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1
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Imran S, Thabah MM, Azharudeen M, Ramesh A, Bobby Z, Negi VS. Liver Abnormalities in Systemic Lupus Erythematosus: A Prospective Observational Study. Cureus 2021; 13:e15691. [PMID: 34277279 PMCID: PMC8284411 DOI: 10.7759/cureus.15691] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/16/2021] [Indexed: 11/12/2022] Open
Abstract
Objectives In this study, we aimed to examine and analyze liver abnormalities among patients with systemic lupus erythematosus (SLE), including both newly diagnosed patients and those being followed up, as well as the prevalence of lupus hepatitis. Methods This was a prospective observational study. Clinical data, liver function tests (LFTs), and the findings from the ultrasonography of the abdomen among the patients were prospectively recorded and evaluated. Results Overall, 28 of the total 135 (20.7%) patients had liver abnormalities, including biochemical and those detected via ultrasonography. Ten patients had transaminitis, defined as aspartate aminotransferase (AST) or alanine aminotransferase (ALT) levels >2 times the upper limit of normal (ULN). Nine patients had elevated alkaline phosphatase (ALP) or gamma-glutamyl transferase (GGT) of >2 times ULN. In three patients, transaminitis was due to anti-tubercular therapy (ATT)-induced hepatitis; in seven (5.2%), no specific cause for transaminitis could be identified, and hence they were classified as cases of lupus hepatitis. On comparing clinical features between patients with (n=7) and without lupus hepatitis (n=128), the condition was more prevalent in newly diagnosed SLE patients compared to those who had been on follow-up [six (85.7%) vs. 30 (23.6%), p=0.002]. All seven patients with lupus hepatitis had complete resolution of the transaminitis on follow-ups. However, one patient who had received ATT (isoniazid, rifampicin, ethambutol, and pyrazinamide) died. Ultrasonography showed fatty liver in seven patients and chronic liver disease in one patient. Conclusion In this study, transaminitis due to lupus hepatitis was seen in newly diagnosed lupus patients and was not associated with disease activity. Before diagnosing lupus hepatitis, drug-induced liver disease has to be ruled out, and if persistent LFT abnormalities are present, further workup is suggested to rule out overlap with primary biliary cirrhosis and/or autoimmune hepatitis.
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Affiliation(s)
- Shaik Imran
- Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
| | - Molly Mary Thabah
- Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
| | - Mohamed Azharudeen
- Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
| | - Ananthakrishnan Ramesh
- Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
| | - Zachariah Bobby
- Biochemistry, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
| | - Vir S Negi
- Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IND
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2
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Bailey BM, Ramos KS, Johnson A, Mitchell C. Acute Hepatic Failure and Epididymitis in a Hispanic Patient With Active Systemic Lupus Erythematosus. J Clin Med Res 2018; 10:722-724. [PMID: 30116443 PMCID: PMC6089580 DOI: 10.14740/jocmr3495w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2018] [Accepted: 06/18/2018] [Indexed: 12/04/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disease known to affect a variety of organ systems. Patients with SLE are more prone to developing common infections that can mimic the complications of SLE. As such, it is essential to differentiate complications of SLE from infection to ensure appropriate management and to improve morbidity and mortality of this patient population. Here we present a 24-year-old, Hispanic male, with SLE complicated by dialysis-dependent end-stage renal disease and dilated cardiomyopathy. The patient presented to the emergency room with nausea, vomiting, and abdominal pain and admitted to the medicine service. Initial evaluation showed hypoalbuminemia coupled with elevated transaminases, INR, and total bilirubin consistent with acute liver failure. Further evaluation was negative for viral, toxic, metabolic, or vascular causes of acute failure. The patient was diagnosed with lupus hepatitis and associated acute hepatic failure, and started on high dose prednisone (60 mg daily). Complete resolution of liver function and symptoms was observed within 1 week at follow-up. The patient was readmitted 2 weeks after discharge with left scrotal pain and swelling after abruptly decreasing the prescribed prednisone dose 3 days after discharge. Physical exam and scrotal ultrasound in the emergency department were consistent with epididymitis. Urinalysis, urine culture, and gonorrhea and chlamydia PCR were all negative. Without evidence of infection, and upon reconfirmation of low serum complement levels, the patient was diagnosed with lupus epididymitis and restarted on high dose prednisone. Complete resolution of symptoms was attained within 1 week at follow-up. This case emphasizes the importance of differentiating the clinical manifestations of SLE from infection and the complexity of disease presentation in Hispanic patients.
