Bilateral avascular necrosis of both hips and both shoulders in a case with systemic lupus erythematosus: What should we blame,steroid or secondary antiphospholipid syndrome?

A 38‐year‐old female patient was diagnosed as a case of systemic lupus erythematosus (SLE) in 1994. Her initial presentation was nephritis which remitted on combination of steroid, azathioprine and pulse cyclophosphamide therapy. One year later the patient developed bilateral avascular necrosis (AVN) both hips and underwent bilateral hip replacement. In 2003 the patient developed bilateral AVN of both shoulders. In view of this uncommon presentation the patient screened for hidden secondary antiphospholipid syndrome and surprisingly investigations revealed negative anticardiolipin antibodies, weakly positive lupus anticoagulant test and positive reactivity against β2 glycoprotein 1. Although steroid is well know for its major role in AVN in patients with SLE, the presence of hidden secondary antiphospholipid syndrome augments the deleterious effects of steroid on bone and leads to AVN in uncommon sites. It is suggested that in SLE patients with positive lupus anticoagulant and negative antiphospholipid antibodies, testing for reactivity against β2 glycoprotein 1 is mandatory.     

Risk factors for avascular necrosis of bone in patients with systemic lupus erythematosus: is there a role for antiphospholipid antibodies?

BACKGROUND: Avascular necrosis of bone (AVN) is a well known complication in patients with systemic lupus erythematosus (SLE). OBJECTIVE: To investigate the role of antiphospholipid antibody status (IgM and IgG anticardiolipin antibodies and lupus anticoagulant) with adjustment for corticosteroid use as risk factors for the development of AVN. METHODS: A cohort of 265 patients receiving long term follow up in our SLE clinic from 1978 to 1998 was analysed. Patients with AVN complications were detected and then matched for age, sex, ethnicity, duration of disease, and organ disease with two other patients with SLE. A further 31 patients were chosen at random for the analysis. RESULTS: Eleven patients had AVN, giving a point prevalence of 4%. There were no significant differences demonstrable in the presence of individual antiphospholipid antibodies (aPL) or their combination between the group with AVN or the two control groups. CONCLUSION: Incorporating an adjustment for corticosteroid use we were unable to show a link between the presence of aPL and the development of AVN in patients with SLE.     

Risk factors for avascular bone necrosis in systemic lupus erythematosus

OBJECTIVE: To study the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). METHOD: The records of 38 SLE patients who developed clinically apparent AVN during the course of their disease were reviewed. Information on clinical presentation, corticosteroid usage and autoantibody profiles was obtained, and comparison was made between these patients and 143 consecutive control SLE patients who did not have AVN. RESULTS: The point prevalence of AVN in our SLE population was 12%. Patients with AVN, when compared with controls, had a significantly higher incidence of neurological disease (39% vs 14%; P < 0.001) and Cushingoid body habitus after steroid treatment (79% vs 53%; P = 0.004). The highest cumulative prednisolone dose in 1 and 4 months was significantly higher in the AVN group than the controls (1.8 vs 1.1 and 4.5 vs 2.8 g, respectively; P < 0.01 in both) and showed a linear trend with the incidence of AVN (chi2 test for trend, P < 0.01 in both). Lupus anticoagulant was associated with AVN (P = 0.02, odds ratio 2.88 [1.14- 7.28]). Logistic regression analysis revealed that the highest cumulative prednisolone dose administered in 4 months, the maximum and mean daily prednisolone dosage, and the lupus anticoagulant were independent risk factors for AVN. CONCLUSIONS: Corticosteroid remains the major predisposing factor for AVN in SLE. Patients who require an initial high-dose steroid for disease control are at risk of AVN, especially if they are positive for the lupus anticoagulant or develop Cushingoid habitus after steroid treatment. High-risk patients should be closely monitored so that early AVN can be diagnosed by sensitive techniques such as magnetic resonance imaging and radioisotope bone scanning.      

