LSINCT5 predicts unfavorable prognosis and exerts oncogenic function in osteosarcoma.

The dysregulated expression of LSINCT5 (long stress-induced non-coding transcript 5) has been found in various human tumors, and was generally related to cancer progression and unfavorable prognosis. Although the role of LSINCT5 in osteosarcoma was reported not long ago, the sample size of that study was limited. Our study presented more evidence about the clinical significance and biological function of LSINCT5 in osteosarcoma. In our results, we found LSINCT5 expression was increased in osteosarcoma tissue samples and cell lines, and high LSINCT5 expression was associated with advanced Enneking stage, large tumor size, high histological grade and present distant metastasis. Meanwhile, we observed high LSINCT5 expression was correlated with worse overall survival, and high LSINCT5 expression could be an independent poor predictor for overall survival in osteosarcoma cases. Moreover, we found inhibition of LSINCT5 expression suppressed cell proliferation, migration and invasion in vitro, and LSINCT5 overexpression dramatically facilitated cell proliferation, migration and invasion in vitro In conclusion, our study suggests that LSINCT5 exerts oncogenic function in osteosarcoma cells, and may be a potential predictor for clinical outcome in osteosarcoma patients.

Skeletal Morbidity in Children and Adolescents during and following Cancer Therapy.

Skeletal abnormalities are common in children and adolescents diagnosed and treated for a malignancy. The spectrum ranges from mild pain to debilitating osteonecrosis and fractures. In this review, we summarize the impact of cancer therapy on the developing skeleton, provide an update on therapeutic strategies for prevention and treatment, and discuss the most recent advances in musculoskeletal research. Early recognition of skeletal abnormalities and strategies to optimize bone health are essential to prevent long-term skeletal sequelae and diminished quality of life in childhood cancer survivors.

An investigation of toxicities and survival in Hispanic children and adolescents with ALL: Results from the Dana-Farber Cancer Institute ALL Consortium protocol 05-001.

PURPOSE: This study compared the relative incidence of treatment-related toxicities and the event-free and overall survival between Hispanic and non-Hispanic children undergoing therapy for acute lymphoblastic leukemia (ALL) on Dana-Farber Cancer Institute ALL Consortium protocol 05-001. PATIENTS AND METHODS: Secondary analysis of prospectively collected data from a phase III multicenter study in children and adolescents of 1-18 years with previously untreated ALL. RESULTS: Between 2005 and 2011, 794 eligible patients enrolled on DFCI 05-001, 730 of whom were included in this analysis (19% [N = 150] Hispanic, 73% [N = 580] non-Hispanic). Hispanic patients were more likely to be ≥10 years of age (32% vs. 24%, P = 0.045) at diagnosis. Toxicity analyses revealed that Hispanic patients had significantly lower cumulative incidence of bone fracture (P < 0.001) and osteonecrosis (ON; P = 0.047). In multivariable risk regression, the risk of ON was significantly lower in Hispanic patients ≥10 years (HR 0.23; P = 0.006). Hispanic patients had significantly lower 5-year event-free survival (EFS) (79.4%; 95% CI: 71.6-85.2) and overall survival (OS) (89.2%; 95% CI: 82.7-93.4) than non-Hispanic patients (EFS: 87.5%; 95% CI: 84.5-90.0, P = 0.004; OS: 92.7%; 95% CI: 90.2-94.6, P = 0.006). Exploratory analyses revealed differences between Hispanic and non-Hispanic patients in the frequency of common variants in genes related to toxicity or ALL outcome. CONCLUSION: Hispanic children treated for ALL on DFCI 05-001 had fewer bone-related toxicities and inferior survival than non-Hispanic patients. While disease biology is one explanatory variable for outcome disparities, these findings suggest that biologic and non-biologic mechanisms affecting drug delivery and exposure in this population may be important contributing factors as well.

Prognostic significance of chemotherapy-induced necrosis in osteosarcoma patients receiving pasteurized autografts.