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Affiliation(s)
- Beau M Bailey
- Department of Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA
| | - Kenneth S Ramos
- Department of Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA.,Department of Medicine, University of Arizona, Tucson, AZ 85721-0202, USA
| | - Alice Johnson
- Department of Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA
| | - Charlene Mitchell
- Department of Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA
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3
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Zizzo AN, Erdman L, Feldman BM, Goldenberg A. Similarity Network Fusion: A Novel Application to Making Clinical Diagnoses. Rheum Dis Clin North Am 2018; 44:285-293. [PMID: 29622295 DOI: 10.1016/j.rdc.2018.01.005] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Similarity Network Fusion (SNF) is a novel methodological tool that integrates multiple different types of data to identify homogeneous subsets of patients in whom disease classification may be otherwise unclear or challenging. In this review article, the authors hope to provide insight into how SNF can be used in clinical decision making where the aim is to have little influence on the data prior to obtaining the results of the analysis.
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Affiliation(s)
- Andréanne N Zizzo
- Department of Pediatrics, Division of Gastroenterology and Hepatology, Western University, Children's Hospital, London Health Sciences Centre, 800 Commissioners Road East, B1-162, London, Ontario N6A 5W9, Canada.
| | - Lauren Erdman
- Genetics and Genome Biology, Department of Computer Science, The Hospital for Sick Children, Peter Gilgan Centre for Research and Learning (PGCRL), University of Toronto, 686 Bay Street, Toronto, Ontario M5G 0A4, Canada
| | - Brian M Feldman
- Department of Pediatrics, Division of Rheumatology, University of Toronto, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
| | - Anna Goldenberg
- Genetics and Genome Biology, Department of Computer Science, The Hospital for Sick Children, Peter Gilgan Centre for Research and Learning (PGCRL), University of Toronto, 686 Bay Street, Toronto, Ontario M5G 0A4, Canada
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4
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Autoimmune Hepatitis as an Initial Presentation of SLE. ARCHIVES OF PEDIATRIC INFECTIOUS DISEASES 2016. [DOI: 10.5812/pedinfect.34653] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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5
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Luijten RK, Fritsch-Stork RD, Bijlsma JW, Derksen RH. The use of glucocorticoids in Systemic Lupus Erythematosus. After 60years still more an art than science. Autoimmun Rev 2013; 12:617-28. [DOI: 10.1016/j.autrev.2012.12.001] [Citation(s) in RCA: 48] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2012] [Accepted: 12/02/2012] [Indexed: 01/18/2023]
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6
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Schiavon LL, Carvalho-Filho RJ, Narciso-Schiavon JL, Lanzoni VP, Ferraz MLG, Silva AEB. Late-onset systemic lupus erythematosus-associated liver disease. Rheumatol Int 2012; 32:2917-2920. [PMID: 20376663 DOI: 10.1007/s00296-010-1492-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2010] [Accepted: 03/27/2010] [Indexed: 01/12/2023]
Abstract
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease, which predominantly affects women under 50 years old. Although liver disease is not included in the diagnostic criteria, abnormal liver tests are common among patients with SLE and, in a significant proportion of those patients, no other underlying condition can be identified. We described a case of liver involvement in late-onset SLE presenting with a predominantly cholestatic pattern. Other conditions associated with abnormal liver tests were excluded, and the patient showed a prompt response to steroid therapy. The spectrum of the liver involvement in SLE is discussed, with emphasis on the differential diagnosis with autoimmune hepatitis.
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Affiliation(s)
- Leonardo L Schiavon
- Division of Gastroenterology, Hepatitis Section, Federal University of Sao Paulo, Sao Paulo, Brazil.
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7
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A patient of lupus presenting with myocarditis and overlapping autoimmune hepatitis. Case Rep Rheumatol 2011; 2011:402483. [PMID: 22937445 PMCID: PMC3420763 DOI: 10.1155/2011/402483] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2011] [Accepted: 09/27/2011] [Indexed: 12/17/2022] Open
Abstract
Systemic lupus erythematosus has myriad presentations. Symptomatic myocarditis and/or symptomatic autoimmune hepatitis associated with lupus are rare at presentation. Here we report a young Asian girl, who presented to us with features of symptomatic myocarditis and overlapping autoimmune hepatitis. She was eventually diagnosed to be harboring systemic lupus erythematosus, in whom rigorous management led to gratuitous recovery.
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8
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Shizuma T, Kuroda H. A case of primary biliary cirrhosis which developed eight years after diagnosis of systemic lupus erythematosus. Intern Med 2011; 50:321-4. [PMID: 21325764 DOI: 10.2169/internalmedicine.50.4357] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
A 29-year-old Japanese female was diagnosed with systemic lupus erythematosus (SLE) at the age of 21 and went into remission after administration of prednisolone. Although no liver dysfunction had been observed upon diagnosis of SLE or during follow-up, an increase of hepatobiliary enzyme levels was seen eight years after the diagnosis of SLE. Antimitochondrial antibodies were positive. Cell infiltration around intrahepatic bile ducts and granuloma formation were observed in the liver. Therefore, she was diagnosed with primary biliary cirrhosis (PBC). Administration of ursodeoxycholic acid resulted in normalization of hepatobiliary enzyme levels. Development of PBC after SLE is extremely rare.
