑 , , 非冻疮 非冻疮 nodous nodous 多节的多节的 Suffice vi.Suffice vi. 足够足够 , , 有能力 有能力 vt.vt. 使满足使满足 Hilar adj.[Hilar adj.[ 解解 ]] 门的门的 Beading n.Beading n. 玻璃珠玻璃珠 , , 珠细工 珠细工 bead n.bead n. 珠子珠子 , , 水珠水珠 Conglomeration Conglomeration 团块团块 , , 聚集聚集 , , 混合物混合物 Dilatation n.Dilatation n. 扩张扩张 ,, 扩张术扩张术 Syncope n.Syncope n. 昏厥昏厥 Mid adj.Mid adj. 中央的中央的 , , 中部的中部的 , , 中间的中间的 Leptomeningeal Leptomeningeal 柔脑脊膜的柔脑脊膜的 Paraparesis Paraparesis 下身轻瘫下身轻瘫 ,, 下肢轻瘫下肢轻瘫

Avid adj.Avid adj. 渴望的渴望的 Myelitis n.Myelitis n. 脊髓炎脊髓炎 Pachymeningeal Pachymeningeal 硬脑膜的硬脑膜的 Ophthalmoplegia Ophthalmoplegia 眼肌瘫痪眼肌瘫痪 , , 眼肌麻痹眼肌麻痹 Ophthalmic adj.Ophthalmic adj. 眼的眼的 , , 眼科的 眼科的 n.n. 眼药眼药 Lacrimal adj.Lacrimal adj. 泪腺的泪腺的 , , 泪的泪的 , , 满是泪水的满是泪水的 Subarachnoid adj.[Subarachnoid adj.[ 解解 ] ] 蛛网膜下的蛛网膜下的 Oculomotor adj.Oculomotor adj. 眼球运动的眼球运动的 Proptosis n. Proptosis n. 突出突出 , (, ( 眼球等器官眼球等器官 )) 前垂前垂 Tenosynovitis Tenosynovitis 腱鞘炎腱鞘炎 Effusion n.Effusion n. 流出物流出物 , , 泻出泻出 , [, [ 医医 ]] 渗出渗出 Lacy adj.Lacy adj. 花边的花边的 Reticular adj.Reticular adj. 网状的网状的 , , 错综的错综的 Conglobate v. (Conglobate v. ( 使使 )) 成球状 成球状 adj.adj. 球状的球状的 , , 圆圆的 圆圆的 diaphragmatic diaphragmatic 横隔膜的横隔膜的 , , 隔膜的 隔膜的 merate n.merate n. 汞溴红汞溴红 , , 红药水红药水 Evade v.Evade v. 规避规避 , , 逃避逃避 , , 躲避躲避

introductionintroduction

Sarcoidosis is an idiopathic multisystem disordSarcoidosis is an idiopathic multisystem disorder, first described by Hutchinson in 1877.er, first described by Hutchinson in 1877.

The pathological hall-mark of the disease is thThe pathological hall-mark of the disease is the non-caseating sarcoid e non-caseating sarcoid (( 肉状的肉状的 ))granulomagranuloma(( 肉肉芽肿芽肿 ),), which is composed of histiocytes surrou which is composed of histiocytes surrounded by a collaret of lymphocytes and mononunded by a collaret of lymphocytes and mononuclear cells.clear cells.

Approximately 3000 new cases of sarcoid are diagnosApproximately 3000 new cases of sarcoid are diagnosed each year in the United Kingdom with an incidenced each year in the United Kingdom with an incidence of 5.0 per 100,000 person-years.e of 5.0 per 100,000 person-years.

The disease most commonly presents between the ageThe disease most commonly presents between the ages of 20s of 20 ～～ 50 years, and although there is a worldwide 50 years, and although there is a worldwide distribution, a geographical preponderance distribution, a geographical preponderance (( 优势优势 )) is re is recognized for African-American, Danish cognized for African-American, Danish (( 丹麦的丹麦的 )), and , and Swedish populations.Swedish populations.

The lungs and intrathoracic lymph nodes are almost aThe lungs and intrathoracic lymph nodes are almost always involved and although patients may be asymptlways involved and although patients may be asymptomatic, sarcoidosis most often presents with insidious omatic, sarcoidosis most often presents with insidious respiratory and systemic symptoms, such as fatigue respiratory and systemic symptoms, such as fatigue (( 乏力乏力 )), weight loss, and fever., weight loss, and fever.