BACKGROUND: Among various reconstruction methods after wide excision for osteosarcoma, pasteurized autograft is often preferred. While the whole area of the tumor can be assessed for chemotherapy-induced necrosis, one of the important prognostic factors, in other reconstructive techniques, only a portion removed from a wide-resection specimen is available when using pasteurized autograft method. The assessment, therefore, may be unreliable. We analyzed the prognostic significance of the chemotherapy-induced necrosis in osteosarcoma patients who underwent reconstruction with pasteurized autografts. PATIENTS AND METHODS: We reviewed the records of osteosarcoma patients who underwent treatment in our institution from 1998 to 2013. Cases of reconstruction with pasteurized autografts were defined as the patient group, and the same number of patients who underwent other reconstruction methods served as controls. Chemotherapy-induced necrosis was evaluated for removed extra-osseous and curetted intramedullary tumor tissues. RESULTS: A total of 22 patients were identified; the median age was 15.5 years, and there were 12 males. The most common tumor location was the distal femur. The most common histological subtype was osteoblastic. Median size was 8.1 cm. Disease status was stage IIB in 13 patients and IIA in 9. Median follow-up was 76 months. No differences between the patient and control groups were observed in potential prognostic factors, overall survival, metastasis-free survival, or recurrence-free survival. Univariate analyses demonstrated that histological response was a significant prognostic factor for metastasis-free survival and also significant for recurrence-free survival. CONCLUSION: Chemotherapy-induced necrosis grading, using only available tumor tissues, could be a prognostic factor for osteosarcoma patients receiving pasteurized autografts for reconstructive surgery.

An underlying diagnosis of osteonecrosis of bone is associated with worse outcomes than osteoarthritis after total hip arthroplasty.

BACKGROUND: Well-designed studies of complications and readmission rates in patients undergoing total hip arthroplasty (THA) with osteonecrosis are lacking. Our objective was to examine if a diagnosis of osteonecrosis was associated with complications, mortality and readmission rates after THA. METHODS: We analyzed prospectively collected data from an integrated healthcare system's Total Joint Replacement Registry of adults with osteonecrosis vs. osteoarthritis (OA) undergoing unilateral primary THA during 2001-2012, in an observational cohort study. We examined mortality (90-day), revision (ever), deep (1 year) and superficial (30-day) surgical site infection (SSI), venous thromboembolism (VTE, 90-day), and unplanned readmission (90-day). Age, gender, race, body mass index, American Society of Anesthesiologists class, and diabetes were evaluated as confounders. We used logistic or Cox regression to calculate odds or hazard ratios (OR, HR) with 95% confidence intervals (CI). RESULTS: Of the 47,523 primary THA cases, 45,252 (95.2%) had OA, and 2,271 (4.8%) had osteonecrosis. Compared to the OA, patients with osteonecrosis were younger (median age 55 vs. 67 years), and were less likely to be female (42.5% vs. 58.3%) or White (59.8% vs. 77.4%). Compared to the OA, the osteonecrosis cohort had higher crude incidence of 90-day mortality (0.7% vs. 0.3%), SSI (1.2% vs. 0.8%), unplanned readmission (9.6% vs. 5.2%) and revision (3.1% vs. 2.4%). After multivariable-adjustment, patients with osteonecrosis had a higher odds/hazard of mortality (OR: 2.48; 95% CI:1.31-4.72), SSI (OR: 1.67, 95%CI:1.11-2.51), unplanned 90-day readmissions (OR: 2.20; 95% CI:1.67-2.91) and a trend towards higher revision rate 1-year post-THA (HR: 1.32; 95% CI: 0.94-1.84), than OA patients. CONCLUSIONS: Compared to OA, a diagnosis of osteonecrosis was associated with worse outcomes post-THA. A detailed preoperative discussion including the risk of complications is needed for informed consent from patients with osteonecrosis.

Revision surgery occurs frequently after percutaneous fixation of stable femoral neck fractures in elderly patients.