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Affiliation(s)
- Toru Shizuma
- Department of Gastroenterology, International University of Health and Welfare Hospital, Japan.
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9
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Khalifa M, Benjazia E, Rezgui A, Ghannouchi N, Alaoua A, Braham A, Létaief A, Bahri F. [Lupus hepatitis: a case series of 12 patients]. Rev Med Interne 2010; 32:347-9. [PMID: 21129825 DOI: 10.1016/j.revmed.2010.10.357] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2010] [Revised: 09/25/2010] [Accepted: 10/25/2010] [Indexed: 01/12/2023]
Abstract
PURPOSE Several liver manifestations have been reported in systemic lupus erythematosus (SLE) and are usually non specific. We report on our experience of lupus hepatitis. METHODS A retrospective monocenter study of 73 patients with SLE. The diagnosis of lupus hepatitis was established after exclusion of other causes of hepatitis and hepatic vein thrombosis. RESULTS Liver involvement was noted in 12 patients (16.4%). There were nine female and three male patients; the mean age of these patients was 29 years. In seven patients liver involvement was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the five remaining patients. In all patients, liver manifestations were associated with other organ involvement. Clinical manifestations were: hepatomegaly (n=4), jaundice (n=4), abdominal pain (n=3), ascites (n=2), portal hypertension (n=1) and hepatic failure with encephalopathy (n=1). Elevated liver enzyme was noted in 11 cases and liver cholestasis in eight cases. Presence of anti-ribosomal P antibodies was noted in one case. Liver biopsy was performed in five patients, and revealed chronic active hepatitis in three cases, chronic hepatic granulomas in one case and nonspecific inflammation in one case. The outcome was favorable in 11 patients without relapse, and one patient died of encephalopathy and liver failure. CONCLUSION Liver involvement associated with SLE is not uncommon. It is frequently asymptomatic and limited to liver test abnormalities. The role of anti-ribosomal P autoantibodies remains uncertain.
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Affiliation(s)
- M Khalifa
- Service de médecine interne, CHU Farhat Hached, Sousse, Tunisia.
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10
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Zazzetti F, Buschiazzo A, Villamil FG, Barreira JC. Liver transplantation in systemic lupus erythematosus: case report and review of the literature. Lupus 2010; 20:90-4. [DOI: 10.1177/0961203310377219] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Severe liver involvement requiring liver transplantation is a rare complication in systemic lupus erythematosus (SLE), but very few cases have been reported. We describe a 39-year-old woman with SLE who underwent successful liver transplantation due to acute liver failure. The patient persisted without reactivation of SLE and with good long-term survival. Management and diagnosis considerations are reviewed.
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Affiliation(s)
- F Zazzetti
- Rheumatology Unit, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
| | - A Buschiazzo
- Rheumatology Unit, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
| | - FG Villamil
- Liver Transplantation Unit, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
| | - JC Barreira
- Rheumatology Unit, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
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11
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Usta Y, Gurakan F, Akcoren Z, Ozen S. An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood. World J Gastroenterol 2007; 13:2764-7. [PMID: 17569152 PMCID: PMC4147132 DOI: 10.3748/wjg.v13.i19.2764] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
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Affiliation(s)
- Yusuf Usta
- Hacettepe University, Faculty of Medicine, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, Ankara 06100, Turkey.
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12
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Kaw R, Gota C, Bennett A, Barnes D, Calabrese L. Lupus-related hepatitis: complication of lupus or autoimmune association? Case report and review of the literature. Dig Dis Sci 2006; 51:813-8. [PMID: 16615009 DOI: 10.1007/s10620-006-3212-1] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/25/2004] [Accepted: 06/03/2005] [Indexed: 12/13/2022]
Affiliation(s)
- Roop Kaw
- Section of General Internal Medicine, Department of General Internal Medicine, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
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13
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Horita T, Tsutsumi A, Takeda T, Yasuda S, Takeuchi R, Amasaki Y, Ichikawa K, Atsumi T, Koike T. Significance of magnetic resonance imaging in the diagnosis of nodular regenerative hyperplasia of the liver complicated with systemic lupus erythematosus: a case report and review of the literature. Lupus 2002; 11:193-6. [PMID: 11999886 DOI: 10.1191/0961203302lu164cr] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Nodular regenerative hyperplasia of the liver (NRH), characterized by multiple hepatic nodules in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus (SLE) associated with non-cirrhotic portal hypertension. The diagnosis of NRH is based on the pathological examination, and radiological findings of NRH are poorly documented. We report a case of a 40-year-old woman with SLE complicated with NRH. Sixteen years after diagnosis of SLE, esophageal varices were incidentally found and diagnosis of portal hypertension due to NRH was made by magnetic resonance imaging (MRI) and confirmed by needle liver biopsy. Although MRI showed the lesions as significant nodules, neither computed tomography nor ultrasonography could demonstrate the nodules. However, serial MRI showed significant enlargement of the nodules for 2 years Because NRH may lead to portal hypertension with life-threatening variceral haemorrhage in patients with SLE, MRI is a useful, non-invasive examination to screen the patients for its presence and follow-up. We reviewed the literature regarding NRH in SLE and discuss the management of the affected patients.
