DOI: 10.1148/radiol.2351031848
(Radiology 2005;235:53-55.)
© RSNA, 2005
Case 81: Antiphospholipid Antibody Syndrome with Adrenal Hemorrhage and Budd-Chiari Syndrome1
Mallar Bhattacharya, MD,
Benjamin M. Yeh, MD,
Aliya Qayyum, MB, BS and
Fergus V. Coakley, MD
1 From the Department of Radiology, University of California-San Francisco, Box 0628, M-372, 505 Parnassus Avenue, San Francisco, CA 94143-0628. Received November 14, 2003; revision requested February 6, 2004; revision received February 12; accepted March 23. Address correspondence to F.V.C. (e-mail: fergus.coakley@radiology.ucsf.edu).
 |
History
|
|---|
A 56-year-old man with end-stage liver disease presented to the emergency department with increasing fatigue and an unexplained weight loss of 16 kg over the preceding 3 months. His past medical history included multiple episodes of abdominal pain and ascites for 30 years. He did not consume alcohol. Physical examination demonstrated a blood pressure of 70/50 mm Hg but was otherwise unremarkable. Laboratory studies demonstrated a sodium level of 131 mEq/L (131 mmol/L) (normal range, 135–145 mEq/L [135–145 mmol/L]), potassium level of 5.5 mEq/L (5.5 mmol/L) (normal range, 3.5–5.0 mEq/L [3.5–5.0 mmol/L]), platelet count of 52 x 109/L (normal range, 140–450 x 109/L), and partial thromboplastin time of more than 100 seconds (normal range, 24.5–35.2 seconds). The results of serology were negative for viral hepatitis. Abdominal magnetic resonance (MR) imaging was performed.
|
Imaging Findings
|
|---|
Both the transverse and coronal MR images (Figs 1 and 2) demonstrate inhomogeneity of the hepatic parenchyma with an irregular hepatic contour. Transverse MR images (Fig 1) demonstrate a right adrenal mass with central low T1 signal intensity, peripheral high T1 signal intensity, diffuse high T2 signal intensity, and lack of enhancement after administration of intravenous gadolinium-based contrast material. Coronal MR images (Fig 2) demonstrate enlarged collateral vein, marked narrowing of the intrahepatic inferior vena cava, and splenomegaly.
View larger version (124K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1a. (a) Transverse in-phase spoiled gradient-echo T1-weighted (repetition time msec/echo time msec, 150/4.2; flip angle, 70°) MR image of the upper abdomen. The liver has an irregular contour (vertical arrows), with an inhomogeneous parenchyma and borderline prominence of the caudate lobe (*). A right adrenal mass (horizontal arrow) is of centrally low T1 signal intensity, with a rim of high T1 signal intensity. (b) Transverse fat-saturated fast-recovery rapid-acquisition refocused-echo T2-weighted (2500/100) MR image of the upper abdomen. Hepatic contour irregularity and parenchymal inhomogeneity are again evident. Right adrenal mass (arrow) is of high T2 signal intensity. (c) Transverse in-phase spoiled gradient-echo T1-weighted (150/4.2; flip angle, 70°) fat-saturated MR image of the upper abdomen obtained after intravenous administration of gadolinium chelate. The right adrenal mass (arrow) is nonenhancing.
|
|
View larger version (116K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1b. (a) Transverse in-phase spoiled gradient-echo T1-weighted (repetition time msec/echo time msec, 150/4.2; flip angle, 70°) MR image of the upper abdomen. The liver has an irregular contour (vertical arrows), with an inhomogeneous parenchyma and borderline prominence of the caudate lobe (*). A right adrenal mass (horizontal arrow) is of centrally low T1 signal intensity, with a rim of high T1 signal intensity. (b) Transverse fat-saturated fast-recovery rapid-acquisition refocused-echo T2-weighted (2500/100) MR image of the upper abdomen. Hepatic contour irregularity and parenchymal inhomogeneity are again evident. Right adrenal mass (arrow) is of high T2 signal intensity. (c) Transverse in-phase spoiled gradient-echo T1-weighted (150/4.2; flip angle, 70°) fat-saturated MR image of the upper abdomen obtained after intravenous administration of gadolinium chelate. The right adrenal mass (arrow) is nonenhancing.
|
|
View larger version (113K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1c. (a) Transverse in-phase spoiled gradient-echo T1-weighted (repetition time msec/echo time msec, 150/4.2; flip angle, 70°) MR image of the upper abdomen. The liver has an irregular contour (vertical arrows), with an inhomogeneous parenchyma and borderline prominence of the caudate lobe (*). A right adrenal mass (horizontal arrow) is of centrally low T1 signal intensity, with a rim of high T1 signal intensity. (b) Transverse fat-saturated fast-recovery rapid-acquisition refocused-echo T2-weighted (2500/100) MR image of the upper abdomen. Hepatic contour irregularity and parenchymal inhomogeneity are again evident. Right adrenal mass (arrow) is of high T2 signal intensity. (c) Transverse in-phase spoiled gradient-echo T1-weighted (150/4.2; flip angle, 70°) fat-saturated MR image of the upper abdomen obtained after intravenous administration of gadolinium chelate. The right adrenal mass (arrow) is nonenhancing.
