Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022
Systemic lupus erythematosus with various clinical manifestations in a patient with hereditary angioedema: a case report.
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Ushio Yusuke, Wakiya Risa, Kameda Tomohiro, Nakashima Shusaku, Shimada Hiromi, Mansour Mai Mahmoud Fahmy, Kato Mikiya, Miyagi Taichi, Sugihara Koichi, Mino Rina, Mizusaki Mao, Ibuki Emi, Kadowaki Norimitsu, Dobashi Hiroaki
Аннотация
BACKGROUND: Hereditary angioedema (HAE) is an inherited disease characterized by recurrent angioedema without urticaria or pruritus. The most common types of HAE are caused by deficiency or dysfunction in C1 esterase inhibitor (C1-INH-HAE). The association between C1-INH-HAE and systemic lupus erythematosus (SLE) is known; however, variations in the underlying pathophysiology, disease course, and treatment in this population remain incompletely understood.
CASE PRESENTATION: A 31-year-old Japanese woman with a prior diagnosis of HAE type 1 based on the episodes of recurrent angioedema, low C1 inhibitor antigen levels and function, and family history presented with new complaints of malar rash, alopecia, and arthralgias in her hands and elbows. She later developed fever, oral ulcers, lupus retinopathy, a discoid rash localized to her chest, and malar rash. Investigations revealed positive antinuclear antibody, leukopenia, thrombocytopenia, hypocomplementemia, and nephritis. Based on these findings, she was diagnosed with SLE according to the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria. There did not appear to be a correlation between HAE disease activity and the timing of presentation with SLE, because HAE disease activity had been stable. The patient was able to achieve and maintain remission with immunosuppressive therapy including prednisolone, hydroxychloroquine, and tacrolimus.
CONCLUSIONS: Our patient presented with a variety of symptoms, including fever and cytopenia in addition to mucocutaneous, joint, ocular, and renal lesions. It is important to better characterize the clinical characteristics of SLE in patients with C1-INH-HAE, and to clarify the mechanisms of SLE in this population.
CASE PRESENTATION: A 31-year-old Japanese woman with a prior diagnosis of HAE type 1 based on the episodes of recurrent angioedema, low C1 inhibitor antigen levels and function, and family history presented with new complaints of malar rash, alopecia, and arthralgias in her hands and elbows. She later developed fever, oral ulcers, lupus retinopathy, a discoid rash localized to her chest, and malar rash. Investigations revealed positive antinuclear antibody, leukopenia, thrombocytopenia, hypocomplementemia, and nephritis. Based on these findings, she was diagnosed with SLE according to the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria. There did not appear to be a correlation between HAE disease activity and the timing of presentation with SLE, because HAE disease activity had been stable. The patient was able to achieve and maintain remission with immunosuppressive therapy including prednisolone, hydroxychloroquine, and tacrolimus.
CONCLUSIONS: Our patient presented with a variety of symptoms, including fever and cytopenia in addition to mucocutaneous, joint, ocular, and renal lesions. It is important to better characterize the clinical characteristics of SLE in patients with C1-INH-HAE, and to clarify the mechanisms of SLE in this population.