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

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.