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Co-occurring Wilson’s disease and non-penicillamine-induced systematic lupus erythematosus: a case report and literature review

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Abstract

Although lupus induced by penicillamine, the first-line medication for Wilson’s disease, is well-documented, primary systematic lupus erythematosus (SLE) co-occurring with Wilson’s disease has only rarely been reported. Symptom overlap can add to difficulties in making the correct and complete diagnosis of these two systemic diseases. An 18-year-old female was diagnosed with simultaneous Wilson’s disease and SLE and was successfully treated with hydroxychloroquine and oral zinc. We also reviewed the literature for cases of Wilson’s disease co-occurring with SLE not induced by penicillamine and found six other cases. Clinical presentations, diagnoses, treatments, and outcomes were analyzed and summarized to expand our understanding of this rare condition. The most frequent diagnostic clues to Wilson’s disease in patients with SLE included unexplained liver damage despite well-controlled SLE, extrapyramidal symptoms and signs, hyper-intense signals of the basal ganglia bilaterally on T2-weighted and fluid-attenuated inversion recovery (FLAIR) MRI images, and Kayser-Fleischer (K-F) rings on physical examination. Penicillamine should be avoided or used cautiously in Wilson’s disease patients complicated by SLE. The overall prognosis is good if treated in a timely manner.

Key Points

SLE complicated by Wilson’s disease or the co-occurrence of the two conditions in the absence of penicillamine may exist in rare conditions.

The diagnostic clues for identifying Wilson’s disease in SLE patients may include unexplained liver damage despite well-controlled SLE, extrapyramidal symptoms and signs, and K-F rings found by physical examination.

Penicillamine should be avoided or used cautiously in Wilson’s patients with SLE.

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References

  1. Pfeiffer RF (2016) Wilson disease. Continuum (Minneap Minn) 22(4 Movement Disorders):1246–1261. https://doi.org/10.1212/con.0000000000000350

    Article  Google Scholar 

  2. Xie JJ, Wu ZY (2017) Wilson’s disease in China. Neurosci Bull 33(3):323–330. https://doi.org/10.1007/s12264-017-0107-4

    Article  PubMed  PubMed Central  Google Scholar 

  3. Czlonkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, Rybakowski JK, Weiss KH, Schilsky ML (2018) Wilson disease. Nat Rev Dis Primers 4(1):21. https://doi.org/10.1038/s41572-018-0018-3

    Article  PubMed  PubMed Central  Google Scholar 

  4. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML (2007) Wilson’s disease. Lancet 369(9559):397–408. https://doi.org/10.1016/S0140-6736(07)60196-2

    Article  CAS  PubMed  Google Scholar 

  5. Dorner T, Furie R (2019) Novel paradigms in systemic lupus erythematosus. Lancet 393(10188):2344–2358. https://doi.org/10.1016/S0140-6736(19)30546-X

    Article  PubMed  Google Scholar 

  6. Ishak R, Abbas O (2013) Penicillamine revisited: historic overview and review of the clinical uses and cutaneous adverse effects. Am J Clin Dermatol 14(3):223–233. https://doi.org/10.1007/s40257-013-0022-z

    Article  PubMed  Google Scholar 

  7. Dell’era L, Boati E, Nebbia G, Corona F (2012) Wilson’s disease treated with penicillamine and lupus erythematosus: related or distinct entities? Minerva Pediatr 64(1):55–57

    PubMed  Google Scholar 

  8. Kim TY, Lee SH, Kim TJ, Cho KR, Cho SC, Han SH, Yang SY, Chung YW, Sohn JH, Baik SH, Bae SC, Lee DH, Kee CS (2002) A case of fulminant hepatic failure in Wilson’s disease combined with systemic lupus erythematosus. Taehan Kan Hakhoe Chi 8(1):100–104

    PubMed  Google Scholar 

  9. Mageed SA, Rawla P, Mahmoud MA, Shahba A (2019) Systemic lupus erythematosus, hypoparathyroidism, and hemolytic anemia in a patient with Wilson’s disease. Reumatologia 57(4):239–242. https://doi.org/10.5114/reum.2019.87622