Asymptomatic avascular necrosis in patients with primary antiphospholipid syndrome in the absence of corticosteroid use: a prospective study by magnetic resonance imaging

OBJECTIVE: To evaluate the prevalence of avascular necrosis (AVN), using magnetic resonance imaging (MRI), in patients with primary antiphospholipid syndrome (APS) and in patients with systemic lupus erythematosus (SLE), with or without anticardiolipin antibodies (aCL), who are asymptomatic for AVN and have not taken corticosteroids. METHODS: Seventy-nine subjects who were asymptomatic for AVN were evaluated by MRI of the femoral heads: 30 patients with primary APS who had never received corticosteroids, 19 SLE patients who had never received steroids (divided into 2 groups, aCL positive and aCL negative, in order to examine any association between AVN and aCL), and 30 healthy subjects who were age- and sex-matched with patients with primary APS. Established MRI criteria were used for a diagnosis of AVN. RESULTS: Asymptomatic AVN was evident in 6 (20%) of 30 patients with primary APS: 3 of them (1 man, 2 women) had intermediate bilateral AVN, and 3 (all women) had early AVN (bilateral in 1 patient). Results of hip and pelvis radiography and dynamic scintigraphy were negative. Followup MRI 6 months later revealed no changes. At the time of the initial MRI examination, the mean (+/-SD) age of patients in whom AVN was identified was 31.2 +/- 7.3 years, and that of patients without AVN was 42.4 +/- 11.9 years (P = 0.036). Livedo reticularis occurred significantly more commonly in the group with AVN (P = 0.041). None of the healthy subjects and none of the patients with SLE demonstrated AVN on MRI. CONCLUSION: AVN can be detected by MRI in 20% of patients with primary APS. Younger patients tend to develop AVN more frequently than do older patients, and the presence of livedo reticularis may identify individuals at risk for AVN. Clinicians should be aware of this possible clinical manifestation of primary APS, because early diagnosis can lead to early intervention.     

Risk factors for avascular bone necrosis in patients with systemic lupus erythematosus

The objective was to investigate the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). The records of 868 patients with SLE from four centers were reviewed retrospectively. Forty-nine patients with AVN were identified. A total of 154 patients with SLE who did not have clinically apparent AVN during the follow-up were evaluated as a control group. The demographic, clinical, laboratory and management characteristics of these two groups of patients were recorded according to predefined protocol and compared. The prevalence of AVN was detected 6% in our SLE population. The highest dose corticosteroid administered within 4?months and total cumulative prednisolone dose were significantly higher in the SLE patients with AVN. The use of cytotoxic agent significantly higher proportion of patients with AVN. AVN tends to develop more frequently in male gender and younger patients. Oral ulcer, pleuritis, Raynaud??s phenomenon, cutaneous vasculitis, lymphadenopathy, autoimmune thyroiditis, peripheral neuropathy and Sj?gren??s syndrome were higher incidence in SLE patients with AVN. The bilateral femoral heads were the commonest site of involvement of AVN in our patients with SLE.     

Multiple avascular necrosis of bone and polyarticular septic arthritis in patients with systemic lupus erythematosus

Objective Avascular necrosis of bone (AVN) and osteoarticular infection share similar risk factors in systemic lupus erythematosus (SLE) patients. However, their coincidental development in SLE has rarely been described. We describe four cases of AVN complicated by Staphylococcus aureus infection in SLE.Methods Patients were identified by retrospectively reviewing an SLE cohort followed between 1979 and 2003. A review of the literature from 1960 until 2003 was also done.Results Among 315 SLE patients, four developed joint infection by S. aureus following or coincidentally with AVN. All presented multifocal disease with severe or relapsing course, resulting in severe incapacity. The clinical course suggests that AVN developed first in active SLE patients with positive antiphospholipid (aPL) antibodies treated with high-dose corticosteroids (CS), and subsequent bone infarcts favor infection. Our patients often required prolonged antibiotic therapy and surgical treatment.Conclusions Active SLE patients with aPL antibodies on high-dose CS seem at high risk of developing multiple AVN complicated by infection. Avascular necrosis and bone or joint infection by S. aureus in these patients is a major complication that leads to severe joint destruction and disability.     

