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M. BANI, K. BOUZAIDI, F. SNENE, I. KECHAOU, F. JABNOUN Radiology service, MT Maamouri hospital, Nabeul, Tunisia Pan Arab Association of Radiological societies (PAARS) 2012 VARIOUS : VR 2 Slide 2 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. Slide 3 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. Slide 4 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 ) Slide 5 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. Slide 6 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) Slide 7 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. Slide 8 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. Slide 9 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. Slide 10 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. Slide 11 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. Slide 12 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. Slide 13 Coronal and axial contrast-enhanced T1-weighted MR image show diffusely enhancing leptomeningeal lesions ( arrows) Slide 14 coronal T1-weighted MR image reveal an enlarged hypothalamus Slide 15 On a contrast-enhanced T1-weighted MR image 1- The V Cranial Nerve enhancement 2- The optic chiasm enhancement. Slide 16 T2-weighted MR images demonstrate an intramedullary hyperintense lesion Contrast-enhanced T1-weighted MR image depicts an leptomeningeal enhancing. Slide 17 contrast-enhanced T1-weighted MR image shows a an enhancing intraaxial lesion Slide 18 Coronal / Sagital contrast-enhanced T1-weighted MR image show enhancing covering the Tentorium cerebelli. Slide 19 Liver enlargement is found on ultrasound or CT scan in up to 40% of cases. Its the commonest CT finding, often accompagned with splenomegaly and (less often) abdominal lymph nodes enlargement. Contrast-enhanced abdominal CT scan show homogenous organomegaly. Slide 20 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 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. Slide 21 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. Slide 22 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 Slide 23 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 Slide 24 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. Slide 25 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. Slide 26 Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M,Nagai S. Radiologic manifestations of sarcoidosis in various organs. Radiographics 2004; 24:87-104. Roberts SD, Mirowski GW, Wilkes D, Kwo PY, Knox KS. Sarcoidosis. Part II: extrapulmonary and systemic manifestations. J Am Acad Dermatol 2004; 51: 628-630. Baughman R. Pulmonary sarcoidosis, MD University of Cincinnati Medical Center, USA. Clin Chest 2004; 25: 521530. Kragiannidis A, Karavalki M, Koulaouzidis A. Hepatic sarcoidosis. Annals of Hepatology 2006; 5: 251-256. Mana J. Magnetic resonance imaging and nuclear imaging in sarcoidosis. Curr Opin Pulm Med 2002; 8: 457463. Neville E, Carstairs LS, James DG. Sarcoidosis of bone. Q J Med 1977; 46:215227.