|
|
View larger version (175K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2a. (a) Coronal steady-state fast gradient-echo (4.0/2.0; flip angle, 70°) MR image of the upper abdomen obtained in the plane of the intrahepatic inferior vena cava. The superior portion of the intrahepatic inferior vena cava is markedly narrowed (arrow). The spleen is enlarged. (b) Steady-state fast gradient-echo (4.0/2.0; flip angle, 70°) coronal subvolume maximum intensity projection reformatted MR image of the upper abdomen. Collateral vein (white arrows) is seen arising from the expected confluence of the hepatic veins with the inferior vena cava, coursing to the left, and communicating with a cluster of variceal collaterals (black arrow) adjacent to the cardiac apex.
|
|
View larger version (184K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2b. (a) Coronal steady-state fast gradient-echo (4.0/2.0; flip angle, 70°) MR image of the upper abdomen obtained in the plane of the intrahepatic inferior vena cava. The superior portion of the intrahepatic inferior vena cava is markedly narrowed (arrow). The spleen is enlarged. (b) Steady-state fast gradient-echo (4.0/2.0; flip angle, 70°) coronal subvolume maximum intensity projection reformatted MR image of the upper abdomen. Collateral vein (white arrows) is seen arising from the expected confluence of the hepatic veins with the inferior vena cava, coursing to the left, and communicating with a cluster of variceal collaterals (black arrow) adjacent to the cardiac apex.
|
|
|
DISCUSSION
|
|---|
MR imaging findings are those of Budd-Chiari syndrome and adrenal hemorrhage. The combination of Budd-Chiari syndrome and adrenal hemorrhage (both of which can be caused by venous thrombosis), in conjunction with a disproportionately elevated partial thromboplastin time, is highly suggestive of antiphospholipid antibody syndrome, which was confirmed by a positive anticardiolipin antibody test.
The antiphospholipid antibody syndrome is characterized by venous and arterial thromboses, thrombocytopenia, recurrent spontaneous abortion, and positive serologic tests for autoantibodies to phospholipids such as anticardiolipin antibody and lupus anticoagulant (1,2). These autoantibodies are procoagulant in vivo but anticoagulant in vitro. The latter frequently results in marked elevation of the partial thromboplastin time. In a series of 47 patients with antiphospholipid antibody syndrome, 45 had partial thromboplastin times of 46–150 seconds (3). Such gross elevation of partial thromboplastin time would be unlikely in cirrhotic liver synthetic dysfunction alone [M. Peters, unpublished data, 2004].
The abdominal imaging findings in antiphospholipid antibody syndrome have been well described. In a retrospective study of 160 hospitalized patients with antiphospholipid antibody syndrome who underwent CT at admission, 42 (26%) were found to have abdominal thrombotic or ischemic findings, including major vessel thromboses, splenic infarction, pancreatitis, and bowel wall thickening (4). Of 14 patients with antiphospholipid antibody syndrome, venous thromboses were seen in 10 and arterial thromboses were seen in four (5). Both Budd-Chiari syndrome and adrenal hemorrhage are part of the thrombotic spectrum of findings in antiphospholipid antibody syndrome.
Strictly, Budd-Chiari syndrome consists of a clinical combination of abdominal pain, jaundice, hepatomegaly, and ascites; however, in radiologic usage, this term generally refers to the characteristic imaging findings seen in a group of conditions characterized by hepatic venous obstruction (6). The syndrome may be primary, due to a membrane in the inferior vena cava, or secondary to neoplasms, oral contraceptive use, pregnancy, hypercoagulable states, myeloproliferative disorders, and paroxysmal nocturnal hemoglobinuria. In practice, no identifiable cause is found in up to 70% of patients (7). Antiphospholipid antibody syndrome is one of the recognized hypercoagulable states that may cause Budd-Chiari syndrome, and it was the only identifiable cause in four of 22 patients with nonneoplastic Budd-Chiari syndrome (8).
Common imaging findings in Budd-Chiari syndrome include hepatosplenomegaly, caudate lobe enlargement, ascites, and hepatic parenchymal inhomogeneity (6). Intraluminal clot may be seen in the acute phase. Findings in the chronic phase include nonvisualization of occluded veins and formation of collateral veins within the liver or from the liver to either the left renal vein or the pericardiophrenic vein (9–11). The appearance of an enlarged collateral vein to the pericardiophrenic vein, as seen in our patient, is the result of shunting of intrahepatic venous flow; this finding was seen in two of nine patients with Budd-Chiari syndrome and intrahepatic collateralization in one series (12). The inferior vena caval ostium of the left hepatic vein lies adjacent to that of the left inferior phrenic vein in 75% of cases. With caval obstruction or stenosis, flow may be shunted from intrahepatic vessels to the left hepatic vein, from the left hepatic vein to the inferior phrenic vein, and from the left inferior phrenic vein to the pericardiophrenic vein—which drains into the left brachiocephalic vein (10).