BACKGROUND: Femoral neck fractures are a major public health problem. Multiple-screw fixation is the most commonly used surgical technique for the treatment of stable femoral neck fractures. QUESTIONS/PURPOSES: We determined (1) the proportion of hips that had conversion surgery to THA, and (2) the proportion of hips that underwent repeat fracture surgery after percutaneous screw fixation of stable (Garden Stages I and II) femoral neck fractures in patients older than 65 years and the causes of these reoperations. METHODS: We performed a retrospective study of all patients older than 65 years with stable femoral neck fractures secondary to low-energy trauma treated surgically at our institution between 2005 and 2008. We identified 121 fractures in 120 patients older than 65 years as stable (Garden Stage I or II); all were treated with percutaneous, cannulated screw fixation in an inverted triangle without performing a capsulotomy or aspiration of the fracture hematoma at the time of surgery. The average age of the patients at the time of fracture was 80 years (range, 65-100 years). Radiographs, operative reports, and medical records were reviewed. Fracture union, nonunion, osteonecrosis, intraarticular hardware, loss of fixation, and conversion to arthroplasty were noted. Followup averaged 11 months (range, 0-5 years) because all patients were included, including those who died. The mortality rate was 40% for all patients at the time of review. RESULTS: Twelve patients (10%) underwent conversion surgery to THA at a mean of 9 months after the index fracture repair (range, 2-24 months); the indications for conversion to THA included osteonecrosis, nonunion, and loss of fixation. Two others had periimplant subtrochanteric femur fractures treated by surgical repair with cephalomedullary nails and two patients had removal of hardware. CONCLUSIONS: Revision surgery after osteosynthesis for stable femoral neck fractures was more frequent in this series than previously has been reported. The reasons for this higher frequency of reoperation may be related to poor bone quality, patient age, and some technical factors, which leads us to believe other treatment options such as nonoperative management or hemiarthroplasty may be viable options for some of these patients. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Recombinant human bone morphogenetic protein-2 in debridement and impacted bone graft for the treatment of femoral head osteonecrosis.

The purpose of this study was to compare the clinical outcomes of impacted bone graft with or without recombinant human bone morphogenetic protein-2 (rhBMP-2) for osteonecrosis of the femoral head (ONFH). We examined the effect of bone-grafting through a window at the femoral head-neck junction, known as the "light bulb" approach, for the treatment of ONFH with a combination of artificial bone (Novobone) mixed with or without rhBMP-2. A total of 42 patients (72 hips) were followed-up from 5 to 7.67 years (average of 6.1 years). The patients with and without BMP were the first group (IBG+rhBMP-2) and the second group (IBG), respectively. The clinical effectiveness was evaluated by Harris hip score (HHS). The radiographic follow-up was evaluated by pre-and postoperative X-ray and CT scan. Excellent, good, and fair functions were obtained in 36, 12, and 7 hips, respectively. The survival rate was 81.8% and 71.8% in the first and second group, respectively. However, the survival rate was 90.3% in ARCO stage IIb, c, and only 34.6% in ARCO stage IIIa (P<0.05). It was concluded that good and excellent mid-term follow-up could be achieved in selected patients with ONFH treated with impacted bone graft operation. The rhBMP-2 might improve the clinical efficacy and quality of bone repair.

Mortality after shoulder arthroplasty.

One year post-operative mortality among patients with primary elective total shoulder arthroplasty (ETSA) and traumatic shoulder arthroplasty (TSA) were compared to the general population of a large healthcare system. Standardized mortality ratios (SMRs) and 95% confidence intervals (CIs) were calculated. 614 ETSA patients, 1.0% one year mortality, and 168 TSA patients, 5.4% mortality rate, were evaluated. Patients with ETSA (SMR = 0.4, 95% CI 0.1-0.7) had lower odds of mortality than expected, while patients with TSA (SMR = 1.8, 95% CI 0.6-3.0) did not have higher than expected odds of mortality compared to the reference population. Understanding excess mortality following shoulder arthroplasty surgery allows providers to evaluate current practices and identify ways to optimize patients prior to surgery.

A retrospective study evaluating frequency and risk factors of osteonecrosis of the jaw in 576 cancer patients receiving intravenous bisphosphonates.

OBJECTIVE: To evaluate the frequency, risk factors, and clinical presentation of bisphosphonate (BP)-related osteonecrosis of the jaw (BRONJ). STUDY DESIGN: We performed a retrospective analysis of 576 patients with cancer treated with intravenous pamidronate and/or zoledronate between January, 2003 and December, 2007 at the University of Minnesota Masonic Cancer Center and Park Nicollet Institute. RESULTS: Eighteen of 576 identified patients (3.1%) developed BRONJ including 8 of 190 patients (4.2%) with breast cancer, 6 of 83 patients (7.2%) with multiple myeloma, 2 of 84 patients (2.4%) with prostate cancer, 1 of 76 patients (1.3%) with lung cancer, 1 of 52 patients (1.9%) with renal cell carcinoma, and in none of the 73 patients with other malignancies. Ten patients (59%) developed BRONJ after tooth extraction, whereas 7 (41%) developed it spontaneously (missing data for 1 patient). The mean number of BP infusions (38.1 ± 19.06 infusions vs. 10.5 ± 12.81 infusions; P<0.001) and duration of BP treatment (44.3 ± 24.34 mo vs. 14.6 ± 18.09 mo; P<0.001) were significantly higher in patients with BRONJ compared with patients without BRONJ. Multivariate Cox proportional hazards regression analysis showed that diabetes [hazard ratio (HR)=3.40; 95% confidence interval (CI), 1.14-10.11; P=0.028], hypothyroidism (HR=3.59; 95% CI, 1.31-9.83; P=0.013), smoking (HR=3.44; 95% CI, 1.28-9.26; P=0.015), and higher number of zoledronate infusions (HR=1.07; 95% CI, 1.03-1.11; P=0.001) significantly increased the risk of developing BRONJ. CONCLUSIONS: Increased cumulative doses and long-term BP treatment are the most important risk factors for BRONJ development. Type of BP, diabetes, hypothyroidism, smoking, and prior dental extractions may play a role in BRONJ development.

