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Abdominal manifestations of sarcoidosis CT appearances
ELSEVIER
ABDOMINAL MANIFESTATIONS OF SARCOIDOSIS (X APPEARANCES J. FARMAN, MB, CHB, MMED, G. RAMIREZ, MD, J. BRUNETTI, J. TUVIA, MD, C. NG, MD, AND H. ROTTERDAM, MD
Sarcoidosis frequently involves the abdomen, although imaging studies often fail to demonstmte disease. The most common computed tomography (CT) findings in abdominal sarcoidosis are hepatosplenomegaly and retroperitoneal adenopathy, followed by focal low-attenuation lesions of the liver and spleen. Other abdominal viscem are involved infrequently. We present four cases of abdominal sarcoidosis demonstrating a range of CT findings. KEY WORDS:
Sarcoidosis; Retroperitonealadenopathy; Liver; Spleen INTRODUCTION Sarcoidosis is a multisystem granulomatous process manifested by hilar adenopathy and pulmonary interstitial disease in the majority of patients. The principal areas of involvement are the chest, eyes, skin, abdominal viscera, central nervous system, and bones. Extrathoracic disease can occur in association with or in the absence of intrathoracic disease. Although granulomatous hepatitis secondary to sarcoidosis is a frequent finding in liver biopsy specimens, radiographic demonstration of liver involvement is rare. The most common computed tomography (CT) findings in abdominal sarcoidosis are retroperitoneal adenopathy and hepatosplenomegaly. In a minority of patients, focal hepatic and splenic lesions may be appreciated on CT scans. We report four cases of abdominal sarcoidosis with significant CT findings. In three
From the Departments of Radiology (J.F., G.R., J.B., J.T.) and Pathology (H.R.), Columbia Presbyterian Medical Center, New York, New York, and Park view Medical Center, Nashville, Tennessee (C.N.). Address reprint requests to: J. Farman, MB, ChB, MMed, Department of Radiology, Columbia Presbyterian Medical Center, 622 West 168th Street, New York, NY 10032. Received February 15, 1994; accepted March 8, 1994. CLINICAL IMAGING 1995;19:30-33 o Elsevier Science Inc., 1995 655 Avenue of the Americas, New York, NY 10010
MD,
patients, associated pulmonary sarcoidosis was present and in one patient, disease was limited to the abdomen. CASE REPORTS Case 1 A 53-year-old Hispanic man with severe pulmonary sarcoidosis treated with prednisone, 40 mg daily for several years, presented with complaints of low-grade fever and malaise. Physical examination was notable only for a palpable spleen. Direct and indirect bilirubin levels were elevated at 3.0 and 0.4 mg/dL, respectively (normal, 0.3 to 1.2 and 0.1 to 0.3 mg/dL). The alkaline phosphatase level was 293 II-I/L (normal, 90 to 200 III/L). Abdominal CT demonstrated splenomegaly and extensive retroperitoneal adenopathy (Figure 1). CTguided biopsy of peripancreatic nodes was performed and noncaseating granulomata were identified on microscopic evaluation (Figure 2). Stains for acid-fast bacilli and fungi were negative. Tuberculosis culture was also negative.
Case 2 A 32-year-old white man with a medical history of insulin-dependent diabetes mellitus for 16 years presented initially to his physician with right axillary swelling. The patient was informed that he had “matted lymph nodes” as determined by biopsy and received no treatment. Recurrent episodes of low-grade fever and malaise caused the patient to seek further medical attention 3 years later, at which time the aspartate aminotransferase level was 85 III/L (normal, 0 to 50 III/L), gamma-glutamyl transpeptidase was 180 IU/L (normal, 0 to 65 III/L), and the alkaline phosphatase level was 365 III/L (normal, 40 to 150 III/L). Physical examination revealed the presence of a right axillary 0899-7071/95/$9.50 SSDI 0899-7071(94)00022-5
JANUARY-MARCH
1995
ABDOMINAL
MANIFESTATIONS
OF SARCOIDOSIS:
FIGURE 1. (A) Multiple discrete lymph nodes are evident in the peripancreatic, CT slice demonstrates the presence of numerous paraaortic nodes.
scar and a palpable spleen. The chest radiograph appeared normal. Abdominal CT demonstrated hepatosplenomegaly with multiple low-attenuation lesions throughout the liver and spleen as well as extensive retroperitoneal adenopathy (Figure 3). Liver and
biopsy
retrocrural,
CT APPEARANCES
31
and celiac regions. (B) Lower
bone marrow biopsies were performed and the specimens showed noncaseating granulomata. Stains for acid-fast bacilli and fungi were negative, as was tuberculosis culture.
Case 3 FIGURE 2. Needle biopsy specimen of a peripancreatic lymph node. A nonnecrotizing granuloma with a central multinucleated giant cell surrounded by epithelioid histiocytes is seen in the lower right corner. At the periphery there is lymphoid tissue mixed with fresh blood (H&E, original magnification x 250).