    Article  PubMed  PubMed Central  Google Scholar 

  10. Pradhan S, Krishnamurthy S, Jagadisan B, Rajesh NG, Kaliaperumal S, Ramasamy S, Subramanian N (2019) Wilson disease and lupus nephritis: is it coincidence or a true association? Paediatr Int Child Health 39(2):146–149. https://doi.org/10.1080/20469047.2018.1443411

    Article  PubMed  Google Scholar 

  11. Santhakumar R, Gayathri K, Ramalingam PK, Manjunath BV, Karuppusamy N, Vetriveeran B, Selvamani S, Vishnuram P, Muruganathan A, Natarajan K (2016) Wilson’s disease with systemic lupus erythematosus. J Assoc Physicians India 64(4):81–82

    PubMed  Google Scholar 

  12. Zhang Y, Wang D, Wei W, Zeng X (2018) Wilson’s disease combined with systemic lupus erythematosus: a case report and literature review. BMC Neurol 18(1):85. https://doi.org/10.1186/s12883-018-1085-5

    Article  PubMed  PubMed Central  Google Scholar 

  13. Poujois A, Woimant F (2019) Challenges in the diagnosis of Wilson disease. Ann Transl Med 7(Suppl 2):S67. https://doi.org/10.21037/atm.2019.02.10

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Hedera P (2019) Wilson’s disease: a master of disguise. Parkinsonism Relat Disord 59:140–145. https://doi.org/10.1016/j.parkreldis.2019.02.016

    Article  PubMed  Google Scholar 

  15. Adiga A, Nugent K (2017) Lupus hepatitis and autoimmune hepatitis (lupoid hepatitis). Am J Med Sci 353(4):329–335. https://doi.org/10.1016/j.amjms.2016.10.014

    Article  PubMed  Google Scholar 

  16. Machado A, Chien HF, Deguti MM, Cançado E, Azevedo RS, Scaff M, Barbosa ER (2006) Neurological manifestations in Wilson’s disease: report of 119 cases. Mov Disord 21(12):2192–2196. https://doi.org/10.1002/mds.21170

    Article  PubMed  Google Scholar 

  17. Czlonkowska A, Litwin T, Dziezyc K, Karlinski M, Bring J, Bjartmar C (2018) Characteristics of a newly diagnosed Polish cohort of patients with neurological manifestations of Wilson disease evaluated with the Unified Wilson’s Disease Rating Scale. BMC Neurol 18(1):34. https://doi.org/10.1186/s12883-018-1039-y

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Hermann W (2014) Morphological and functional imaging in neurological and non-neurological Wilson’s patients. Ann N Y Acad Sci 1315:24–29. https://doi.org/10.1111/nyas.12343

    Article  CAS  PubMed  Google Scholar 

  19. European Association for Study of L (2012) EASL clinical practice guidelines: Wilson’s disease. J Hepatol 56(3):671–685. https://doi.org/10.1016/j.jhep.2011.11.007

    Article  Google Scholar 

  20. Durand F, Bernuau J, Giostra E, Mentha G, Shouval D, Degott C, Benhamou JP, Valla D (2001) Wilson’s disease with severe hepatic insufficiency: beneficial effects of early administration of D-penicillamine. Gut 48(6):849–852. https://doi.org/10.1136/gut.48.6.849

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Kiss JE, Berman D, Van Thiel D (1998) Effective removal of copper by plasma exchange in fulminant Wilson’s disease. Transfusion 38(4):327–331. https://doi.org/10.1046/j.1537-2995.1998.38498257369.x

    Article  CAS  PubMed  Google Scholar 

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Funding

Medical Education Quality Project 2019 by Peking Union Medical College (2019zlgc0104) provided the financial support for polishing the language in the article and publication.

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Contributions

YJ conceived this study. WS, XH, and SZ collected clinical data and took care of the patient. WS and SZ drafted the manuscript. XH and YJ critically revised the manuscript. All authors have revised the final version of the manuscript and approved it for publication.

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Correspondence to Yang Jiao.

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Our study has been granted an exemption from review by the Institutional Review Board of Peking Union Medical College Hospital.

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Shi, W., Huang, X., Zhang, S. et al. Co-occurring Wilson’s disease and non-penicillamine-induced systematic lupus erythematosus: a case report and literature review. Clin Rheumatol 40, 2485–2490 (2021). https://doi.org/10.1007/s10067-020-05463-z

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