Two escalated followed by six standard BEACOPP in advanced-stage high-risk classical Hodgkin lymphoma: high cure rates but increased risk of aseptic osteonecrosis.

BACKGROUND: From 1999, Norwegian guidelines recommend two escalated (esc) BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone) followed by six standard (s) BEACOPP for patients with advanced-stage classical Hodgkin lymphoma (HL) with an international prognostic score (IPS) ≥ 4. We evaluated retrospectively the experience with this recommendation at the Norwegian Radium Hospital, also including all IPS 3 patients treated with the same regimen. PATIENTS AND METHODS: Forty-seven patients were treated between June 1999 and December 2008. IPS was 3 in 10 patients and ≥ 4 in 37. RESULTS: Thirty-five patients received eight cycles of BEACOPP, 12 patients received one to six cycles only, mainly due to toxicity. Sixty percent of patients had dose reductions. With median follow-up of survivors of 89 months, 5-year progression-free and overall survival are 84% [95% confidence interval (CI) 73% to 95%] and 91% (95% CI 82% to 100%), respectively. Toxicity was considerable with grade 3 or more infections/febrile neutropenia in 66% of patients, including one death and three cases of Pneumocystis jiroveci pneumonia. Of note, 10 patients (21%) experienced symptomatic aseptic osteonecrosis, of whom 3 have had hip replacement surgery after treatment. CONCLUSION: Two escBEACOPP plus six sBEACOPP is efficacious in advanced-stage high-risk HL. We document a high incidence of aseptic bone necrosis, possibly related to prednisolone.

Proximal femoral allograft: prognostic indicators.

Between 1972 and 1999, the Orthopedic Oncology Service treated 150 patients with resection and allograft transplantation of the proximal femur. Of the group, 121 patients had malignant tumors of the proximal femur and 29 had benign disorders. Four types of allografts were used: osteoarticular (46 patients), allograft-prosthesis (73), intercalary (20), and allograft-arthrodesis (5). Only 16% of the patients died of disease and 3% required amputation. The overall success rate for the series was 77% with the best results for the allograft prosthetic (82%) and intercalary procedures (87%). Graft infection (15 patients), allograft fracture (26 patients), and local recurrence (11 patients) most markedly affected outcome. With the exception of deaths of disease, no significant outcome difference occurred between the patients with malignant and benign disorders. In conclusion, allograft implantation especially for aggressive or malignant tumors of the proximal femur appears to be a competent system for therapy.

Unicompartmental knee arthroplasty for avascular osteonecrosis.

UNLABELLED: The data analyzing clinical and radiological outcomes after modern unicompartmental knee arthroplasty (UKA) for spontaneous and secondary avascular osteonecrosis are limited. We determined whether: (1) UKA for osteonecrosis was as reliable for alleviating pain and improving function (measured by Knee Society scores) as it is for osteoarthritis, (2) lower limb alignment could be restored after UKA for osteonecrosis, and (3) UKA for osteonecrosis is as durable as UKA for osteoarthritis (measured by survivorship at 12 years). We retrospectively reviewed 30 patients (31 knees) with osteonecrosis; 21 knees had spontaneous osteonecrosis and 10 had secondary osteonecrosis. Mean patient age was 71 years. Clinical and radiological evaluations were performed by an independent observer at a minimum followup of 3 years (mean, 7 years; range, 3-16 years). Reliable pain relief and function improvement were obtained in 30 knees (96%). Restoration of an appropriate lower-limb mechanical axis was achieved for 27 knees (88%). The Kaplan-Meier survivorship was 96.7% at 12 years. Our data suggest UKA is a reasonable solution for restoring clinical function and radiological lower-limb alignment for spontaneous or secondary osteonecrosis limited to one compartment of the knee, with a durable survivorship. LEVEL OF EVIDENCE: Level IV, retrospective study. See the Guidelines for Authors for a complete description of levels of evidence.