Septic arthritis in patients with systemic lupus erythematosus: salmonella and nonsalmonella infections compared

OBJECTIVES: To assess the clinical characteristics and outcome of systemic lupus erythematosus (SLE) with septic arthritis. METHODS: In this 20-year retrospective study, we reviewed the charts of SLE patients with septic arthritis confirmed by synovial fluid analysis and culture. To identify risk factors for septic arthritis, data of SLE patients with septic arthritis were compared with data of 100 hospitalized SLE patients without septic arthritis. RESULTS: There were 10,732 inpatient records of 3,127 SLE patients; 29 SLE patients had septic arthritis. Their ages ranged from 14 to 68 years (mean, 35.1 +/- 14.1 years). The mean SLE duration before septic arthritis onset was 30.6 months. All patients received corticosteroids; 93% had active disease (SLEDAI > or = 4). Compared with controls, avascular necrosis (AVN) of the femoral head was the most common predisposing articular disease (Odds ratio, 3.799;CI, 1.59 to 9.05). Of the 29 patients, 17 (59%) had salmonella infections and 12 (41%) had other infections. Salmonella-infected patients were younger (28.7 +/- 10.4 years) than those with nonsalmonella infections (44.1 +/- 14.0 years; P = 0.002). The hip was the most commonly affected joint, especially in the salmonella group, followed by the knees and ankles. Salmonella-infected patients were more prone to oligo-articular septic arthritis. The overall mortality rate was 10%. CONCLUSIONS: Salmonella enteritidis B is the most common pathogen causing septic arthritis in younger SLE patients. Septic arthritis tended to be oligo-articular and involve the hip joint. AVN of the femoral head was the most common predisposing articular disease. Once septic arthritis is suspected, culture specimens should be collected and appropriate antibiotics given immediately.     

Outcome of total hip replacement for avascular necrosis in systemic lupus erythematosus

OBJECTIVE: To describe the short and medium term results of total hip arthroplasty (THA) for avascular necrosis in patients with systemic lupus erythematosus (SLE). METHODS: Nineteen patients with SLE and avascular necrosis of the femoral head (AVNFH), who underwent 26 THA were retrospectively reviewed with a minimum followup of 2 years. To determine whether these patients had results similar to those of patients with other conditions, we formed a control group of 19 patients who had 29 THA. They were matched for age, sex, and followup to the patients with SLE. Controls had THA for juvenile rheumatoid arthritis (n = 7), osteoarthritis (5), adult onset rheumatoid arthritis (8), developmental dysplasia of the hip (4), and other diagnoses (5). Outcome measures included a 10 point visual analog scale (VAS) for pain, the Harris hip score, and the SF-36 self-administered health outcome questionnaire. We used the methods of Delee, Harris, and Engh for radiological assessment. RESULTS: Mean age at surgery was 46 years (range 21-71 years) and average followup was 4 years, 7 months (range 1 yr 9 mo to 9 yrs 6 mo), similar in both groups. Technical problems, mostly consisting of small, nonpropagating cracks of the calcar in uncemented stems, were encountered in 4 SLE hips and 1 control hip. Six complications were noted in the SLE group, including 2 early, nonrecurrent dislocations, 1 patient with thigh pain for 1 year, 1 pericarditis, 1 sick-sinus syndrome, and 1 urinary tract infection. There was one case of urinary tract infection in the control group. One SLE patient developed a low grade prosthetic infection and underwent successful revision 2 years after primary surgery. Clinical outcome measures had similar scores in the 2 groups: average VAS pain score = 2.00 in SLE hips (maximum 10) and 1.97 in control hips; mean Harris hip score = 86.7 in SLE patients (maximum 100) and 81.9 in controls; average SF-36 score = 63.4 in SLE patients (maximum 100) and 60.5 in controls. There was no radiological evidence of implant loosening in controls; there was 1 asymptomatic cup migration in the SLE group. CONCLUSION: In the short and medium term, patients with SLE and AVN had good results after THA. Results were similar in patients who had hip replacement for other diagnoses. Less favorable clinical outcomes of hip replacement have been reported in young patients who have AVN of other etiology (e.g., alcoholic, post-traumatic), but this was not the case in our young patients who had AVN and SLE. Thus, AVNFH and SLE should not constitute a contraindication to hip replacement.     