Extrathoracic manifestations , present in 25Extrathoracic manifestations , present in 25 ～～ 50% o50% of cases, are usually associated with intrathoracic diseaf cases, are usually associated with intrathoracic disease.se.

There is a spontaneous remission rate of 40There is a spontaneous remission rate of 40 ～～ 90% i90% in those with limited stage disease, and systemic treatn those with limited stage disease, and systemic treatment with corticosteroids or chemotherapy is usually ment with corticosteroids or chemotherapy is usually reserved for patients with progressive pulmonary invreserved for patients with progressive pulmonary involvement or critical organ dysfunction.olvement or critical organ dysfunction.

confirmation of the diagnosis usually requires confirmation of the diagnosis usually requires histological proof; occasionally, appropriate imaging histological proof; occasionally, appropriate imaging features coupled with compatible clinical findings features coupled with compatible clinical findings may suffice.may suffice.

Recognition of the varied radiological features of Recognition of the varied radiological features of this condition not only plays a vital role in aiding this condition not only plays a vital role in aiding diagnosis, but can also provide prognostic diagnosis, but can also provide prognostic information as well as guiding therapy.information as well as guiding therapy.

Figure 1 Figure 1

Chest radiographic findings Chest radiographic findings in sarcoidosis (stage 2 diseain sarcoidosis (stage 2 disease). se).

Plain chest radiograph showPlain chest radiograph shows bilateral symmetric hilar as bilateral symmetric hilar and right paratracheal lymphnd right paratracheal lymphadenopathyadenopathy （淋巴结病）（淋巴结病） witwith diffusely distributed small h diffusely distributed small nodules throughout both lunnodules throughout both lungs.gs.

Figure 2 Figure 2

Typical high-resolution CT Typical high-resolution CT (HRCT) findings in sarcoid(HRCT) findings in sarcoidosis. osis.

Axial, high-resolution CT iAxial, high-resolution CT image shows nodules in a chmage shows nodules in a characteristic perilymphatic anaracteristic perilymphatic and subpleural distribution witd subpleural distribution with “beading” of the fissures.h “beading” of the fissures.

Figure 3Figure 3 Advanced pulmonary sarcoidosis (stage 4 disease).Advanced pulmonary sarcoidosis (stage 4 disease).(a) Plain chest radiograph shows advanced pulmonary sarcoidosis with(a) Plain chest radiograph shows advanced pulmonary sarcoidosis with

loss of lung volume and fibrosis.loss of lung volume and fibrosis. (b) Axial, high-resolution CT image in the same patient depicts abnormal (b) Axial, high-resolution CT image in the same patient depicts abnormal

conglomeration of peribronchovascular structures in the upper conglomeration of peribronchovascular structures in the upper lobes associated with tractional airways dilatation in keeping lobes associated with tractional airways dilatation in keeping

with pulmonary fibrosis.with pulmonary fibrosis.

Figure 4Figure 4

Cardiac magnetic resonance (CMR) demonstration of endomyocCardiac magnetic resonance (CMR) demonstration of endomyocardial sarcoidosis. A female patient with known pulmonary sarcoiardial sarcoidosis. A female patient with known pulmonary sarcoi

dosis was under investigation for episodesdosis was under investigation for episodes of pre-syncope. Delayed enhancement inversion recovery of pre-syncope. Delayed enhancement inversion recovery

images obtained 12 min following a bolus of 0.1 mmol/kg intraveimages obtained 12 min following a bolus of 0.1 mmol/kg intravenous gadolinium .nous gadolinium .

a) Axial image showing a fa) Axial image showing a focus of mid wall enhancemocus of mid wall enhancement (arrow) in the basal inteent (arrow) in the basal interventricular septum and furrventricular septum and further nodular foci in the latether nodular foci in the lateral wall.ral wall.

(b) Left ventricular short axis ima(b) Left ventricular short axis image at mid-ventricular level showinge at mid-ventricular level showing mid wall enhancement (arrow) ag mid wall enhancement (arrow) along the interventricular septum.long the interventricular septum.