A !%year-old woman with bronchoscopic biopsyproved sarcoidosis and end-stage interstitial lung disease with recurrent bouts of bronchitis underwent chest CT to evaluate bronchiectasis. Incidentally noted were multiple focal hepatic and splenic lesions and retroperitoneal adenopathy (Figure 4). The alkaline phosphatase level was mildly elevated at 185 IU/L. In view of the confirmed sarcoidosis involving the chest, further evaluation of the abdomen was regarded as unnecessary. The patient’s symptoms have remained unchanged for 2 years, without a change in blood chemistries. Case 4 A 30-year-old man with a 4-year history of recurrent bouts of uveitis and stage 2 chest sarcoidosis proved by transbronchial biopsy (Figure 5) had an abdominal CT for evaluation of inferior vena caval patency in the work-up of deep venous thrombosis of the lower extremities. The CT demonstrated marked splenomegaly with multiple focal lesions and extensive retroperitoneal adenopathy (Figure 6). Even with the pa-
32
CLINICAL IMAGING VOL. 19, NO. 1
FARMAN ET AL.
’
A
FIGURE
4. (A) On precontrast CT scan, paraaortic adenopathy is evident. (B) Postcontrast CT scan demonstrates hepatosplenomegaly with numerous low-attenuation areas in both organs.
JANUARY-MARCH
1995
ABDOMINAL MANIFESTATIONS
6. CT scan of the abdomen demonstrating hepatosplenomegaly with multiple focal splenic lesions and celiac, retrocrural, and preipancreatic adenopathy. Scattered tiny low-attenuation lesions are present predominantly in the right lobe of the liver. FIGURE
tient’s documented sarcoidosis in the chest and presumed eye involvement, CT-guided biopsy of retroperitoneal nodes was performed to rule out lymphoma.
Microscopic evaluation of the biopsy specimen confirmed the diagnosis of sarcoidosis.
Autopsy studies of sarcoid patients have demonstrated the frequency of abdominal and pelvic adenopathy to be as high as 90% and that of liver granulomata to be
as high as 70% (1). Clinically, however, only 20% of patients with sarcoidosis have hepatosplenomegaly. Moreover, abnormal liver enzyme levels are found in only 40% and the percentage of patients with advanced liver disease [e.g., chronic cholestasis and portal hypertension) is approximately 1% (2). Radiographically evident abdominal sarcoid, therefore, may be present in the absence of a clinical or laboratory abnormality.
33
On abdominal CT scans, sarcoid is manifested by the presence of adenopathy, splenomegaly, and hepatomegaly. Adenopathy is present most frequently in the paraaortic, paracaval, and peripancreatic regions. Less commonly, there is enlargement of gastmhepatic, mesenteric, pelvic, and retrocrural nodes (3). Often there is no correlation between the extent of involvement of pulmonary and abdominal sarcoidosis. As in case 2, for example, extensive abdominal sarcoidosis may be present in the absence of pulmonary findings. Although focal hepatic and splenic lesions have been reported, the most frequently described CT finding in hepatic and splenic sarcoidosis is diffuse parenchymal heterogeneity. Histologically, sarcoid granulomata are frequently less than 2 mm in diameter and it is likely that the inhomogeneity described on abdominal CT is the result of imaging poorly resolved tiny lesions (7). In two of our patients (cases 2 and 4), however, diffusely distributed focal lesions were identified. With higher-resolution CT, these small granulomata may be a more frequent finding on abdominal scans in patients with sarcoidosis. REFERENCES 1.
DISCUSSION
OF SARCOIDOSIS: CT APPEARANCES
Iwai K, Oka H. Sarcoidosis: report of ten autopsy cases in Japan. Am Rev Respir Dis 1964;90:612-622.
2. Takada K, Ina Y, Noda M, Sato T, Yamamoto M, Morishita M. The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol 1993:46:359-366. 3. Britt AR, Francis IR, Glazer GM, Illis JH. Sarcoidosis: abdominal manifestations at CT. Radiology 1991;178:91-94. 4. Sagalow BR, Miller CL, Wechsler RJ. Pancreatic sarcoidosis mimicking pancreatic cancer. J Clin Ultrasound 1988;16:131-134. 5. Sartin JS, Walker RC. Granulomatous hepatitis: a retrospective review of 88 cases at the Mavo Clinic. Mavo Clin Proc 199X66: 914-918.
6. Meranze S, Coleman B, Arger P, Mintz M, Markow L. Retroperitoneal manifestations of sarcoidosis on computed tomography. J Comput Assist Tomogr 1985;9:50-52. 7. Nakata K, Iwata K, Kojima K, Kanai K. Computed tomography of liver sarcoidosis. J Comput Assist Tomogr 1989;13:7(n-708.
