AVASCULAR NECROSIS OF THE HIP IN SYSTEMIC LUPUS ERYTHEMATOSUS: THE ROLE OF MAGNETIC RESONANCE IMAGING

The value of magnetic resonance imaging (MRI) in the early diagnosisof avascular necrosis (AVN) of the hip in SLE was investigated. Twenty females with severe SLE were studied prospectively. Eachunderwent 6-monthly X-rays, technetium -99m (Tc-99m) pyrophosphatebone scans and MRI of the hips over a 3-yr period. AVN was diagnosed in five hips of three patients (15%) duringthe study period. It was confirmed histologically in three hipsof two patients who underwent core decompression. Radiologicalevidence of AVN was present in two patients at diagnosis. Onepatient developed progressive radiological changes despite coredecompression. Bone scintigraphy was abnormal at some stagein all three patients with AVN however failed to detect theearly ischaemic stage of AVN. MRI was the most reliable investigation and was able to detectasymptomatic AVN prior to the appearance of radiological orscintigraphic abnormalities. KEY WORDS: Osteonecrosis, Core decompression, Femur     

AVASCULAR NECROSIS OF THE FEMORAL HEAD IN SICKLE CELL TRAIT

Avascular necrosis of the femoral head is known to occur insickle cell anaemia (Tanake, Clifford and Axelrod, 1956), andsome of the genetic variants of sickle cell disease (Chung andRalston, 1969). In sickle cell trait, only two cases of thisrare complication are reported, both in American negroes (Ratcliffand Wolf, 1962). We now report a third patient, a woman, withsickle cell trait and avascular necrosis. ^*Present address: Norfolk and Norwich Hospital, St. StephensRoad, Norwich.     

THE EFFECT OF SYSTEMIC LUPUS ERYTHEMATOSUS AND LONG-TERM STEROID THERAPY ON BONE MASS IN PRE-MENOPAUSAL WOMEN

The aim of our study was to assess bone mineral density (BMD)in pre-menopausal women with systemic lupus erythematosus (SLE)and the influence of disease activity and use of corticosteroids.Lumbar and femoral BMD were measured in 43 patients with SLE(28 on regular steroid therapy, 15 with recent onset, non-treated)and compared with 43 healthy women matched for age. In addition,21 SLE patients treated with corticosteroids were followed-upwith a mean of 36.6±12.7 months. BMD was significantlylower in SLE patients with prednisone doses     

系统性红斑狼疮患者外周血淋巴细胞早期凋亡的研究

目的：为了解观察ＳＬＥ患者休内细胞凋亡情况，并其外周血淋巴细胞早期凋亡率进行了检测，方法：应用最新的ＡｎｎｅｘｉｎＶ检测试剂盒及流式细胞仪检测早期凋亡细胞。结果：ＳＬＥ活动期患者外周血淋巴细胞早期凋亡率（１２．６１±６．３２，ｎ＝２）明显高于稳定期患者（４．４７±３．３９，ｎ＝８，Ｐ〈０．０１）及正常人（５．１３±３．３７，ｎ＝１１，Ｐ〈０．００１），但与ＳＬＡＭ评分无相关，活动期患者初发病组（１     

RAPID DEVELOPMENT OF CEREBRAL ATROPHY IN SYSTEMIC LUPUS ERYTHEMATOSUS

A 21-year-old female is described who developed severe cerebrallupus 9 months after the onset of her disease. A dramatic changein her CT brain scan from normal to gross cerebral atrophy wasobserved in the month following onset of her cerebral disease. This patient developed hepatitis during the course of her hospitalization.This is an unusual manifestation of systemic lupus. The patient'sserum also contained anti-Sm antibodies. This is a relativelyinfrequent finding in the serum of lupus patients outside theUnited States. KEY WORDS: Systemic lupus erythematosus, Cerebral disease, Sm antibodies     

DETECTION OF AUTOANTIBODIES TO THE 90 kDa HEAD SHOCK PROTEIN IN SYSTEMIC LUPUS ERYTHEMATOSUS AND OTHER AUTOIMMUNE DISEASES

Expression of the highly conserved 90 kDa heat shock protein(Hsp90) is elevated in the peripheral blood mononuclear cellsof approximately 25% of patients with SLE. Conflicting datahave been published about the frequency of antibodies to Hsp90with the previous methodology using a complex Western blot system.We now describe an ELISA to measure autoantibodies to Hsp90and Hsp70 in SLE patients, healthy controls and patients witha variety of autoimmune rheumatic diseases, IgG and IgM antibodieswere elevated in 26 and 35% of SLE patients, respectively. Theseresults show autoantibodies to Hsp90 (but not Hsp70) are elevatedin a significant proportion of patients with SLE (P<0.025)compared to healthy controls; and that those with raised antibodylevels were more likely to have renal disease and a low C3 level(P<0.02). KEY WORDS: Heat shock protein 90, Autoantibodies, Systemic lupus erythematosus, Autoimmunity, Myositis     