These findings in conjunction These findings in conjunction with the clinical history were suffiwith the clinical history were sufficient to establish a diagnosis of sacient to establish a diagnosis of sarcoid heart disease without the nercoid heart disease without the need for endomyocardial biopsy. (Led for endomyocardial biopsy. (LV, left ventricle)V, left ventricle)

Figure 5 Figure 5 Leptomeningeal sarcoid resulting in myelitis. A patient with systLeptomeningeal sarcoid resulting in myelitis. A patient with systemic sarcoid presented with a subacute paraparesis. Contrast-enhemic sarcoid presented with a subacute paraparesis. Contrast-enh

anced T1-weighted imageanced T1-weighted image

(a) demonstrates avid nod(a) demonstrates avid nodular infiltrate over the pialular infiltrate over the pial（软膜的） （软膜的） surface of thsurface of the spinal cord and brainstee spinal cord and brainstem. m.

A T2-weighted image in A T2-weighted image in the same planethe same plane

(b) shows high signal (b) shows high signal and slight expansion oand slight expansion of the cord due to a secf the cord due to a secondary myelitisondary myelitis （脊髓（脊髓炎）炎） ..

Figure 6 Figure 6

Pachymeningeal sarcoid.Pachymeningeal sarcoid. A male patient with pulmonary A male patient with pulmonary

sarcoid presented with ophthalsarcoid presented with ophthalmoplegia and headaches. An axmoplegia and headaches. An axial contrast-enhanced, T1-weigial contrast-enhanced, T1-weighted MRI image demonstrates hted MRI image demonstrates marked thickening of the tentorimarked thickening of the tentorial dura extending into the caveral dura extending into the cavernous sinuses (arrows). nous sinuses (arrows).

Appearances are typical of pachAppearances are typical of pachymeningeal sarcoid.ymeningeal sarcoid.

Figure 7 Figure 7 Ophthalmic manifestations of sarcoid. Four gadolinium-enhanced Ophthalmic manifestations of sarcoid. Four gadolinium-enhanced T1-weighted images demonstrate the typical MRI appearances of T1-weighted images demonstrate the typical MRI appearances of

ophthalmic sarcoid in different patients:ophthalmic sarcoid in different patients:

(a) marked enlargement and e(a) marked enlargement and enhancement of the lacrimal glnhancement of the lacrimal glands (thick arrows) secondary ands (thick arrows) secondary to sarcoid infiltrationto sarcoid infiltration

(b) expanded enhancing opti(b) expanded enhancing optic nerve (arrowhead) typical fc nerve (arrowhead) typical for sarcoid infiltration (the difor sarcoid infiltration (the differential includes meningioferential includes meningioma andma andlymphoma.lymphoma.

(c) marked enhancement of (c) marked enhancement of the subarachnoid portion of the subarachnoid portion of the oculomotor nerve on ththe oculomotor nerve on the left (thin arrows) typical fe left (thin arrows) typical for sarcoid.or sarcoid.

(d) mass-like enlargement (d) mass-like enlargement of the superior and lateral of the superior and lateral rectus muscles (curved arrrectus muscles (curved arrow) in a patient with proptow) in a patient with proptosis. osis.

Features are typical of Features are typical of sarcoid, but this is indistinsarcoid, but this is indistinguishable from lymphoma guishable from lymphoma and orbital pseudotumour and orbital pseudotumour on imaging.on imaging.

Figure 8 Figure 8 MRI images of large joint sarcoid arthropathy.MRI images of large joint sarcoid arthropathy.

(a) Sagittal and (b) axial, T2-w(a) Sagittal and (b) axial, T2-weighted, fat-suppressed, MRI sequeighted, fat-suppressed, MRI sequences show marked tenosynovitis ences show marked tenosynovitis (arrows) and ankle arthropathy wi(arrows) and ankle arthropathy with effusion (arrowhead) in a younth effusion (arrowhead) in a young male with a 6-week history of big male with a 6-week history of bilateral ankle swelling.lateral ankle swelling. Ultrasound-guided biopsy confi Ultrasound-guided biopsy confirmed the presence of multiple normed the presence of multiple non-caseating granulomas consistent n-caseating granulomas consistent with sarcoid arthropathy.with sarcoid arthropathy.

Figure 9Figure 9

Osseous involvement in sarcoidosOsseous involvement in sarcoidosis. is.

Plain radiograph of the foot in a pPlain radiograph of the foot in a patient with biopsy-confirmed pulatient with biopsy-confirmed pulmonary sarcoidosis shows multiplmonary sarcoidosis shows multiple lucent lesions in the phalanges, re lucent lesions in the phalanges, resulting in the “lacy” reticular pattesulting in the “lacy” reticular pattern that is characteristic of osseouern that is characteristic of osseous sarcoidosis.s sarcoidosis.