Osteonecrosis after allogeneic stem cell transplantation in childhood. A case-control study in Italy.

A case-control study was conducted among Italian children treated with a stem cell transplant (SCT). Cases (n = 43) were allogeneic recipients with osteonecrosis, and the controls (n = 129) were matched to the corresponding cases on the basis of survival, SCT center and date of transplant. Univariate analysis showed that older age at SCT (OR 1.39; 95% CI 1.24-1.57), total body irradiation (TBI) (OR 5.73; 95% CI 2.38-13.83), chronic graft-versus-host disease (GvHD) (OR 4.31; 95% CI 2.05-9.07), and duration of steroid treatment after SCT (OR 1.118; 95% CI 1.034-1.209) were statistically correlated with osteonecrosis. However, multivariate analysis revealed that the only factors that were significantly associated with osteonecrosis were older age at SCT (p = 0.0001), TBI (p = 0.001) and chronicGvHD (p = 0.001).

Osteonecrosis of the jaw associated with pamidronate therapy.

Bisphosphonates are commonly used in the treatment and prevention of osteoporosis, and they are also an important therapeutic adjunct in multiple myeloma and other cancers metastatic to bone. Bisphosphonates are generally well tolerated and associated with minimal adverse effects; however, there exists a growing concern that intravenous bisphosphonate use is associated with osteonecrosis of the jaw (ONJ). We report the occurrence of osteonecrosis of the jaw associated with pamidronate therapy in 12 patients diagnosed with multiple myeloma, breast carcinoma, or renal cell carcinoma, all involving bone. At the onset of jaw osteonecrosis, pamidronate therapy was the single medication common to all 12 patients. The duration of therapy varied from 12 to 77 months before osteonecrosis was observed; 92% (11/12) of cases involved the posterior mandible and all cases have been refractory to a variety of medical therapies, including surgical debridement and systemic antibiotics. Available tissue biopsies revealed inflammation consistent with osteomyelitis. In one biopsy, Actinomyces spp. were recovered from culture, but treatment with an extended course of clindamycin conferred no clinical benefit. The persistence of exposed bone remains a significant source of morbidity and pain for each surviving patient. Discontinuation of pamidronate therapy has not helped reverse the presence of osteonecrosis, and surgical manipulation of the involved site appears to worsen the underlying bone pathology. ONJ is an important adverse outcome associated with bisphosphonate therapy, and physicians prescribing pamidronate or zoledronate must be aware of the association between these drugs and this serious clinical entity. Failure to recognize the signs of ONJ can lead to unnecessary surgical procedures, which ultimately exacerbate the condition and impact quality of life. The unremitting nature of this clinical development, and the long-lasting morbidity associated with it suggests that patients should be counseled regarding the possible occurrence of ONJ prior to initiating therapy with pamidronate.

Adjusted mean Systemic Lupus Erythematosus Disease Activity Index-2K is a predictor of outcome in SLE.

OBJECTIVE: To test the predictability of the adjusted mean Systemic Lupus Erythematosus Disease Activity Index-2K (AMS) for main outcomes in systemic lupus erythematosus (SLE), namely presence of damage, coronary artery disease (CAD), and avascular necrosis (AVN). METHODS: Included in this study are patients with regular followup from the University of Toronto Lupus Clinic. This was defined as a minimum of 3 visits and no absence exceeding 18 consecutive months. For each visit, AMS was evaluated. The ability of the AMS to predict each of the main outcomes was evaluated through time-dependent covariate survival analysis. Adjustments to the regression models were made to include other risk factors such as sex, age at diagnosis (AGE), SLEDAI-2K at presentation (SLEDAI), disease duration (DD), and use of corticosteroids, immunosuppressives (IM), or antimalarials (AM). RESULTS: Five hundred and seventy-five patients were included covering the period from 1970 to 2002. A total of 325 developed damage, 55 had CAD, and 68 had AVN. Presence of damage was not associated with sex, SLEDAI, or AM but was significantly associated with AMS, AGE, DD, and use of steroids or IM (all p < 0.001). CAD was not associated with SLEDAI or use of steroids or AM but with all other variables AMS (p = 0.046), sex (p = 0.009), AGE (p < 0.0001), DD (p < 0.0001), and IM (p = 0.035). Predictors of AVN were DD (p = 0.032) and IM (p < 0.0001) but not sex, AGE, use of steroids, AM, SLEDAI, or AMS. CONCLUSION: AMS is associated with the presence of damage and CAD. It is not associated with AVN.

