Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient

Masanobu Horikoshi; Shigeko Inokuma; Erika Matsubara; Yasunori Honda; Rika Okada; Mika Kobuna; Yoko Miura; Shoko Kobayashi; Shigeo Kawai; Toshio Kumasaka

doi:10.2169/internalmedicine.54.5150

CASE REPORTS

Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient

Masanobu Horikoshi, Shigeko Inokuma, Erika Matsubara, Yasunori Honda, Rika Okada, Mika Kobuna, Yoko Miura, Shoko Kobayashi, Shigeo Kawai, Toshio Kumasaka

Author information

Masanobu Horikoshi
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Shigeko Inokuma
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Erika Matsubara
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Yasunori Honda
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Rika Okada
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Mika Kobuna
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Yoko Miura
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Shoko Kobayashi
Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan
Shigeo Kawai
Department of Pathology, Japanese Red Cross Medical Center, Japan
Toshio Kumasaka
Department of Pathology, Japanese Red Cross Medical Center, Japan

Keywords: catastrophic antiphospholipid syndrome, microangiopathic hemolytic anemia, relapse, rituximab

JOURNAL OPEN ACCESS

2015 Volume 54 Issue 22 Pages 2923-2927

DOI https://doi.org/10.2169/internalmedicine.54.5150

Details

Abstract

Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.

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