An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

Usta, Yusuf; Gürakan, Figen; Akçören, Zühal; Özen, Seza

doi:10.3748/wjg.v13.i19.2764

Cited by 32 publications

(28 citation statements)

References 30 publications

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“…Co-occurrence of SLE and AIH (SLE-AIH) is rare, and the latest reports involve isolated cases or a small series of patients, which makes it difficult to establish definitive clinical or laboratory features, treatment responses, and prognoses (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). Thus, the aims of the present study were to identify distinct features of SLE-AIH compared with primary AIH (P-AIH) and to examine factors involved in treatment responses or prognosis of SLE-AIH.…”

Section: Introductionmentioning

confidence: 99%

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Lim

Lee

Ahn

et al. 2016

Arthritis Care & Research

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Objective. Autoimmune hepatitis (AIH) is a chronic progressive liver disease characterized by circulating autoantibodies and hyperglobulinemia. This study was conducted to identify the features of AIH accompanied by systemic lupus erythematosus (SLE-AIH) that differ from those of primary AIH (P-AIH), and to evaluate factors that affect the outcome for SLE-AIH patients. Methods. From May 1995 to April 2014, clinical data (including liver pathology) from 164 patients with P-AIH and 23 patients with SLE-AIH were collected from an electronic database at a tertiary referral center. AIH was diagnosed according to the diagnostic scoring system for AIH (revised in 1999). SLE patients fulfilled at least 4 of the 1997 revised American College of Rheumatology criteria. Progression was defined as occurrence of cirrhosis, hepatocellular carcinoma, liver transplantation, or death from hepatic failure. Results. The age at the time of AIH diagnosis was lower and initial levels of serum IgG were higher in SLE-AIH than in P-AIH patients. Progression was more common in P-AIH, and hepatocellular carcinoma, liver transplantation, or death occurred only in P-AIH patients. Among 23 patients with SLE-AIH, 8 with cirrhosis had higher levels of serum IgG than 15 patients without cirrhosis (mean 6 SD 4,077.4 6 1,641.0 mg/dl and 2,560.7 6 932.2 mg/dl, respectively; P 5 0.017). Moreover, a serum IgG level more than 2-fold the upper normal limit was associated with a high risk of cirrhosis in SLE-AIH (odds ratio 11.00 [95% confidence interval 1.420-85.201]; P 5 0.026). Conclusion. Initially high levels of serum IgG are a poor prognostic factor for SLE-AIH. Additionally, the long-term outcome for SLE-AIH might be better than for P-AIH.

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Section: Introductionmentioning

confidence: 99%

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Lim

Lee

Ahn

et al. 2016

Arthritis Care & Research

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“…Systemic lupus erythematosus (SLE) belongs to a family of related autoimmune rheumatic disorders, including dermatomyositis, scleroderma, polymyositis, mixed connective tissue disease, and rheumatoid arthritis. All of these disorders are capable of affecting multiple organs, and the symptoms in systemic rheumatic diseases often overlap as has been repeatedly reported in the literature [1][2][3][4][5][6][7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus with Severe Nephritis That Mimicked Henoch-Schoenlein Purpura

Al-Attrach

Al-Shibli²,

Al-Riyami³

et al. 2011

Arab J. Nephr. Trans

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Introduction: Systemic lupus erythematosus (SLE) belongs to a family of related autoimmune rheumatic disorders that are capable of affecting multiple organs, and they are all associated with a variety of autoantibodies. Henoch Schoenlein purpura (HSP) is a sort of systemic vasculitis that is not associated with auto-antibodies and can affect different organs including the kidneys.Case report: A 12 year-old girl presented with abdominal pain, low grade fever, swollen and tender feet and left hand, skin rash on the lower extremities, and high blood pressure. Initial laboratory tests revealed severe proteinuria, microscopic hematuria and low C3 level. Renal biopsy showed diffuse proliferative glomerulonephritis with IgA, fibrinogen and C3 deposits. The case was accordingly diagnosed as HSP with severe IgA nephropathy. Treatment was started with mycophenolate mofetil (MMF) and pulse methylprednisolone followed by prednisolone. The patient improved and treatment was discontinued after 5.5 months. One month after withdrawal of her medications, the patient presented again with serositis and recurrent proteinuria. Both antinuclear antibodies (ANA) and anti dsDNA were positive. At this point she was diagnosed to have SLE disease and immunosuppressive treatment was restarted. Following this, symptoms disappeared, proteinuria regressed and anti-dsDNA titer dropped. Conclusion:This case presented with features of HSP and was later-on diagnosed to have SLE. This kind of clinical overlapping has not been reported in the literature to the best of our knowledge.

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“…Her first clinical and laboratory presentations were suggestive of AIH, while over the following 3 months, obvious clinical and serologic manifestations of SLE emerged, such as photosensitivity, malar rash, arthritis, and oral ulcers, as well as positive ANA and anti-dsDNA (24). By continuing her past treatment and adding new drugs, we saw rapid clinical improvement in her arthritis, malaise, and general condition, as well as her hepatitis (25,26).…”

Section: Pathological Analysismentioning

confidence: 99%

Autoimmune Hepatitis as an Initial Presentation of SLE

Yeganeh

Zafari

Sardarinia

et al. 2016

Arch Pediatr Infect Dis

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An 11-year-old female patient with autoimmune hepatitis (AIH) was referred to our rheumatology clinic due to her current musculoskeletal manifestations. The patient had been diagnosed with AIH 3 months previously, based on jaundice and impaired liver function tests, and she had been treated with low-dose prednisolone and azathioprine. She presented with malaise, arthritis, a malar rash on the face, and oral ulcers. Laboratory tests revealed a positive ANA/anti-dsDNA test. Liver biopsy showed chronic hepatitis with severe inflammatory activity, in favor of a diagnosis of definite AIH. She fulfilled the international criteria for both SLE and AIH. The clinical symptoms and laboratory findings of SLE improved with ongoing treatment with corticosteroids and azathioprine, accompanied with hydroxychloroquine sulfate. The present case indicates that AIH can be the first manifestation of SLE in children.

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An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

Cited by 32 publications

References 30 publications

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Systemic Lupus Erythematosus with Severe Nephritis That Mimicked Henoch-Schoenlein Purpura

Autoimmune Hepatitis as an Initial Presentation of SLE

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