Avascular necrosis in systemic lupus erythematosus patients: Analysis of the demographics,clinical manifestations,management and outcomes

BackgroundAvascular necrosis (AVN) is a rare complication of systemic lupus erythematosus (SLE) that may develop from the disease or its treatment.Aim of the workTo present the clinical characteristics, management and outcomes of SLE patients with AVN.Patients and methodsThe SLE patients with AVN on X-ray or magnetic resonance imaging (MRI) following up at Fatima Memorial Hospital College of Medicine and Dentistry Shadman, Lahore, Pakistan were studied. Detailed medical history, clinical examination, laboratory investigations, treatment details and outcomes were recorded.ResultsOut of 230 SLE patients, 20 (8.6%) had AVN; 80% females, F:M 4:1 with a mean age at onset of SLE 19.8 ± 6.5 years, and mean duration till occurrence of AVN 3.9 ± 2.3 years. AVN developed within 4 years of onset in 80% of patients. Mucocutaneous symptoms were the most common presentation (95%) and 65% had positive antiphospholipid antibodies. Until AVN development, all patients received a mean of 3.5 ± 0.7 g pulse steroid and a mean cumulative oral steroid dose of 18.6 ± 6.2 g. AVN was in the hip joint in 95% of cases, 80% were bilateral. AVN was grade II in 45% patients, grade III in 25% (n = 5), I in 20% (n = 4) and grade IV in 10% (n = 2). Grade I and II had preserved joint function and didn't need any surgery compared to stage III and IV.ConclusionsIn SLE patients, AVN is a relatively early complication occurring within 4 years of illness in patients with a young age at onset, and have a better outcome when detected at earlier stages.     

Significance of anticardiolipin and anti-beta(2)-glycoprotein I antibodies in lupus nephritis

OBJECTIVE: To investigate whether anticardiolipin (aCL) and anti-beta(2)-glycoprotein I (anti-beta(2)GPI) antibodies are associated with lupus nephritis (group II patients), and whether there are differences in the prevalence of these two autoantibodies between group II patients and patients with non-nephritis SLE (group I) and primary antiphospholipid syndrome (PAPS) patients (group III). METHODS: IgG and IgM aCL were measured in 31 patients and anti-beta(2)GPI in 30 patients with systemic lupus erythematosus (SLE) nephritis and 25 without SLE nephritis and in 36 PAPS patients by validated enzyme immunoassays. Relationships of anti-double-stranded DNA (anti-dsDNA) antibodies and antibodies to the collagenous region of C1q (anti-C1q) with SLE nephritis were also examined. RESULTS: The prevalence and levels were higher for aCL, but not for anti-beta(2)GPI, antibodies in group II than in group I patients. Absolute values of aCL and anti-beta(2)GPI in all three patient groups correlated with each other. The prevalences of aCL, anti-dsDNA and anti-C1q antibodies were significantly higher in group II than in group I and group III patients. CONCLUSION: The observations in this paper suggest that raised levels of aCL antibodies are associated with lupus nephritis. We were not able to demonstrate an association between anti-beta(2)GPI antibodies and kidney disease either in patients with lupus or in patients with primary antiphospholipid syndrome. In SLE, we demonstrated that the presence of anticardiolipin antibodies in conjunction with elevated levels of anti-dsDNA and anti-C1q antibodies is highly specific for glomerulonephritis in patients with lupus.     

Antiphospholipid antibodies in mixed connective tissue disease

Summary We studied the prevalence and clinical significance of antiphospholipid antibodies (ab) in 28 patients affected with well-defined mixed connective tissue disease (MCTD). Forty-two patients affected with systemic lupus erythematosus (SLE) and 60 healthy subjects were also evaluated, as controls. In MCTD the prevalence of anticardiolipin (aCL) ab was: IgG high level 17.8% (p<0.01 versus healthy controls), IgG low level 7.1% and IgM high level 7.1%. No patients had low level of aCL IgM, lupus anticoagulant or false positive VDRL. The aCL profile was similar to that found in SLE patients, but in SLE all prevalences were higher than in MCTD. Furthermore, in MCTD patients the aCL ab were correlated with thrombocytopenia but not with recurrent thrombosis and/or abortions.     

Antiphospholipid antibodies: a risk factor for occlusive ocular vascular disease in systemic lupus erythematosus and the ''primary'' antiphospholipid syndrome.