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Affiliation(s)
- T Horita
- Department of Medicine II, Hokkaido University School of Medicine, Sapporo, Japan.
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14
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Heyman SN, Spectre G, Aamar S, Rubinger D, Pappo O, Ackerman Z. Autoimmune cholangiopathy associated with systemic lupus erythematosus. LIVER 2002; 22:102-6. [PMID: 12028402 DOI: 10.1034/j.1600-0676.2002.01533.x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
We report a 39-year-old female who presented over 11 years with autoimmune cholangiopathy associated with kaleidoscopic manifestations of systemic lupus erythematosus (SLE), including, arthritis, skin changes, pleuritis, diffuse proliferative glomerulonephritis, lymphadenopathy, splenomegaly, hyperglobulinemia, and major depression. While antimitochondrial antibodies (AMA) were absent, antinuclear (ANA) and anti-DNA antibodies were detected in high titres associated with hypocomplementemia. The patient also had vitamin B12 deficiency and antiphospholipid antibodies. The patient required steroids and repeated courses of cyclophosphamide for the management of lupus nephritis, and ursodeoxycholic acid (ursolite) administration resulted in amelioration of cholestatic laboratory abnormalities. This unusual case report and review of literature illustrate that immune liver disease may be an important clinical manifestation of SLE, especially autoimmune cholangiopathy.
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MESH Headings
- Adult
- Anemia, Pernicious/etiology
- Antibodies, Antinuclear/analysis
- Antibodies, Antiphospholipid/analysis
- Antirheumatic Agents/therapeutic use
- Autoimmune Diseases/drug therapy
- Autoimmune Diseases/etiology
- Autoimmune Diseases/immunology
- Cyclophosphamide/therapeutic use
- Drug Therapy, Combination
- Female
- Glomerulonephritis, Membranoproliferative/etiology
- Humans
- Liver Cirrhosis, Biliary/drug therapy
- Liver Cirrhosis, Biliary/etiology
- Liver Cirrhosis, Biliary/immunology
- Lupus Erythematosus, Systemic/complications
- Lupus Erythematosus, Systemic/drug therapy
- Lupus Erythematosus, Systemic/immunology
- Treatment Outcome
- Ursodeoxycholic Acid/therapeutic use
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Affiliation(s)
- Samuel N Heyman
- Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, Israel.
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15
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Mukai M, Bohgaki T, Notoya A, Kohno M, Tateno M, Kobayashi S. Liver dysfunction due to apoptosis in a patient with systemic lupus erythematosus. Lupus 2000; 9:74-7. [PMID: 10713652 DOI: 10.1177/096120330000900115] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
We report on a 23-year-old Japanese female with a 13-year history of systemic lupus erythematosus (SLE), and two episodes of deterioration followed by treatment with high dose prednisolone. Although she had been recently treated with prednisolone (12.5 mg daily), her liver function became worse in July 1998. Results of a liver biopsy revealed multi-focal hepatic cell death in a severe fatty liver, without any inflammatory cell invasion. The biopsy also showed a positive TUNEL (Tdt-catalysed DNA nick end labelling) reaction indicating apoptosis. Her liver function recovered rapidly following steroid pulse therapy. Serum soluble Fas ligand (sFasL) was found to be elevated to a concentration of 0.395 ng/ml at the time of liver damage, but was less than 0.03 ng/ml before liver damage and after prednisolone treatment. The liver damage in this case appeared to be involved with apoptosis induced by sFasL. Although hepatitis associated with SLE is rare, apoptosis directly related to elevated sFasL levels might cause this complication.
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Affiliation(s)
- M Mukai
- Division of Clinical Immunology and Hematology, Department of Medicine,Sapporo City General Hospital, Chuo-ku, Sapporo, Japan.
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