The First Case of Multifocal Osteonecrosis in Behcet’s Disease
Background: Osteonecrosis is the death of bone tissue due to the interruption of the blood supply. It can occur in autoimmune disease due to multiple mechanisms. It occurs rarely in some kind of multisystem disease such as Behcet’s disease (BD). The aim of this article is to present a case of BD with multifocal osteonecrosis.
Case Presentation: A 26-year-old woman with oral aphthosis, panuveitis, and retinal vasculitis was diagnosed to have BD, and oral treatment of prednisolone (0.5 mg/kg/day) together with azathioprine (2 mg/kg/day) and intravenous (IV) cyclophosphamide (CYC) (0.75 g/m2) was administered for her. Prednisolone was tapered to 15 mg/day after 3 months. Because of unresponsiveness of eye involvement to traditional immunosuppressive therapy, infliximab (5 mg/kg) was started for her, and IV CYC was discontinued. 4 months after initial therapy, she complained of her right shoulder pain. Regarding shoulder pain, magnetic resonance imaging (MRI) showed osteonecrosis of the right shoulder. After the diagnosis of osteonecrosis, prednisolone was discontinued by herself. 3 months later, she complained of both knees, both hips, and right ankle pain. According to the previous diagnosis of osteonecrosis of her right shoulder, MRI was done for all involved joints, which revealed osteonecrosis of all of them. After the diagnosis of multifocal osteonecrosis, the following tests were done that all results were normal: anticardiolipin antibody (ACA) [immunoglobulin G (IgG), immunoglobulin M (IgM)], lupus anticoagulant (LA), anti-beta-2 glycoprotein 1 (antiB2GP1) (IgG, IgM), coagulation tests, and lipid profiles.
Conclusions: Bone infarction should be considered in the differential diagnosis of patients with joint pain and without the evidence of arthritis; however, it rarely occurs in patients with BD.
2. Kurosawa M, Inaba Y, Nishibu A. Nationwide epidemiological survey of Beheet's disease in 2003 in Japanese. Clin Exp Rheumatol. 2004;22(4):S84.
3. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, et al. Behcet's disease in Iran: Analysis of 6500 cases. Int J Rheum Dis. 2010;13(4):367-73. doi: 10.1111/j.1756-185X.2010.01549.x. [PubMed: 21199472].
4. Feng Y, Yang SH, Xiao BJ, Xu WH, Ye SN, Xia T, et al. Decreased in the number and function of circulation endothelial progenitor cells in patients with avascular necrosis of the femoral head. Bone. 2010;46(1):32-40. doi: 10.1016/j.bone.2009.09.001. [PubMed: 19747991].
5. Kenzora JE, Glimcher MJ. Accumulative cell stress: The multifactorial etiology of idiopathic osteonecrosis. Orthop Clin North Am. 1985;16(4):69-79. [PubMed: 3903604].
6. Jager M, Thorey F, Wild A, Voede M, Krauspe R. Osteonecrosis of Behcet's disease: Diagnosis, therapy, and course. Z Rheumatol. 2003;62(4):390-4. doi: 10.1007/s00393-003-0473-3. [PubMed: 12928943].
7. Chang HK, Choi YJ, Baek SK, Lee DH, Won KS. Osteonecrosis and bone infarction in association with Behcet's disease: Report of two cases. Clin Exp Rheumatol. 2001;19(5 Suppl 24):S51-S54. [PubMed: 11760400].
8. Ronco P, Wechsler B, Saillant G, Godeau P. Aseptic osteonecrosis during corticosteroid treatment of Behcet's disease (author's transl). Nouv Presse Med. 1981;10(21):1707-10. [PubMed: 7232160]. [In French].
9. Oktayoglu P, Cevik F, Tahtasiz M, Em S, Bozkurt M, Kapukaya A, et al. Bilateral knee pain associated with bone infarction in a patient with behcet's disease. Case Rep Rheumatol. 2012; 2012:539310. doi: 10.1155/2012/539310. [PubMed: 23198245]. [PubMed Central: PMC3502802].
10. Duygulu F, Evereklioglu C, Calis M, Borlu M, Cekmen M, Ascioglu O. Synovial nitric oxide concentrations are increased and correlated with serum levels in patients with active Behcet's disease: A pilot study. Clin Rheumatol. 2005;24(4):324-30. doi: 10.1007/s10067-004-1015-3. [PubMed: 15902528].
11. Seyahi E, Yazici H. To anticoagulate or not to anticoagulate vascular thrombosis in Behcet's syndrome: an enduring question. Clin Exp Rheumatol. 2016;34(1 Suppl 95):S3-S4. [PubMed: 26967193].
12. Leiba M, Seligsohn U, Sidi Y, Harats D, Sela BA, Griffin JH, et al. Thrombophilic factors are not the leading cause of thrombosis in Behcet's disease. Ann Rheum Dis. 2004;63(11):1445-9. doi:
10.1136/ard.2003.014241. [PubMed: 15479893]. [PubMed Central: PMC1754810].
13. Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and ultrasonographic evaluation of lower-extremity
vein thrombosis in behcet syndrome: An observational study.
Medicine (Baltimore). 2015;94(44):e1899. doi: 10.1097/MD.0000000000001899. [PubMed: 26554787]. [PubMed Central: PMC4915888].
14. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, et al. How to deal with Behcet's disease in daily practice. Int J Rheum Dis. 2010;13(2):105-16. doi: 10.1111/j.1756-185X.2010.01462.x. [PubMed: 20536594].
15. Weinstein RS. Glucocorticoid-induced osteonecrosis. Endocrine. 2012;41(2):183-90. doi: 10.1007/s12020-011-9580-0. [PubMed: 22169965]. [PubMed Central: PMC3712793].
16. Akbarian M, Faezi ST, Gharibdoost F, Shahram F, Nadji A, Jamshidi AR, et al. Systemic lupus erythematosus in Iran: A study of 2280 patients over 33 years. Int J Rheum Dis. 2010;13(4):374-9. doi: 10.1111/j.1756-185X.2010.01547.x. [PubMed: 21199473].
17. Jones JP Jr. Intravascular coagulation and osteonecrosis. Clin Orthop Relat Res. 1992;(277):41-53. [PubMed: 1532547].
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