Seven cases of occlusive ocular vascular disease affecting retinal and choroidal vessels were found among 84 consecutive patients with raised levels of anticardiolipin antibodies attending the lupus arthritis clinic at St Thomas's Hospital from 1985 to 1987. Six patients with systemic lupus erythematosus (SLE) and one with a 'primary antiphospholipid syndrome' had occlusive ocular vascular disease affecting a variety of vessels. This gives a prevalence of occlusive ocular vascular disease of 8% in this subgroup of patients, significantly higher than the 0.5-2.0% previously reported in patients with SLE. Four of these patients also suffered from cerebrovascular disease, supporting the previously documented association between occlusive ocular vascular disease and central nervous system disease in SLE. Additionally, other features of the antiphospholipid syndrome were frequently present. These findings suggest that patients with SLE and raised anticardiolipin antibodies have a higher risk of developing occlusive ocular vascular disease than has been previously reported.     

Primary antiphospholipid syndrome as the forerunner of systemic lupus erythematosus

The objective of this study was to analyse whether primary antiphospholipid syndrome (PAPS) may precede and modify the characteristics of systemic lupus erythematosus (SLE). Out of the total 362 SLE patients in our service, 223 patients had antiphospholipid antibodies (aPL), of whom 110 met the criteria of antiphospholipid syndrome. In 26 cases (7.2%) PAPS appeared 5.5 years before the onset of lupus (PAPS+SLE Group). Their clinical findings were compared to lupus patients without (SLE only Group, n = 26) and with secondary APS (SLE+SAPS Group, n = 26). The prevalence of deep venous thrombosis, stroke/TIA, recurrent fetal loss, coronary heart disease and myocardial infarction was significantly higher in PAPS+SLE Group as compared to SLE only Group. The difference in prevalence of fetal loss (P = 0.014) between PAPS+SLE and SLE+SAPS Groups was also recorded. On comparison to PAPS+SLE Group, patients without APS (SLE only Group) were younger at onset of lupus, with more frequent flares and a higher prevalence of WHO type III/IV nephritis (P = 0.007), requiring higher doses of cyclophosphamide and corticosteroids. Lupus started in the form of PAPS in 7.2% of our SLE patients, who presented with more thrombotic and less inflammatory complications than in SLE patients without a prior or with a following secondary APS. Considering the long latency between the two diseases, PAPS may be a forerunner of lupus, but it may also coexist with SLE as an independent autoimmune disorder.     

Anti-chromatin antibodies in systemic lupus erythematosus: a useful marker for lupus nephropathy

Background: Anti-chromatin antibodies have recently been described in patients with systemic lupus erythematosus (SLE) and it has been suggested that their presence is associated with lupus nephritis. Objective: To assess the prevalence and clinical associations of these antibodies in SLE. Methods: The presence of anti-chromatin antibodies in 100 patients with SLE was investigated by an enzyme linked immunosorbent assay (ELISA). To determine the specificity of these antibodies, 100 patients with primary Sjögren''s syndrome, 30 with primary antiphospholipid syndrome (APS), 10 with systemic sclerosis, and 100 normal controls were also tested. Results: Positive levels were detected in 69/100 (69%) patients with SLE. In contrast, they were found in only 8/100 (8%) of those with primary Sjögren''s syndrome, in 1/10 (10%) with systemic sclerosis, in 2/30 (7%) with primary APS, and in none of the 100 healthy controls. Patients with anti-chromatin antibodies had a twofold higher prevalence of lupus nephropathy than those without these antibodies (58% v 29%, p<0.01). A significant correlation was found between the levels of anti-chromatin antibodies and disease activity score as measured by the European Consensus Lupus Activity Measurement (ECLAM; p=0.011). Conclusions: The measurement of anti-chromatin antibodies appears to be a useful addition to the laboratory tests that can help in the diagnosis and treatment of SLE. These antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis.     

Osteonecrosis secondary to antiphospholipid syndrome: a case report, review of the literature, and treatment strategy

Osteonecrosis is commonly present in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Treatment of this condition remains extremely controversial. We present a treatment strategy of avascular necrosis of the knee in a patient with catastrophic antiphospholipid syndrome with a history of SLE and APS. Aggressive treatment with 12 rounds of plasmapheresis, intravenous immunoglobulin, rituximab, and cylophosphamide led to the patient’s recovery with no recurrence of symptoms during 16 months of follow up. In this report, we further discuss the pathogenesis of osteonecrosis and current understanding of the treatment of this disease.     

