Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancyt...

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Bibliographic Details
Published in:Internal Medicine 2018/09/15, Vol.57(18), pp.2747-2752
Main Authors: Toko, Hirofumi, Tsuboi, Hiroto, Umeda, Naoto, Honda, Fumika, Ohyama, Ayako, Takahashi, Hidenori, Abe, Saori, Yokosawa, Masahiro, Asashima, Hiromitsu, Hagiwara, Shinya, Hirota, Tomoya, Kondo, Yuya, Matsumoto, Isao, Sumida, Takayuki
Format: Article
Language:English
Subjects:
acute lupus hemophagocytic syndrome
Bone marrow
Case Report
Corticoids
Corticosteroids
Cyclophosphamide
cyclosporine A
Cyclosporins
Fever
Hemophagocytic syndrome
Internal medicine
Intravenous administration
Lupus
Methylprednisolone
Pancytopenia
Remission
Systemic lupus erythematosus
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Summary:Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond. However, the addition of cyclosporine A (CsA) led to a rapid remission from ALHS. This suggests the usefulness of CsA in the treatment of intractable, corticosteroid- and IVCY-resistant ALHS.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.0571-17