A Rare Case of Acute Fibrinous and Organizing Pneumonia Associated with Systemic Lupus Erythematosus and Autoimmune-associated Hemophagocytic Syndrome: The Involvement of CD163-positive Macrophages

Mariko Taira; Takeshi Matsumura; Yoshiko Sumita; Mayuko Moriyama; Masahiro Kondo; Noriyoshi Ishikawa; Yasuko Wada; Mamiko Nagase; Emiko Nishikawa; Yukari Tsubata; Koji Kishimoto; Yohko Murakawa

doi:10.2169/internalmedicine.7184-21

This article has now been updated. Please use the final version.

A Rare Case of Acute Fibrinous and Organizing Pneumonia Associated with Systemic Lupus Erythematosus and Autoimmune-associated Hemophagocytic Syndrome: The Involvement of CD163-positive Macrophages

Mariko Taira, Takeshi Matsumura, Yoshiko Sumita, Mayuko Moriyama, Masahiro Kondo, Noriyoshi Ishikawa, Yasuko Wada, Mamiko Nagase, Emiko Nishikawa, Yukari Tsubata, Koji Kishimoto, Yohko Murakawa

Author information

Mariko Taira
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
Takeshi Matsumura
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
Yoshiko Sumita
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
Mayuko Moriyama
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
Masahiro Kondo
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
Noriyoshi Ishikawa
Department of Surgical Pathology, Shimane University Faculty of Medicine, Japan
Yasuko Wada
Internal Medicine III, Shimane University Faculty of Medicine, Japan
Mamiko Nagase
Organ Pathology, Shimane University Faculty of Medicine, Japan
Emiko Nishikawa
Respiratory Medicine & Medical Oncology, Shimane University Faculty of Medicine, Japan
Yukari Tsubata
Respiratory Medicine & Medical Oncology, Shimane University Faculty of Medicine, Japan
Koji Kishimoto
Thoracic Surgery, Shimane University Faculty of Medicine, Japan
Yohko Murakawa
Department of Rheumatology, Shimane University Faculty of Medicine, Japan
General Treatment Center for Intractable Diseases, Shimane University Faculty of Medicine, Japan

Keywords: acute fibrinous and organizing pneumonia, autoimmune-associated hemophagocytic syndrome, systemic lupus erythematosus, rheumatic disease, CD163 positive macrophages, intravenous cyclophosphamide

JOURNAL OPEN ACCESS Advance online publication

Article ID: 7184-21

DOI https://doi.org/10.2169/internalmedicine.7184-21

The final version of this article is now available: Vol. 61 (2022), No. 4 pp. 559-565

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Abstract

Acute fibrinous and organizing pneumonia (AFOP) is rare in patients with systemic lupus erythematosus (SLE). We herein report a case of AFOP with SLE and hemophagocytic syndrome. Early-phase high-resolution computed tomography showed a fine granular lung pattern. A pathological examination revealed AFOP. An immunohistological examination revealed numerous CD163+ and fewer CD68+ macrophages present in the lung tissue and in alveolar spaces as well, including fibrin balls, the interstitium, and bronchial walls. Pneumonia and thrombocytopenia worsened during high-dose steroid therapy, plasma exchange, and intravenous immunoglobulin administration. The addition of intravenous cyclophosphamide successfully ameliorated the symptoms and radiographic lesions. Therefore, this therapy may be useful for treating severe AFOP.

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