SLE发病中可溶性细胞间粘附分子-1变化的研究

目的：研究呆溶性细胞间粘附分子－１在系统性红斑狼疮中的变化。方法：用双抗体夹心ＥＬＩＳＡ方法对２５例活动期ＳＬＥ病人血清和末梢血单个核细胞培养上清液ｓＩＣＡＭ－１水平进行检测。对部分稳定期病人重复检测。结果：活动期ＳＬＥ病人血清ｓＩＣＡＭ－１水平较正常对照组明显增高，活动期ＳＬＥ病人ＰＢＭＣ培养上清液中ｓＩＣＡＭ－１水平低于正常对照组，但两组间无明显差异。     

ASEPTIC NECROSIS OF THE FEMORAL HEAD IN SICKLE-CELL DISEASE

Aseptic necrosis of the femoral head accounted for 75 (28.2%)of the 266 major skeletal complications seen in 207 patientswith sickle-cell disease in a 66-month period. Forty-five (60%)of the 75 patients were males. The onset of symptoms occurredbetween the ages of 10 and 29 years in 60 (80.0%) of the patients,and the mean age at onset was 20.8 (range 8–54) years.There were 37 patients with sickle-cell anaemia (SS) with 46hips affected by necrosis, and 38 patients with sickle-cellhaemoglobin C with 40 affected hips. Perthes-like changes occurredin 40 hips, osteochondritis dissecans-like lesion in one hipand severe hip deformity in 45 hips. Four of the five hips withPerthes-like necrosis which were treated by rotation upper femoralosteotomy had partial reconstitution of the femoral head, andall five were symptom-free. The other hips were treated conservativelywith generally poor results. KEY WORDS: Femoral head necrosis, Osteonecrosis, Anaemia, sickle-cell     

CYCLOSPORIN A IN THE TREATMENT OF SYSTEMIC LUPUS ERYTHEMATOSUS: RESULTS OF AN OPEN CLINICAL STUDY

In order to define the effects and safety of cyclosporin A (CsA)in systemic lupus erythematosus (SLE), we conducted an openclinical trial with 16 SLE patients. During an observation periodof up to 64 months and an average treatment period of 30.3 months,16 SLE patients, who did not have adequate disease control orexperienced side-effects with their previous immunosuppressivetherapy, were treated with CsA (3–5 mg/kg). In 3/16 patients,CsA treatment was discontinued because of side-effects, in twobecause of inefficacy and in 2/16 because of a pregnancy. Fourout of 16 patients had a flare of disease during CsA therapy7, 24, 36 and 40 months after initial response to therapy; onepatient stopped CsA treatment after 54 months of successfuldisease control. Four out of 16 patients are still on CsA. Thebest beneficial effect was observed in 10 patients with proteinuria,which decreased from 4.7 ± 2.6 to 1.5 ± 1.1 g/24h.In 3/3 patients with thrombocytopenia and 3/3 patients withleucocytopenia, platelets and leucocytes returned to normalvalues. The most frequent side-effects were hypertension anddeterioration of renal function (3/16) and hypertrichosis (5/16).According to the preliminary results of this study, CsA waswell tolerated and able to control disease activity over anextended time period. These data should encourage investigatorsto perform a multicentre controlled trial on CsA therapy inSLE. KEY WORDS: Systemic lupus erythematosus, Therapy, Cyclosporin A, Proteinuria     

TRANSVERSE MYELITIS IN SYSTEMIC LUPUS ERYTHEMATOSUS: TWO CASES WITH MAGNETIC RESONANCE IMAGING

Transverse myelitis is one of the most serious neurologicalcomplications occurring in the course of systemic lupus erythematosus.We describe two lupus patients, with transverse myelitis, oneof whom had associated optic neuritis. In both, magnetic resonanceimaging of the spinal cord showed an abnormal signal. In onecase a good response to steroid and immunosuppressive therapywas observed; the other case failed to improve despite the therapyapplied. KEY WORDS: Transverse myelitis, Systemic lupus erythematosus, Magnetic resonance imaging     