The findings of fatigue, weight loss, hypotension, hyponatremia, and hyperkalemia in our patient are consistent with adrenal insufficiency. Adrenal insufficiency is a recognized consequence of antiphospholipid antibody syndrome (13), and it may be due to adrenal vein thrombosis, resulting in parenchymal hemorrhage because of the profuse small venous channels found throughout the adrenal gland (14). The MR findings of adrenal hemorrhage vary according to the time course of hematoma formation (15). In the subacute setting (1–7 weeks), the hematoma is of high T1 and T2 signal intensity. The T1 hyperintensity may first be seen as a rim of high signal intensity (Fig 1a). In the present case, adrenal hemorrhage was seen in the right adrenal gland, but unilateral hemorrhage would not account for the patient’s adrenal insufficiency. One may speculate that the left adrenal gland was damaged by previous episodes of hemorrhage that were not radiologically evident.
This case underscores the importance of abdominal imaging findings in facilitating the diagnosis of a disease in which treatment with aggressive anticoagulation can be life saving. The combination of Budd-Chiari syndrome and adrenal hemorrhage (both of which can be caused by venous thrombosis), in conjunction with a disproportionately elevated partial thromboplastin time, is highly suggestive of antiphospholipid antibody syndrome.
|
FOOTNOTES
|
|---|
Authors stated no financial relationship to disclose.
|
REFERENCES
|
|---|
- Harris EN, Gharavi AE, Hughes GRV. Antiphospholipid antibodies. Clin Rheum Dis 1985; 11:591-609.[Medline]
- Harris EN, Baguly E, Asherson RA, Hughes GRV. Clinical and serological features of the antiphospholipid syndrome [abstr]. Br J Rheumatol 1987; 26(suppl 2):19.
- Rote NS, Dostal-Johnson D, Branch DW. Antiphospholipid antibodies and recurrent pregnancy loss: correlation between the activated partial thromboplastin time and antibodies against phosphatidylserine and cardiolipin. Am J Obstet Gynecol 1990; 163:575-584.[Medline]
- Kaushik S, Federle MP, Schur PH, Krishnan M, Silverman SG, Ros PR. Abdominal thrombotic and ischemic manifestations of the antiphospholipid antibody syndrome: CT findings in 42 patients. Radiology 2001; 218:768-771.[Abstract/Free Full Text]
- Kim JH, Ha HK, Yoon KH, et al. CT features of abdominal manifestations of primary antiphospholipid antibody syndrome. J Comput Assist Tomog 1999; 23:678-683.[CrossRef][Medline]
- Blum U, Rossle M, Haag K, et al. Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology 1995; 197:805-811.[Abstract/Free Full Text]
- Soyer P, Rabenandrasana A, Barge J, et al. MRI of Budd-Chiari syndrome. Abdom Imaging 1994; 19:325-329.[Medline]
- Pelletier S, Landi B, Piette JC, et al. Antiphospholipid antibody syndrome as the second cause of non-tumorous Budd-Chiari syndrome. J of Hepatology 1994; 21:76-80.
- Miller WJ, Federle MP, Straub WH, Davis PL. Budd-Chiari syndrome: imaging with pathologic correlation. Abdom Imaging 1993; 18:329-335.[CrossRef][Medline]
- Cho O, Koo J, Kim Y, Rhim H, Koh B, Seo H. Collateral pathways in Budd-Chiari syndrome: CT and venographic correlation. AJR Am J Roentgenol 1996; 167:1163-1167.[Free Full Text]
- Redmond PL, Kadir S, Cameron JL. Transhepatic venous collaterals in a patient with the Budd-Chiari syndrome. Cardiovasc Intervent Radiol 1988; 11:285-287.[Medline]
- Lin J, Chen X, Zhou K, et al. Budd-Chiari syndrome: diagnosis with three-dimensional contrast-enhanced magnetic resonance angiography. World of Gastroenterology 2003; 9:2317-2321.
- Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome. Lupus 2003; 12:569-572.[Abstract/Free Full Text]
- Fox B. Venous infarction of the adrenal glands. J Pathol 1976; 119:65-89.[CrossRef][Medline]
- Kawashima A, Sandler CM, Ernst RD, et al. Imaging of non-traumatic hemorrhage of the adrenal gland. RadioGraphics 1999; 19:949-963.[Abstract/Free Full Text]
Congratulations to the seven individuals who submitted the most likely diagnosis (antiphospholipid antibody syndrome with adrenal hemorrhage and Budd-Chiari syndrome) for Diagnosis Please, Case 81. Credit was given only if antiphospholipid antibody syndrome was included in the submitted diagnosis. The names and locations of the individuals, as submitted, are as follows:
- Gilles Genin, MD, Annecy, France
- David Klein, MD, Fairfield, Conn
- Mario Laguna, West Allis, Wis
- John A. Mattingly, MD, Belleville, Ill
- Tammam Nehme, East Wenatchee, Wash
- Shawn P. Quillin, MD, Charlotte, NC
- Meriç Tüzün, Ankara, Turkey
TOP
History
Imaging Findings