Figure 10Figure 10 Gastrointestinal manifestations of sarcoidosis demonstrated on mGastrointestinal manifestations of sarcoidosis demonstrated on m

ultidetector CT. ultidetector CT.

(a) Contrast-enhanced axial CT i(a) Contrast-enhanced axial CT image in a female with biopsy-promage in a female with biopsy-proven pulmonary sarcoidosis and abven pulmonary sarcoidosis and abnormal liver function tests shows normal liver function tests shows multiple small, ill-defined, low-attmultiple small, ill-defined, low-attenuation lesions in theenuation lesions in theliver and more conglomerate arealiver and more conglomerate areas of low-attenuation throughout ths of low-attenuation throughout the spleen, representing coalescing e spleen, representing coalescing （接合）（接合） granulomas.granulomas.

(b) Following completion of a (b) Following completion of a course of corticosteroid therapy, course of corticosteroid therapy, there was complete resolution of there was complete resolution of the previously demonstrated the previously demonstrated abnormalities.abnormalities.

Figure 11 Figure 11

Ultrasound features of soft-tissue Ultrasound features of soft-tissue sarcoidosis.sarcoidosis.

Ultrasound shows a hypoechoic, Ultrasound shows a hypoechoic, subcutaneous nodule exhibiting csubcutaneous nodule exhibiting colour Doppler flow in a young adolour Doppler flow in a young adult presenting with a lump on the ult presenting with a lump on the thigh. thigh.

This was histologically proven to This was histologically proven to be a sarcoid granuloma.be a sarcoid granuloma.

Figure 12 Figure 12 Disseminated sarcoidosis on FDG PET/CTDisseminated sarcoidosis on FDG PET/CT..

(a) FDG PET maximum intensity (a) FDG PET maximum intensity projection (MIP) image in a patieprojection (MIP) image in a patient with biopsy-provennt with biopsy-provensarcoidosis shows moderate FDG sarcoidosis shows moderate FDG uptake in bilateral hilar, right parauptake in bilateral hilar, right paratracheal, and mediastinal nodes (ltracheal, and mediastinal nodes (large arrow) as well as focal areas arge arrow) as well as focal areas ofofincreased tracer activity in the splincreased tracer activity in the spleen (small arrow) and bilateral Feen (small arrow) and bilateral FDG-avid pelvic lymph nodes (arrDG-avid pelvic lymph nodes (arrowheads)owheads)

(b) Coronal fused PET/CT image (b) Coronal fused PET/CT image shows FDG-avid right apical lung shows FDG-avid right apical lung nodules (arrow).nodules (arrow).

(c) Axial fused PET/CT image con(c) Axial fused PET/CT image confirms a markedly hypermetabolic sfirms a markedly hypermetabolic splenic lesion (arrow). plenic lesion (arrow).

The splenic and sub-diaphragmatic The splenic and sub-diaphragmatic disease had evaded detection by codisease had evaded detection by conventional imagingnventional imaging

Figure 13 Figure 13 Disease response assessment in sarcoidosis with FDG PET. FDG Disease response assessment in sarcoidosis with FDG PET. FDG

PET maximum intensity projection (MIP) imagesPET maximum intensity projection (MIP) images

(a) before and (b) after corticoster(a) before and (b) after corticosteroid therapy in a patient with biopsoid therapy in a patient with biopsy-proven sarcoidosis show comply-proven sarcoidosis show complete resolution of markedly hyperete resolution of markedly hypermetabolic and extensive mediastinmetabolic and extensive mediastinal nodal disease (arrows) and apical nodal disease (arrows) and apical pulmonarynodules (arrowhead),al pulmonarynodules (arrowhead), representing a complete metaboli representing a complete metabolic response to therapy.c response to therapy.

ConclusionConclusion

Sarcoidosis is an important multisystem disorder with proteanSarcoidosis is an important multisystem disorder with protean(( 多变的多变的 )) imaging manifestations. imaging manifestations.

It is important for the radiologist to recognize the typical and It is important for the radiologist to recognize the typical and more unusual features of this disease across a variety of imagimore unusual features of this disease across a variety of imaging techniques in order to suggest the diagnosis in the appropring techniques in order to suggest the diagnosis in the appropriate clinical setting.ate clinical setting.