ABDOMINAL MANIFESTATIONS OF SARCOIDOSIS (X APPEARANCES J. FARMAN, MB, CHB, MMED, G. RAMIREZ, MD, J. BRUNETTI, J. TUVIA, MD, C. NG, MD, AND H. ROTTERDAM, MD
Sarcoidosis frequently involves the abdomen, although imaging studies often fail to demonstmte disease. The most common computed tomography (CT) findings in abdominal sarcoidosis are hepatosplenomegaly and retroperitoneal adenopathy, followed by focal low-attenuation lesions of the liver and spleen. Other abdominal viscem are involved infrequently. We present four cases of abdominal sarcoidosis demonstrating a range of CT findings. KEY WORDS:
Sarcoidosis; Retroperitonealadenopathy; Liver; Spleen INTRODUCTION Sarcoidosis is a multisystem granulomatous process manifested by hilar adenopathy and pulmonary interstitial disease in the majority of patients. The principal areas of involvement are the chest, eyes, skin, abdominal viscera, central nervous system, and bones. Extrathoracic disease can occur in association with or in the absence of intrathoracic disease. Although granulomatous hepatitis secondary to sarcoidosis is a frequent finding in liver biopsy specimens, radiographic demonstration of liver involvement is rare. The most common computed tomography (CT) findings in abdominal sarcoidosis are retroperitoneal adenopathy and hepatosplenomegaly. In a minority of patients, focal hepatic and splenic lesions may be appreciated on CT scans. We report four cases of abdominal sarcoidosis with significant CT findings. In three
From the Departments of Radiology (J.F., G.R., J.B., J.T.) and Pathology (H.R.), Columbia Presbyterian Medical Center, New York, New York, and Park view Medical Center, Nashville, Tennessee (C.N.). Address reprint requests to: J. Farman, MB, ChB, MMed, Department of Radiology, Columbia Presbyterian Medical Center, 622 West 168th Street, New York, NY 10032. Received February 15, 1994; accepted March 8, 1994. CLINICAL IMAGING 1995;19:30-33 o Elsevier Science Inc., 1995 655 Avenue of the Americas, New York, NY 10010
MD,
patients, associated pulmonary sarcoidosis was present and in one patient, disease was limited to the abdomen. CASE REPORTS Case 1 A 53-year-old Hispanic man with severe pulmonary sarcoidosis treated with prednisone, 40 mg daily for several years, presented with complaints of low-grade fever and malaise. Physical examination was notable only for a palpable spleen. Direct and indirect bilirubin levels were elevated at 3.0 and 0.4 mg/dL, respectively (normal, 0.3 to 1.2 and 0.1 to 0.3 mg/dL). The alkaline phosphatase level was 293 II-I/L (normal, 90 to 200 III/L). Abdominal CT demonstrated splenomegaly and extensive retroperitoneal adenopathy (Figure 1). CTguided biopsy of peripancreatic nodes was performed and noncaseating granulomata were identified on microscopic evaluation (Figure 2). Stains for acid-fast bacilli and fungi were negative. Tuberculosis culture was also negative.
Case 2 A 32-year-old white man with a medical history of insulin-dependent diabetes mellitus for 16 years presented initially to his physician with right axillary swelling. The patient was informed that he had “matted lymph nodes” as determined by biopsy and received no treatment. Recurrent episodes of low-grade fever and malaise caused the patient to seek further medical attention 3 years later, at which time the aspartate aminotransferase level was 85 III/L (normal, 0 to 50 III/L), gamma-glutamyl transpeptidase was 180 IU/L (normal, 0 to 65 III/L), and the alkaline phosphatase level was 365 III/L (normal, 40 to 150 III/L). Physical examination revealed the presence of a right axillary 0899-7071/95/$9.50 SSDI 0899-7071(94)00022-5
JANUARY-MARCH
1995
ABDOMINAL
MANIFESTATIONS
OF SARCOIDOSIS:
FIGURE 1. (A) Multiple discrete lymph nodes are evident in the peripancreatic, CT slice demonstrates the presence of numerous paraaortic nodes.
scar and a palpable spleen. The chest radiograph appeared normal. Abdominal CT demonstrated hepatosplenomegaly with multiple low-attenuation lesions throughout the liver and spleen as well as extensive retroperitoneal adenopathy (Figure 3). Liver and
biopsy
retrocrural,
CT APPEARANCES
31
and celiac regions. (B) Lower
bone marrow biopsies were performed and the specimens showed noncaseating granulomata. Stains for acid-fast bacilli and fungi were negative, as was tuberculosis culture.
Case 3 FIGURE 2. Needle biopsy specimen of a peripancreatic lymph node. A nonnecrotizing granuloma with a central multinucleated giant cell surrounded by epithelioid histiocytes is seen in the lower right corner. At the periphery there is lymphoid tissue mixed with fresh blood (H&E, original magnification x 250).
