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Published in: BMC Neurology 1/2018

Open Access 01-12-2018 | Case report

Wilson’s disease combined with systemic lupus erythematosus: a case report and literature review

Authors: Yun Zhang, Dongmei Wang, Wei Wei, Xuejun Zeng

Published in: BMC Neurology | Issue 1/2018

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Abstract

Background

Wilson’s disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation, particularly in the liver and brain. Systemic lupus erythematosus (SLE) is a multi-system disorder that can manifest in any system. Cases with concomitant WD and SLE, unrelated to treatment with penicillamine, have been rarely reported.

Case presentation

We report a case of a young woman who had typical neuropsychiatric symptoms and laboratory tests results of WD. She also had concomitant massive hematuria and proteinuria, fever, multiple positive autoimmune antibodies, hypocomplementemia, abnormal lumbar puncture findings and evidence of Sjögren syndrome, which are all rare in WD. Hence, we considered the diagnosis of SLE. Tapering of steroid dosage also confirmed the diagnosis.

Conclusion

Wilson’s disease and SLE have varied clinical manifestations. Herein, we reported a rare case in which the two conditions concomitantly existed. In clinical practice, differential diagnosis of the two diseases is necessary for patients with hepatic, neurological, and psychiatric manifestations.
Literature
1.
go back to reference Roberts EA, Schilsky ML. A practice guideline on Wilson disease. Hepatology. 2003;38(2):1475–92.CrossRef Roberts EA, Schilsky ML. A practice guideline on Wilson disease. Hepatology. 2003;38(2):1475–92.CrossRef
3.
go back to reference Thomas GR, Forbes JR, Roberts EA, et al. The Wilson disease gene: spectrum of mutations and their consequences. Nat Genet. 1995;9(2):210–7.CrossRefPubMed Thomas GR, Forbes JR, Roberts EA, et al. The Wilson disease gene: spectrum of mutations and their consequences. Nat Genet. 1995;9(2):210–7.CrossRefPubMed
4.
go back to reference Milkiewicz P, Saksena S, Hubscher SG, et al. Wilson’s disease with superimposed autoimmune features: report of two cases and review. J Gastroenterol Hepatol. 2000;15(5):570–4.CrossRefPubMed Milkiewicz P, Saksena S, Hubscher SG, et al. Wilson’s disease with superimposed autoimmune features: report of two cases and review. J Gastroenterol Hepatol. 2000;15(5):570–4.CrossRefPubMed
5.
go back to reference Guha SK, Modak D, Basu A, et al. Coomb’s positive autoimmune Haemolytic Anaemia in a case of Wilson disease: co-incidence or association? JIACM. 2009;10(1 & 2):78–80. Guha SK, Modak D, Basu A, et al. Coomb’s positive autoimmune Haemolytic Anaemia in a case of Wilson disease: co-incidence or association? JIACM. 2009;10(1 & 2):78–80.
6.
go back to reference Walshe JM. Penicillamine and the SLE syndrome. J Rheumatol Suppl. 1981;7:155.PubMed Walshe JM. Penicillamine and the SLE syndrome. J Rheumatol Suppl. 1981;7:155.PubMed
7.
go back to reference Dell'Era L, Boati E, Nebbia G, et al. Wilson’s disease treated with penicillamine and lupus erythematosus: related or distinct entities? Minerva Pediatr. 2012;64(1):55.PubMed Dell'Era L, Boati E, Nebbia G, et al. Wilson’s disease treated with penicillamine and lupus erythematosus: related or distinct entities? Minerva Pediatr. 2012;64(1):55.PubMed
8.
go back to reference Kim TY, Lee SH, Kim TJ, et al. A case of fulminant hepatic failure in Wilson's disease combined with systemic lupus erythematosus. Taehan Kan Hakhoe Chi. 2002;8(1):100–4.PubMed Kim TY, Lee SH, Kim TJ, et al. A case of fulminant hepatic failure in Wilson's disease combined with systemic lupus erythematosus. Taehan Kan Hakhoe Chi. 2002;8(1):100–4.PubMed
9.
go back to reference Santhakumar R, Gayathri K, Ramalingam PK, et al. Wilson’s disease with systemic lupus erythematosus. J Assoc Physicians India. 2016;64(4):81.PubMed Santhakumar R, Gayathri K, Ramalingam PK, et al. Wilson’s disease with systemic lupus erythematosus. J Assoc Physicians India. 2016;64(4):81.PubMed
10.
go back to reference Stassi G, Di FV, Todaro M, et al. Involvement of Fas/FasL system in the pathogenesis of autoimmune diseases and Wilson’s disease. Arch Immunol Ther Exp. 1999;47(3):129. Stassi G, Di FV, Todaro M, et al. Involvement of Fas/FasL system in the pathogenesis of autoimmune diseases and Wilson’s disease. Arch Immunol Ther Exp. 1999;47(3):129.
11.
go back to reference Ranucci G, Dato FD, Tufano M, et al. Long term follow-up of Wilson disease patients diagnosed in childhood: reasons for treatment changes. Dig Liver Dis. 2013;45(5444):e273.CrossRef Ranucci G, Dato FD, Tufano M, et al. Long term follow-up of Wilson disease patients diagnosed in childhood: reasons for treatment changes. Dig Liver Dis. 2013;45(5444):e273.CrossRef
Metadata
Title
Wilson’s disease combined with systemic lupus erythematosus: a case report and literature review
Authors
Yun Zhang
Dongmei Wang
Wei Wei
Xuejun Zeng
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2018
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-018-1085-5

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