Multifocal osteonecrosis affecting all four limbs in systemic lupus erythematosus: A case report

Abstract

Osteonecrosis is a condition that frequently affects patients with systemic lupus erythematosus (SLE). However, reports on severe multifocal osteonecrosis involving more than three anatomical sites in patients with SLE are scarce. The present study describes a case of SLE with multifocal osteonecrosis involving all four limbs. A 22-year-old woman was diagnosed with SLE in 2010 and the disease was controlled by treatment with methylprednisolone and hydroxychloroquine. Approximately 1 year following the diagnosis of SLE, the patient developed pain in the elbows and hips. Magnetic resonance imaging revealed evidence of multifocal osteonecrosis in the bilateral distal segments of the humerus and femoral head, the distal segment of the right femur and the proximal segment of the right tibia. The patient was treated with right total hip arthroplasty and core decompression of the right femur and tibia, followed by continuation of the earlier medical treatment for SLE. The present case highlights the importance of conducting a careful investigation of patients with SLE who present with multiple joint involvement, and the choice of the appropriate treatment according to the presentation.

Bone Involvement in Systemic Lupus Erythematosus

“…Osteonecrosis or avascular necrosis (AVN) is a serious and well-recognized complication in SLE patients, which may cause disability and affect quality of life because of pain and mobility limitation [ 44 , 45 , 46 , 47 , 48 ].…”

“…It is defined as necrosis of the bone marrow and trabecular bones due to inadequate blood supply, and may affect any bone, even more than one at different times; the femoral head and knee are the most commonly affected sites [ 48 , 49 ].…”

“…Among rheumatic diseases, SLE is the most commonly associated with AVN [ 46 , 48 ]. AVN was first described as an SLE complication in the 1960s and has been frequently reported in these patients [ 47 , 48 ]: prevalence of symptomatic AVN is reported to be 0.8–33%, this difference being due to variability in diagnostic tools and study methodology, but it can have a clinically silent course.…”

“…Among rheumatic diseases, SLE is the most commonly associated with AVN [ 46 , 48 ]. AVN was first described as an SLE complication in the 1960s and has been frequently reported in these patients [ 47 , 48 ]: prevalence of symptomatic AVN is reported to be 0.8–33%, this difference being due to variability in diagnostic tools and study methodology, but it can have a clinically silent course. The prevalence of asymptomatic AVN is reported to be 29–45% [ 24 , 47 ], but its incidence has gradually decreased over the past two decades, probably owing to the improvement in SLE management and lowering use of GCs [ 44 , 47 ].…”

“…In SLE, AVN mainly affects weight-bearing joints and femoral head is the most commonly affected site; bilateral hip involvement has been reported in up to 90% of SLE patients with AVN [ 44 , 45 , 47 , 50 ]. Multifocal osteonecrosis is defined as involvement of at least three sites, and it is usually associated with GCs; it is an uncommon condition in SLE, as described in some case reports [ 48 , 51 ].…”