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Polyarteritis nodosa confined to the breasts associated with polyenthesitis Yoshiko Shimamura, Yoshinori Taniguchi, Kazu Hamada-Ode, Kosuke Inoue, Shimpei Fujimoto & Yoshio Terada To cite this article: Yoshiko Shimamura, Yoshinori Taniguchi, Kazu Hamada-Ode, Kosuke Inoue, Shimpei Fujimoto & Yoshio Terada (2015): Polyarteritis nodosa confined to the breasts associated with polyenthesitis, Modern Rheumatology To link to this article: http://dx.doi.org/10.3109/14397595.2015.1031720
Accepted author version posted online: 24 Mar 2015. Published online: 30 Apr 2015. Submit your article to this journal
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Date: 05 November 2015, At: 17:08
http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2015; Early Online: 1–2 © 2015 Japan College of Rheumatology DOI: 10.3109/14397595.2015.1031720
Polyarteritis nodosa confined to the breasts associated with polyenthesitis Yoshiko Shimamura, Yoshinori Taniguchi, Kazu Hamada-Ode, Kosuke Inoue, Shimpei Fujimoto, and Yoshio Terada Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, Nankoku, Japan
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Breast, Enthesitis, Polyarteritis nodosa History Received 17 February 2015 Accepted 17 March 2015 Published online 20 April 2015
Polyarteritis nodosa (PAN) was first described as an inflammatory arterial disorder by Kussmaul and Maier in 1866 . PAN was characterized as a necrotizing vasculitis of small- to-medium-sized arteries. It was subclassified into systemic, cutaneous/subcutaneous, and microscopic types. Cutaneous/subcutaneous PAN is generally recognized to have a good prognosis . PAN-associated vasculitis localized to the breast is very rare, with few reported cases [2–9]. Most of the cases were diagnosed as giant cell arteritis (GCA) [2–5], as PAN was considered uncommon [6–8]. We herein report a case of PAN of the breast associated with polyenthesitis. A 71-year-old Japanese woman presented with bilateral breast pain, myalgia, enthesitis of both patellar ligaments, fever, weight loss, and general fatigue. She was admitted to our hospital. Physical examination was significant for five tender breast nodules, approximately 1–2 cm in diameter, with associated erythema (Figure 1A). There were no other skin lesions. Laboratory tests demonstrated elevated levels of C-reactive protein (CRP) of 19.8 mg/dl, erythrocyte sedimentation rate (ESR) of 124 mm/1 h, and white blood cell count of 18.4 109/L. Antiproteinase 3, myeloperoxidase neutrophil cytoplasmic antibodies, and anti-nuclear antibodies were all negative. Hepatic and renal functions were normal. Human leukocyte antigen (HLA) typing demonstrated positive A2, A31, B46, and B61 antigens. Physical examination, mammography, and ultrasonography did not show any architectural distortions and calcifications which are considered as the sign of breast cancer. A biopsy of a nodule in the left breast revealed leukocytoclastic medium-sized arteritis without giant cell invasion (Figure 1B and C), indicating PAN. Breast nodule did not reveal typical septal and lobular panniculitis. Flurodeoxyglucose (FDG)-positron emission tomography/computed tomography showed high FDG uptake in multiple entheses, including spinous processes (Figure 1D), both ischial tuberosities, and patellar ligaments (Figure 1E), Correspondence to: Yoshinori Taniguchi, MD, Department of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University, Nankoku 783-8505, Japan. Tel: 81-88-880-2343. Fax: 81-88-880-2344. E-mail: [email protected]
indicating polyenthesitis. Ultrasonography of the left patellar ligament demonstrated a positive power Doppler signal in the tibial insertion, indicating tibial tuberosity enthesitis (Figure 1F). The patient was diagnosed with PAN limited to the breasts associated with polyenthesitis. Treatment with daily oral prednisolone (40 mg) was initiated, resulting in immediate improvement in the patient’s symptoms and normalization of the serum CRP and ESR values. She was discharged and had experienced no subsequent complications or flare-ups for 3 years. Arteritides of the breast are pathologically classified as GCA, granulomatosis with polyangiitis (GPA), and PAN [2–9]. About 20 cases of GCA of the breast have been reported [2–5]. GPA rarely affects only the breast . Mammary PAN is differentiated from mammary GCA and GPA by the characteristic pathology of each vasculitis. Clinical manifestations of PAN in the breast have been described as follows: (i) a breast nodule, with or without other cutaneous or systemic findings; and (ii) predominantly systemic PAN with involvement of the breast . In general, PAN localized to the breast has a good prognosis with a positive response to corticosteroids . In this case, oral prednisolone therapy produced a prompt response. Given that PAN is often diagnosed as a cause of fever of unknown origin (FUO), evaluation of the breasts may be helpful in the workup of FUO cases. This case featured high FDG uptake and a positive power Doppler signal in multiple entheses, leading us to diagnose HLA-B27-negative breast PAN associated with polyenthesitis. Simultaneously, enthesitis and PAN are rare; this is the first report of PAN of the breast associated with polyenthesitis. Only four previous cases of PAN associated with spondyloarthritis, including polyenthesitis, have been reported [10–13]. Three of these four patients had HLA-B27-positive polyenthesitis [10–12]. A strong correlation between polyenthesitis/spondyloarthritis and HLA-B27, B39, B7, B60, B61 has been reported [14,15]. Our case was HLA-B61-positive, which might suggest a pathologic mechanism of PAN presenting polyenthesitis. In patients with breast nodules accompanied by systemic symptoms including fever and enthesitis, PAN of the breast should be included in the differential diagnosis.
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Figure 1. (A) Five tender nodules (black arrowheads) with erythema in the breasts; (B) Biopsy specimen (hematoxylin– eosin stain) from one nodule in the left breast revealed leukocytoclastic medium-sized arteritis without giant cell invasion (original magnification 40); (C) Magnified image of biopsy specimen presented leukocytoclasis and vessel wall destruction (original magnification 200); (D) High FDG uptake in the enthesis of a spinous process (white arrowhead); (E) High FDG uptake in the enthesis of the patellar ligament (white arrowhead); (F) Positive power Doppler signal indicating tibial tubercle enthesitis.
Acknowledgements No funding was provided for this study.
Conflict of interest None.
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