Risk factors for avascular necrosis among Filipino patients with systemic lupus erythematosus

Aim: To describe the clinical features and risk factors for avascular necrosis (AVN) in a cohort of Filipino patients with systemic lupus erythematosus (SLE). Methods: We reviewed the medical records of SLE patients with a diagnosis of AVN, seen at the University of Santo Tomas (Manila, Philippines) Section of Rheumatology, from 1995 to 2005. The diagnosis of AVN was based on clinical symptoms and confirmed by plain radiographs or magnetic resonance imaging. Possible risk factors for the development of AVN were identified. The clinical data of SLE patients without AVN were also obtained and served as controls. Results: Of the 540 patient charts reviewed, 43 (8.0%) patients (41 female, 2 male) with AVN were included. Out of a total of 66 joints involved, the hip was the most frequently involved. We included 93 SLE patients without AVN who were matched for age, sex and disease duration as the control group. Mean daily prednisone dose (11.9 ± 7.2 vs 9.3 ± 6.6 mg, P = 0.023), mean cumulative prednisone‐equivalent dose in first month of SLE diagnosis (1.5 ± 0.8 vs 1.3 ± 0.8 g, P = 0.011), and total cumulative prednisone‐equivalent dose (30.0 ± 2.7 vs 20.3 ± 1.9 g, P = 0.023) were higher in the AVN group than in the controls. Clinical variables significantly associated with AVN included the presence of vasculitis (OR = 4.45, 95% CI 1.65–12.18, P = 0.0007), the use of intravenous pulse steroids (OR = 2.92, 95% CI 1.21–7.08, P = 0.008), and the mean total cumulative prednisone‐equivalent dose ≥ 23.4 g (OR = 2.92, 95% CI 1.3–6.6, P = 0.007). Conclusion: Corticosteroid use and vasculitis were consistent risk factors seen among Filipino SLE patients who developed AVN during the course of their disease.     

Increased level of tumor necrosis factor-�� in patients with antiphospholipid syndrome: marker not only of inflammation but also of the prothrombotic state

Connections between inflammation and thrombosis are intriguing, especially in a condition such as an antiphospholipid syndrome (APS), a disease characterized by immune-mediated thrombosis. Tumor necrosis factor alpha (TNF-α) is a cytokine which shares proinflammatory and prothrombotic actions, while a soluble form of interlukin-2 receptor (sIL-2R) is considered a typical marker of (auto)immune inflammation with not known direct links to thrombosis. The differences in the pathogenesis of APS as compared to other autoimmune diseases might be connected with different serum levels of both mediators. To answer this question, we studied 147 patients with systemic lupus erythematosus (SLE), 21 with SLE-like syndrome (SLE-LS), 20 with isolated APS (primary antiphospholipid syndrome, PAPS), and 32 healthy controls. Thirty-six patients from the SLE group fulfilled the updated APS criteria (secondary APS, SAPS). In comparison to healthy subjects, TNF-α concentration was increased in all patients, while sIL-2R rose significantly in the SLE group only. APS (both SAPS and PAPS) was characterized by the highest levels of TNF-α. Moreover, patients with lupus anticoagulant or elevated levels of IgG anticardiolipin or IgG anti-β₂-glycoprotein I antibodies had higher TNF-α levels than patients without the presence of any type of antiphospholipid antibodies (aPL). In conclusion, the presence of aPL is associated with higher TNF-α level, whereas increased level of sIL-2R is rather connected with definite SLE where inflammatory processes prevail. It might be hypothesized that TNF-α plays a major role in pathogenesis of APS thrombotic phenomena.     

Risk of contralateral avascular necrosis (AVN) after total hip arthroplasty (THA) for non-traumatic AVN

The risk of developing bilateral disease progressing to total hip arthroplasty (THA) among patients who undergo unilateral THA for non-traumatic avascular necrosis (AVN) remains poorly understood. An analysis of the time-course to contralateral THA, as well as the effects of underlying AVN risk factors, is presented. Forty-seven consecutive patients who underwent THA for AVN were evaluated. Peri-operative and annual post-operative antero-posterior pelvis radiographs were examined for evidence of contralateral involvement. Patient age, weight, height, underlying AVN risk factor(s), date of onset of contralateral hip pain if occurred, and date of contralateral THA if performed, were recorded. Bone scan, computerized tomography and magnetic resonance imaging data were utilized when available. Twenty-one patients (46.6%) underwent contralateral THA for AVN within a median of 9 months after the initial THA (range 0–93, interquartile range 28.5 months). The median follow-up for patients without contralateral THA was 75 months (range 3–109, interquartile range 69 months). Thirty-four patients had radiographic findings of contralateral AVN at study entry; 25 were symptomatic bilaterally at entry and 7 developed contralateral symptoms within a mean time of 12 months (median 10 months, interquartile range 12 months). None of the 13 patients who were free of radiographic evidence of contralateral AVN at study entry developed evidence of AVN during the follow-up. AVN associated with glucocorticoid use was more likely to manifest as bilateral disease than either idiopathic AVN or ethanol-associated AVN (P=0.02 and P=0.03 respectively). Radiographically-evident AVN in the contralateral hip at THA is unlikely to remain asymptomatic for a prolonged period of time. Conversely, asymptomatic contralateral hips without radiographic evidence of AVN are unlikely to develop clinically significant AVN.     

