Objective: Wilson’s disease (WD) is an inherited disorder of copper metabolism with copper accumulation in different organs and clinical symptoms related to the affected organs (mainly hepatic and/or neuropsychiatric). D-penicillamine (DPA) is  a drug frequently used in WD therapy.

Background: DPA may cause adverse drug reactions (ADRs) which justify treatment discontinuation. Therefore DPA treatment should be monitored to detect ADRs as early as possible.

Method: We describe a case of patient with WD, treated with DPA for one year, who was diagnosed with drug induced lupus erythematosus (DIL) as a ADR of DPA treatment.

Results: Thirty two-years-old man was initially diagnosed with anxiety syndrome. After eight years the patient reported abdominal pain and the ultrasound examination of abdomen showed the liver cirrhosis. Based on clinical symptoms, abnormal copper metabolism, Kayser-Fleischer ring in ophthalmological examination and genetic test, the WD has been diagnosed. The treatment with DPA was introduced with increasing dose up to 1500mg/day. Twelve months later patient presented with fever, arthralgia, the pain was located in several joints (shoulders, hips, knees, feet) as well as in thoracic spine, leading to patient immobilization. In additional tests we recognized pleuritis with hydrothorax in chest X-ray, in blood analysis increased level of C-reactive protein 161mg/L(N:0-5) and erythrocyte sedimentation rate 40mm/h (N:0-10). In further blood tests  positive lupus anticoagulant 50.58s (N:30.5-40.6) and positive antinuclear antibodies (ANA:S 1:320) (anti-dsDNA; c-and p-ANCA; CCP antibodies negative) were found. Based on clinical picture and laboratory results the diagnosis of DPA-DIL were established. The WD treatment with DPA was stopped and zinc salts were introduced. The steroid treatment was started – methylprednisolone 16mg orally was introduced with gradual dose reduction. Within one week of treatment, most of the symptoms gradually diminished and disappear.

Conclusion: The knowledge of ADRs related to WD therapy is crucial for physicians and patients. DIL should be taken into account in WD patients treated with DPA, if arthralgia, fever, or renal injury occur.

References: [1] Członkowska A, Litwin T, Dusek P, et al. Wilson Disease. Nat Rev Dis Primers 2018; 4: 21. [2] European Association For The Study of The Liver Disease. EASL Clinical Practice Guidelines: Wilson’s Disease. J Hepatol 2012; 56: 671-85. [3] Członkowska A, Litwin T. Wilson disease – currently used anticopper therapy. Handb Clin Neurol 2017; 142: 181-191. [4] Antonov D, Kazandijeva J, Etugov D, et al. Drug-Induced Lupus Erythematosus. Clinics in Dermatology 2004; 22: 157-166. [5] Borchers A, Keen CL, Gershwin ME. Drug-Induced Lupus. Ann NY Acad Sci 2007; 1108: 166-182. [6] Pradhan S, Krishnamurthy S, Jagadisan B, et al. Wilson disease and lupus nephritis: is it coincidence or a true association? Pediatr Int Child Health 2019; 39: 146-149.

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