A Rare Case of Kikuchi-Fujimoto Disease With Diffuse Lung
Involvement Presenting a Lymphatic-like Distribution on
Thin-section Computed Tomography
Ryoko Egashira, MD, PhD,* Takahiko Nakazono, MD, PhD,*
Ken Yamaguchi, MD, PhD,* Keita Kai, MD, PhD,†
Nobuyuki Ono, MD, PhD,‡ Mariko Yoshimura, MD, PhD,§
and Hiroyuki Irie, MD, PhD*
Key Words: diffuse lung disease, Kikuchi-Fujimoto disease,
histiocytic necrotizing lymphadenitis, computed tomography
(J Thorac Imaging 2018;00:000–000)
K
ikuchi-Fujimoto disease (KFD) is a rare condition
of unknown etiology that affects primarily young, female
patients. It is symptomatically characterized by cervical lym-
phadenopathy and fever and histologically by histiocytic necrot-
izing lymphadenitis. Pulmonary involvement is extremely rare.
CASE REPORT
A female patient in her 30s with a fever and finger stiffness was
referred to our hospital because of a dyspnea and cervical lym-
phadenopathies and pulmonary infiltrate on computed tomography
(CT). She previously had common cold-like symptoms, which were
followed by fever and cervical lymphadenopathies.
The patient had a 10-year history of similar episodes. Eight years
ago, she underwent a neck lymph-node biopsy that was inconclusive;
the lymphadenopathy then regressed spontaneously. One year ago, she
presented with a fever and finger stiffness, and was assessed for a
possible connective tissue disease. However, the symptoms once again
regressed spontaneously before a conclusive diagnosis was made. She
had no history of smoking, allergy, or inhalation. Her family history
indicated that her father had similar episodes in his 20s and even
5 years before her admission in our hospital.
Laboratory findings were as follows: decreased white blood cell
count, 1500/μL; increased C-reactive protein level, 3.91 mg/dL; lactate
dehydrogenase level, 750 U/L; polyclonal hypergammaglobulinemia;
and soluble interleukin-2 receptors, 865 U/mL. KL-6 level was normal,
and serum markers related to connective tissue disease were all neg-
ative. There was no evidence of an active viral infection.
On admission, the patient’s condition worsened. She had a
fever of 38.9°C and dyspnea on exertion without abnormal sounds
on auscultation.
A chest radiograph showed bilateral ground-glass opacity on
the middle to lower lung zone. Thin-section CT performed at the
previous hospital 7 days before referral showed bilateral, patchy,
and ill-defined ground-glass opacity along the blood vessels
(Figs. 1A, B). The lesions seemed nodular; however, they were
found to consist of small vessels with surrounding ground-glass
FIGURE 1. A and B, The initial thin-section CT shows bilateral, patchy, and ill-defined ground-glass opacity along the blood vessels, which make
the vessels more prominent and larger (arrows). The nodule-like lesions comprised the vessels and their surrounding ground-glass opacities.
From the Departments of *Radiology; †Pathology; ‡Rheumatology; and §Hematology, Respiratory Medicine and Oncology, Faculty of Medicine,
Saga University, Saga, Japan.
The authors declare no conflicts of interest.
Correspondence to: Ryoko Egashira, MD, PhD, Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501,
Japan (e-mail: egashira@cc.saga-u.ac.jp).
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
DOI: 10.1097/RTI.0000000000000353
JSTR MEETING NOTES
J Thorac Imaging
Volume 00, Number 00, ’’ 2018 www.thoracicimaging.com | 1
Copyright r 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
This paper can be cited using the date of access and the unique DOI number which can be found in the footnotes.