The prevalence of antibodies to anionic phospholipids in patients with the primary antiphospholipid syndrome, systemic lupus erythematosus and their relatives and spouses

OBJECTIVES: Antiphospholipid antibodies (aPL) have been associated with syndromes involving thrombosis, fetal loss and thrombocytopenia. Genetic and environmental conditions are among the factors attributed to the cause of autoimmune diseases such as the antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). The aim of this study was to determine whether these factors determine the prevalence of aPL. METHODS: Three groups of patients were tested for the presence of IgG, IgM and IgA anticardiolipin (aCL), antiphosphatidylinositol (aPI), antiphosphatidylglycerol (aPG) and antiphosphatidylserine (aPS) antibodies: (i) patients with primary APS (PAPS); (ii) patients with SLE and secondary APS; and (iii) patients with SLE without APS. First-degree relatives and spouses of patients with SLE/APS were also tested for circulating aPL. RESULTS: IgG aPL were particularly prevalent in patients with PAPS. IgG aPI and aCL were more prevalent in patients with PAPS than the IgM equivalents (P < 0.0001). Notably, none of the patients with PAPS had IgA aPL. A significantly higher number of relatives of patients with SLE/APS possessed IgG aPL than the normal controls. Except for aPG (P < 0.03), the prevalence of these antibodies in the relatives was not significantly different from patients with SLE/APS. The relatives also had significantly higher prevalence of IgG aPI, aPS and aCL antibodies than IgM aPL antibodies. In contrast, the prevalence of IgG aPL in the spouses was no different than in the healthy controls. CONCLUSIONS: Genetic factors, shared by patients and their relatives, seem to have some effect on the prevalence of aPL in the subjects studied, while environmental factors shared by spouses appear to have no influence.     

Preclinical vascular disease in systemic lupus erythematosus and primary antiphospholipid syndrome

OBJECTIVE: To determine the prevalence of preclinical vascular disease and associated risk factors in patients with systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (APS). METHODS: We consecutively studied 70 SLE patients and 25 primary APS patients without clinical coronary artery disease. The control group included 40 healthy women. Carotid ultrasound was performed and the intima-media wall thickness (IMT) and presence of plaque was investigated in all patients and controls. Traditional vascular risk factors and SLE-disease and treatment related factors were also analysed. RESULTS: SLE patients had a higher prevalence of traditional atherosclerosis risk factors: hypertension (P<0.005) and dyslipidaemia (P<0.05) and higher levels of total cholesterol (P = 0.03), triglycerides (P = 0.004) and apolipoprotein B (P = 0.04). The prevalence of carotid plaque was higher and appeared earlier in SLE patients than in the primary APS patients or controls (P<0.001). The IMT was similar in the three groups. SLE patients with secondary APS had a higher prevalence of carotid plaque than patients with primary APS (37.5% vs 8%, P = 0.03). The presence of plaque in SLE patients was associated with a higher SLICC score (2.40 +/- 1.78 vs 1.02 +/- 1.18, P = 0.002), higher ECLAM score (3.10 +/- 2.32 vs 1.84 +/- 1.59, P = 0.02) and older age (47.3 +/- 8.44 vs 37.38 +/- 11.28, P = 0.003) at the time of carotid ultrasound study. CONCLUSION: Plaque prevalence in patients with primary APS is similar to that of controls and inferior to that of SLE patients with secondary APS. SLE patients have a high prevalence of early carotid atherosclerosis that is associated with cumulative disease damage and disease activity.