RADIOLOGIC MANIFESTATIONS OF SARCOIDOSIS IN VARIOUS ORGANS

M. BANI, K. BOUZAIDI, F. SNENE, I. KECHAOU, F. JABNOUN

Radiology service, MT Maamouri hospital, Nabeul, TunisiaPan Arab Association of Radiological societies (PAARS) 2012

VARIOUS : VR 2

INTRODUCTION

Sarcoidosis is a systemic disorder of unknown cause with a wide variety of clinical and radiologic manifestations.

The diagnosis is usually made on the basis of these manifestations supported by histologic findings (noncaseating granulomes with proliferation of epithelioid cells)

Systemic manifestations (eg, Lofgren syndrome, Heerfordt syndrome) are commonly seen at clinical examination. Bilateral hilar

lymphadenopathy is the most common radiologic finding. However, extra thoracic involvement can be an initial manifestation of symptomatic patients.

Familiarity with the clinical and radiologic features of sarcoidosis in various anatomic locations plays a crucial role in diagnosis and management.

OBJECTIVES In this Study, we review sarcoidosis in terms of clinical and radiologic manifestations in a variety

of organs.

We illustrate through some observations rare localization of sarcoidosis and discuss the contribution of imaging.

Material and methods

A series of nine patients collected over 4 years in MT Maamouri hospital, Nabeul, Tunisia.

The diagnosis of sarcoidosis is established on the basis of: * Clinical and radiologic findings * Histologic findings: Lung biopsy (transbronchial)

Extrapulmonary sites (cervical lymph node and liver biopsy )

Results and discussion Average age: 41 years Sex ratio: 8 F/ 1 M Clinical signs and symptoms are non specific and include: cough headache polyuria poldipsia syndrome depression, dizziness bone pain skin lesions abdominal pain

Biology shows: Inflammatory syndrome in all patients. Hypercalcaemia and elevated angiotensin converting enzyme are noted in 5

patients.

Various locations have been reported:

Mediastinal and/or lungs involvements (n=8) Neurosarcoidosis involving: . Hypothalamic-pituitary axis (n=2) . Brain parenchymal localization (n=1) . Leptomeningeal involvement (n=2) Hepatic sarcoidosis: . Homogeneous hepatomegaly (n=2) . Nodular form (n=2) Splenic localization: . Homogenous splenomegaly (n=2) Thyroid involvement (n=1) Peripheric Bone localization (n=2)

Results and discussion

Pulmonary involvement is reported to about 90% of our patients. There are five radiologic stages of

intrathoracic changes:

Stage 0: Normal chest radiograph; Stage 1: Lymphadenopathy only Stage 2: Lymphadenopathy with parenchymal infiltration Stage 3: Parenchymal disease only Stage 4: Pulmonary fibrosis. One-half of patients present with stage 1 disease.

Thoracic Involvement

Chest radiograph of stage 1 sarcoidosis

Patient has hilar adenopathy and no evidence of parenchymal lung involvment.

Chest radiograph of stage 3

sarcoidosis Patient has diffuse lung disease but no significant

adenopathy.

Mediastinal Lymph Nodes Intrathoracic lymphadenopathy is the most commonly encountered radiologic finding in

sarcoidosis (80% of cases) and typically manifests as bilateral hilar adenopathy with right paratracheal adenopathy.

Contrast enhanced (CT) scan clearly depicts the bilateral hilar adenopathy.

LUNGS INVOLVEMENTS Lung involvement in sarcoidosis is seen approximately 20% of

patients and it has a strong predilection for the upper lung.

At histologic analysis, sarcoid granulomas in the lung are typically distributed along the lymphatic vessels, which run within the interstitial tissues of bronchovascular bundles and the subpleural and perilobular spaces .

High-resolution CT can accurately depict this characteristic distribution and typically demonstrates multiple small nodules in a perivascular distribution, along with irregular thickening of bronchovascular bundles and interlobular septa.

Pulmonary sarcoidosis in a 37-year-old man

HR chest CT scan demonstrates:

* Small nodules with a Perivascular distribution

and along the interlobular pleura.

* Irregular thickening of

bronchovascular bundles.