Avascular necrosis in long-term survivors after allogeneic or autologous stem cell transplantation: a single center experience and a review.

BACKGROUND: The most debilitating skeletal complication of stem cell transplantation (SCT) is avascular necrosis (AVN). METHODS: Two hundred seven consecutive patients were evaluated prospectively for AVN. They survived disease free for more than 180 days after autologous or allogeneic SCT for hematologic malignancies. The diagnosis of AVN in suspicious cases was confirmed by magnetic resonance imaging. Possible correlations with treatments, bone mineral density (BMD), graft versus host disease (GVHD), and in vitro growth of fibroblast progenitors were investigated. Bone mineral density was evaluated by dual-energy X-ray absorptiometry in 100 transplanted patients, and the in vitro growth of fibroblast progenitors was monitored by a fibroblast colony-forming unit (CFU-F) assay in 30 patients after allogeneic SCT. RESULTS: Twelve patients developed AVN 3-114 months (median, 26 months) following SCT: 10 (10%) after allogeneic SCT and 2 (1.9%) after autologous SCT (P = 0.04). Twenty-five joints were affected by AVN. All patients had femoral head involvement, which was managed with hip replacement in six of them. All but one patient who developed AVN after allogeneic SCT suffered from chronic GVHD (cGVHD). Avascular necrosis occurred 1-4 months after exacerbation or progression of cGVHD. Cumulative dose of steroids was similar in both SCT groups (including steroids given pretransplant for the basic disease), whereas treatment duration was significantly longer in the allogeneic SCT group. Avascular necrosis was related to the decreased number of bone marrow CFU-F colonies in vitro, but not to BMD values. CONCLUSIONS: Avascular necrosis is a skeletal complication that occurs more often after allogeneic than after autologous SCT. Occurrence of AVN symptoms after clinical follow-up of cGVHD suggests that cGVHD requiring long-term steroid therapy is one of the main risk factors for AVN. Avascular necrosis may be facilitated by a severe deficit in the repopulating capacity of bone marrow stromal stem cells after SCT.

Ten to fourteen year survival and functional analysis of the AGC total knee replacement system.

A study of 562 Anatomic Graduated Component (AGC) total knee arthroplasties that were performed in 402 patients between November 1986 and September 1990 is reported. All patients had implantation with a cemented posterior cruciate-retaining design, with resurfacing of the patella using all polyethylene patella components. Mean age at surgery was 71 years (range 41-92 years). Patients were followed for a minimum of 10 years (range 10-14 years). Nine knees were lost to follow-up (1.4%). The mean Knee Society Score for pain and function were analyzed by Charnley categories: Category A -- 97 (pain) and 89 (function); Category B -- 91 (pain) and 84 (function); and Category C -- 89 (pain) and 62 (function). The survival analysis at 14 years was 97% with revision for any reason as the endpoint and the authors continue to utilize this implant system.

Outcomes of symptomatic osteonecrosis in 95 patients with systemic lupus erythematosus.

OBJECTIVE: To describe the frequency and type of symptomatic osteonecrosis (ON) in a large cohort of patients with systemic lupus erythematosus (SLE) followed in a single center and to describe the outcome in terms of mortality and disability compared to SLE patients without ON. METHODS: Patients with ON were identified from the University of Toronto Lupus Clinic Database. The diagnosis of ON was confirmed by radiographs, bone scans, tomograms, or magnetic resonance images. A comparison group of patients with SLE without ON was selected from the same database, matched by year of birth, sex, and year of entry to the clinic. Mortality, disability, and health related quality of life were compared between patients with and without ON. RESULTS: Ninety-nine patients with ON were identified with 217 affected joints, the majority hips and knees, often in a bilateral distribution. There was no increase in mortality. Patients with ON had higher Health Assessment Questionnaire scores and lower SF-20 scores of physical functioning, suggesting increased disability. Hip joints that underwent surgery were more likely to have higher grades of ON at diagnosis. CONCLUSION: Symptomatic ON occurred in 12.8% of 744 patients with SLE and often involved multiple joints. ON was not associated with increased mortality but was associated with physical disability. Radiological class of the hip jointsat diagnosis of ON was predictive of subsequent surgery.