Hepatic Manifestations of the Antiphospholipid Syndrome
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EDITORIAL Hepatic Manifestations of the Antiphospholipid Syndrome Key words: autoimmune hepatitis, Sjogren's syndrome, Budd- autoimmune hepatitis (7). Examinations prior to liver biopsy Chiari syndrome, nodular regenerative hyperpla- revealed a greatly prolonged APTT,and the patient was found sia to be positive for IgG anticardiolipin antibody and lupus anti- coagulant. However, this patient showedno signs of thrombo- sis or any other symptomssuggestive ofAPS. In this issue, Katayama et al (8) report a case of Sjogren's Antiphospholipid syndrome (APS) is characterized by the syndrome complicated with autoimmune hepatitis and APS. presence of antiphospholipid antibodies (anticardiolipin anti- bodies and/or lupus anticoagulant) in association with symp- See also p 73. toms including venous/arterial thromboses, recurrent fetal loss and thrombocytopenia. Thromboticepisodes mayoccur at any In previously reported cases of autoimmune hepatitis asso- part of the circulation system, hence, any organ may be in- ciated with the presence of antiphospholipid antibodies, anti- volved. As for hepatic involvement, the most well knownmani- nuclear antibody was present, but the presence of anti-SS-A festation ofAPSis Budd-Chiari syndrome, which is caused antibody or sicca symptomshave not been documented. There- mostly by obstruction of hepatic veins or inferior vena cava. fore, this case may possibly be the first documented case of Pelletier et al (1) have reported that of their 22 non-tumourous autoimmune hepatitis with APSand Sjogren's syndrome. Their Budd-Chiari patients, 4 had antiphospholipid antibodies with- patient had autoimmune hepatitis, and Sjogren's syndrome out other causes of hepatic vein thrombosis. Amongthese 4 confirmed by Schirmer's test and sialography. The patient patients, 2 seemed to have primary APS, i.e., no signs of other showed positive for (32-GPI dependent anticardiolipin antibody autoimmunediseases such as SLE. Thus, APSshould be con- and lupus anticoagulant. This patient did not showany signs sidered as a cause of unexplained Budd-Chiari syndrome. An- of thrombosis, but marked thrombocytopenia, which responded other uncommonbut important hepatic complication of APS favourably to 30 mg/day of prednisolone, was observed. Al- is nodular regenerative hyperplasia (NRH). NRHis occasion- though not included in the recently proposed diagnostic crite- ally found in patients with SLE, and association with the pres- ria for APS(9), thrombocytopenia is a well known complica- ence of antiphospholipid antibodies has been documented. NRH tion ofAPS. In addition, Katayamaet al have found that serum has also been found in patients with primary APS (2, 3). Since sIL2R, IFNy and GM-CSFwere elevated in this patient, and NRHmay lead to portal hypertension and variceal hemorrhage, suggested that ThO-Thlcells may be activated in this patient. it is important to detect its presence. MRImay be useful among Of course, this mayor maynot be commonin patients with the more noninvasive examinations. Other hepatic manifesta- this condition, and accumulationof similar cases is necessary. tions of APSinclude hepatic veno-occlusive disease, hepatic Although Sjogren's syndrome is a well recognized compli- infarction, occlusion of small hepatic veins and portal hyper- cation of autoimmunehepatitis, association of APSand au- tension (4). toimmunehepatitis is not well known. As both are autoim- Recently, possible association of autoimmunehepatitis and munediseases, a thorough survey of patients with autoimmune presence of antiphospholipid antibodies have been reported. hepatitis mayreveal a more close connection between these Nagayamaet al reported a case of a 57-year-old female, who conditions. presented with multiple cerebral infarctions and livedo Whenan APS patient is found to have liver dysfunction or reticularis (5). She had elevated transaminase activity, and portal hypertension, it maybe purely coincidental, such as an showed positivity for antinuclear, anti-DNA, anti-smooth association of HCVhepatitis, but mayalso be associated with muscle, IgManticardiolipin antibodies, and lupus anticoagu- APS in some way. These possibilities should always be con- lant. Liver biopsy revealed changes compatible with autoim- sidered. mune hepatitis. The patient was successfully treated with ursodeoxycholic acid, anticoagulant and antiplatelet therapies. Akito Tsutsumi, MDand Takao Koike, MD Kobayashi et al reported a 76-year-old female with autoim- The Department of Medicine II, Hokkaido University School of Medicine, mune hepatitis (6). During treatment with prednisolone, the Kital5, Nishi7, Kita-ku, Sapporo 060-8635 patient developed lung infarction, and subsequent examina- References tion revealed the presence of IgManticardiolipin antibody. This patient was successfully treated with warfarin. In another case, 1) Pelletier S, Landi B, Piette JC, et al. Antiphospholipid syndrome as the reported by Pathmakanthan et al, a 24-year male developed 6 Internal Medicine Vol. 39, No. 1 (January 2000) second cause of non-tumorous Budd-Chiari syndrome. J Hepatol 21: 76- Shokakibyo Gakkai Zasshi 94: 695-699, 1997 (In Japanese). 80,1994. 6) Kobayashi N, Sakai M, Ohta M, et al. A case of autoimmune hepatitis 2) Perez Ruiz F, Orte Martinez FJ, Zea Mendoza AC, Ruiz del Arbol L, with anti-phospholipid syndrome. Nippon Shokakibyo Gakkai Zasshi 95: Moreno Caparros A. Nodular regenerative hyperplasia of the liver in rheu- 557-562, 1998 (In Japanese). matic diseases: report of seven cases and review of the literature. Semin 7) Pathmakanthan S, Kay EW, Murray FE. Autoimmune chronic active hepa- Arthritis Rheum 21: 47-54, 1991. titis associated with the presence of antiphospholipid antibodies. Eur J 3) Minoshima S, Motohashi S, Soejima A, et al. A case of antiphospholipid Gastroenterol Hepatol 10: 155-157, 1998. syndrome with nodular regenerative hyperplasia and portal hypertension. 8) Katayama Y, Kohriyama K, Kirizuka K, Nishizaki H, Fujii H, Tanji Y. Nippon Naika Gakkai Zasshi 79: 1451-1452, 1990 (In Japanese). Sjogren's syndrome complicated with autoimmunehepatitis and anti- 4) Asherson RA. Adrenal, hepatic, and other intraabdominal manifestations phospholipid antibody syndrome. Intern Med 39: 73-76, 2000. in the antiphospholipid syndrome, in: The Antiphospholipid Syndrome, 9) Wilson WA, Gharavi AE, Koike T, et al. International consensus state- Asherson RA, Cervera R, Piette J-C, Schoenfeld Y, Eds. CRCPress, Boca menton preliminary classification criteria for definite antiphospholipid Raton, FL. 1996: 183-194. syndrome: report of an international workshop. Arthritis Rheum42: 1 309- 5) NagayamaK, Izumi N, Watanabe H, et al. A case of autoimmune hepati- 1311, 1999. tis associated with anti-phospholipid antibody syndrome. Nippon 7 Internal Medicine Vol. 39, No. 1 (January 2000).