NEUROSARCOIDOSIS Neurosarcoidosis has a strong predilection for

Leptomeninges especially observed the base of the brain.

Cranial nerve involvement, especially facial nerve, the optic nerve and chiasm are also frequently involved.

Neuroendocrine disorders such as diabetes insipidus that arise owing to involvement of the pituitary gland and hypothalamus are relatively common.

LEPTOMENINGEAL INVOLVEMENT

Coronal and axial contrast-enhanced T1-weighted MR imageshow diffusely enhancing leptomeningeal lesions ( arrows)

HYPOTHALAMIC-PITUITARY AXIS

coronal T1-weighted MR image reveal an enlarged hypothalamus

CRANIAL NERVE INVOLVEMENT

On a contrast-enhanced T1-weighted MR image1- The V Cranial Nerve enhancement2- The optic chiasm enhancement.

SPINAL CORD

T2-weighted MR images demonstrate an intramedullary hyperintense lesion Contrast-enhanced T1-weighted MR image depicts an leptomeningeal enhancing.

INTRA AXIAL BRAIN LESION

contrast-enhancedT1-weighted MR image shows aan enhancing intraaxial lesion

PACHYMENINGEAL INVOLVEMENT

Coronal / Sagital contrast-enhanced T1-weighted MR image show enhancing covering the Tentorium cerebelli.

HEPATIC AND SPLENIC SARCOIDOSIS

Liver enlargement is found on ultrasound or CT scan in up to 40% of cases . It’s the commonest CT finding, often accompagned with splenomegaly and (less often) abdominal lymph nodes enlargement.

Contrast-enhanced abdominal CT scan show homogenous organomegaly .

Hepatic granulomas are found on CT in only few cases, typically visualized as multiple, discrete, low attenuating, non enhancing (after contrast injection) nodules of variable size.

Simultaneous Splenic nodules favors a diagnosis of sarcoidosis.

HEPATIC AND SPLENIC SARCOIDOSIS

Hepatic involvement in A 49-year-old woman with Pulmonary sarcoidosis.

Contrast-enhanced abdominal CT scan shows multiple, irregularly shaped nodules of variable size in the liver.

O S S E O U S S A R C O I D O S I S

Skeletal involvement is seen in approximately 10% of patients with sarcoidosis.

The phalanges in the hands and feet are most frequently affected.

These lesions are often multiple Radiologic features may include: - cystlike radiolucent areas - a lacelike honeycomb appearance - extensive bone erosion with pathologic fractures.

A subcutaneous soft-tissue mass, swelling or tenosynovitis may also be present.

The articular spaces are usually intact, unless extensive neuropathic lesions Develop.

a radiograph of the right hand reveals a radiolucent lesion in the proximal phalanges of the fourth and fifth digitis.

The lesion has a lacelike appearance and is accompanied by a soft-tissue swelling

O S S E O U S S A R C O I D O S I S

The distal phalanges of the right third and left fourth toes present similar trabucular architecture and bony remodeling changes.

On the distal phalanges of both halluces minute cortical defects are demonstrated.

The joints space width is normal

O S S E O U S S A R C O I D O S I S

THYROID INVOLVEMENT Involvement of the thyroid gland occurs in 6% of patients with sarcoidosis. It is commonly associated with widespread disease in other organs.

Thyroid ultrasound in 59- years woman shows diffuse enlargement With iso hypo- echoid Heterogenous noduls.

Thyroid biopsy Specimens after loboisthmectomy demonstrates a noncaseating granulomas with epithelioid cells.

Laboratory data show that the angiotensin-converting enzyme (ACE) level is commonly elevated.

Salivary glands biopsy shows the same sarcoidosic Granulomas.

SUMMARY

Sarcoidosis is a multisystem disease and diagnosis must be made on radio-clinic laboratory findings combined with histological evidence of non caseating granulomas.

Because the disease frequently involves multiple organs, familiarity with the clinical and radiologic features of sarcoidosis in various anatomic locations plays a crucial

role in diagnosis and management.

Research is underway to improve imaging to guide the diagnosis and lead to an early and accurate diagnosis.

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