A !%year-old woman with bronchoscopic biopsyproved sarcoidosis and end-stage interstitial lung disease with recurrent bouts of bronchitis underwent chest CT to evaluate bronchiectasis. Incidentally noted were multiple focal hepatic and splenic lesions and retroperitoneal adenopathy (Figure 4). The alkaline phosphatase level was mildly elevated at 185 IU/L. In view of the confirmed sarcoidosis involving the chest, further evaluation of the abdomen was regarded as unnecessary. The patient’s symptoms have remained unchanged for 2 years, without a change in blood chemistries. Case 4 A 30-year-old man with a 4-year history of recurrent bouts of uveitis and stage 2 chest sarcoidosis proved by transbronchial biopsy (Figure 5) had an abdominal CT for evaluation of inferior vena caval patency in the work-up of deep venous thrombosis of the lower extremities. The CT demonstrated marked splenomegaly with multiple focal lesions and extensive retroperitoneal adenopathy (Figure 6). Even with the pa-
32
CLINICAL IMAGING VOL. 19, NO. 1
FARMAN ET AL.
’
A
FIGURE
4. (A) On precontrast CT scan, paraaortic adenopathy is evident. (B) Postcontrast CT scan demonstrates hepatosplenomegaly with numerous low-attenuation areas in both organs.
JANUARY-MARCH
1995
ABDOMINAL MANIFESTATIONS
6. CT scan of the abdomen demonstrating hepatosplenomegaly with multiple focal splenic lesions and celiac, retrocrural, and preipancreatic adenopathy. Scattered tiny low-attenuation lesions are present predominantly in the right lobe of the liver. FIGURE
tient’s documented sarcoidosis in the chest and presumed eye involvement, CT-guided biopsy of retroperitoneal nodes was performed to rule out lymphoma.
Microscopic evaluation of the biopsy specimen confirmed the diagnosis of sarcoidosis.
Autopsy studies of sarcoid patients have demonstrated the frequency of abdominal and pelvic adenopathy to be as high as 90% and that of liver granulomata to be
as high as 70% (1). Clinically, however, only 20% of patients with sarcoidosis have hepatosplenomegaly. Moreover, abnormal liver enzyme levels are found in only 40% and the percentage of patients with advanced liver disease [e.g., chronic cholestasis and portal hypertension) is approximately 1% (2). Radiographically evident abdominal sarcoid, therefore, may be present in the absence of a clinical or laboratory abnormality.
33
On abdominal CT scans, sarcoid is manifested by the presence of adenopathy, splenomegaly, and hepatomegaly. Adenopathy is present most frequently in the paraaortic, paracaval, and peripancreatic regions. Less commonly, there is enlargement of gastmhepatic, mesenteric, pelvic, and retrocrural nodes (3). Often there is no correlation between the extent of involvement of pulmonary and abdominal sarcoidosis. As in case 2, for example, extensive abdominal sarcoidosis may be present in the absence of pulmonary findings. Although focal hepatic and splenic lesions have been reported, the most frequently described CT finding in hepatic and splenic sarcoidosis is diffuse parenchymal heterogeneity. Histologically, sarcoid granulomata are frequently less than 2 mm in diameter and it is likely that the inhomogeneity described on abdominal CT is the result of imaging poorly resolved tiny lesions (7). In two of our patients (cases 2 and 4), however, diffusely distributed focal lesions were identified. With higher-resolution CT, these small granulomata may be a more frequent finding on abdominal scans in patients with sarcoidosis. REFERENCES 1.
DISCUSSION
OF SARCOIDOSIS: CT APPEARANCES
Iwai K, Oka H. Sarcoidosis: report of ten autopsy cases in Japan. Am Rev Respir Dis 1964;90:612-622.
2. Takada K, Ina Y, Noda M, Sato T, Yamamoto M, Morishita M. The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol 1993:46:359-366. 3. Britt AR, Francis IR, Glazer GM, Illis JH. Sarcoidosis: abdominal manifestations at CT. Radiology 1991;178:91-94. 4. Sagalow BR, Miller CL, Wechsler RJ. Pancreatic sarcoidosis mimicking pancreatic cancer. J Clin Ultrasound 1988;16:131-134. 5. Sartin JS, Walker RC. Granulomatous hepatitis: a retrospective review of 88 cases at the Mavo Clinic. Mavo Clin Proc 199X66: 914-918.
6. Meranze S, Coleman B, Arger P, Mintz M, Markow L. Retroperitoneal manifestations of sarcoidosis on computed tomography. J Comput Assist Tomogr 1985;9:50-52. 7. Nakata K, Iwata K, Kojima K, Kanai K. Computed tomography of liver sarcoidosis. J Comput Assist Tomogr 1989;13:7(n-708.