IDENTIFICATION OF THE 9G4 IDIOTOPE IN SYSTEMIC LUPUS ERYTHEMATOSUS

An idiotope designated 9G4 (9G4^Id) is known to be a marker forimmunoglobulins which utilize a particular VH gene, V_H4–21.The idiotope has been found to be present on anti-DNA antibodies,and have been identified in 45% of sera from patients with SLE.This idiotope is strongly associated with lupus being very uncommonamongst the other autoimmune rheumatic diseases tested. Thisdistinction is unlike virtually any of the other DNA antibodyidiotypes described which are much more widely distributed.9G4^Id levels were found to fluctuate with disease activity insome lupus patients and this idiotope was detected in 3/11 SLErenal biopsies tested. Its presence is associated with the HLAmarkers A1 and B8 and raised 9G4^Id levels are not simply a reflectionof hypergammaglobulinaemia. Thus a new DNA antibody associatedidiotope has been identified. Expression of the idiotope indicatesthat a notable proportion of anti-DNA antibodies have VH segmentsencoded by the same, or closely related genes, and that theserestricted immunoglobulins are involved in the renal pathologyfound in SLE. KEY WORDS: Idiotope, Systemic lupus erythematosus, DNA antibodies     

系统性红斑狼疮消化系统表现的特殊类型——附4例报告

本文报告了4例以消化系统症状为首发和突出表现的系统性红斑狼疮病人，结合文献复习，分析了系统性红斑狼疮消化症状产生的可能原因，并总结了系统性红斑狼疮的以消化道表现为首发和突出症状的一些特殊类型，它们在临床上尤易误诊，值得同道共识。     

系统性红斑狼疮患者自身肾上腺皮质功能状态与发病关系的初步研究

目的：探讨系统性红斑狼疮（ＳＬＥ）患者自身肾上腺皮质功能状态与发病的可能关系。方法：用放射免疫分析（ＲＩＡ）法测定了２６例初发ＳＬＥ患者的血浆皮质醇（ｃｏｒｔｉｓｏｌ）浓度及其昼夜节律的变化，并与１８例正常人对照。结果：观察组皮质醇浓度晨８时较对照组降低，夜２３时及午１６时较对照组升高（Ｐ＜００１，Ｐ＜００２），部分患者峰值出现在１６时或２３时，使皮质醇昼夜节律呈平坦曲线。结论：提示ＳＬＥ患者部分存在肾上腺皮质功能紊乱，体内这一免疫自稳机能失常可能与ＳＬＥ的发病有关     

CURRENT CONCEPTS IN THE ETIOPATHOGENESIS AND TREATMENT OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Abstract Systemic lupus erythematosus is an autoimmune disorder that occurs spontaneously in humans and mice. Genetic factors play an important role in the predisposition to and expression of disease. Environmental factors augment the expression of illness and in the absence of normal control mechanisms may provide the stimulus to autoimmunity. Sex hormones modulate the immune response and tend to modify disease expression. Disordered immune regulation may be due to a primary or secondary abnormality in cellular, cytokine, and/or humoral function. Therapy for SLE is directed towards suppression of exaggerated immunological and inflammatory activity. This review will re-evaluate current therapy and describe newer approaches including the use of pharmacological, hormonal, immunological, dietary, and physical modalities. (Aust NZ J Med 1986; 16: 729–743.)     

SYSTEMIC LUPUS ERYTHEMATOSUS IN 61 ORIENTAL MALES. A STUDY OF CLINICAL AND LABORATORY MANIFESTATIONS

The clinical and laboratory features of 61 oriental male lupuspatients were compared to those of 86 oriental female patientsto determine whether gender differences occur. Arthritis wassignificantly less common in the males. Neuropsychiatric disorderswere less frequent but the difference did not reach statisticalsignificance. Renal disease was the commonest clinical manifestationand diffuse proliferative glomerulonephritis the dominant histologicalfinding on renal biopsy in the males. The prevalences of leucopeniaandantibodies to extractable nuclear antigens in particular anti-Ro(SSA) and anti-La(SSB), were lower in men. Arthritis and serositiswere less common in our oriental males in contrast to the Caucasianpatients. These findings provide further evidence of differencesbetween the genders in SLE and suggest racial factors may affectclinical presentation. KEY WORDS: Male lupus, Arthritis, Leucopenia, Anti-Ro, Anti-La, Serositis     

系统性红斑狼疮患者淋巴细胞粘附分子表达的观察

用流式细胞术及免疫双荧光染色法，分析了３５例系统性红斑狼疮（ＳＬＥ）患者外周血淋巴细胞粘附分子表型（ＣＤ＿（１１ａ）／ＬＦＡ－ｌα、ＣＤ＿（１８）／ＬＦＡ－１β、ＣＤ＿（５４）／ＩＣＡＭ－１）。结合淋巴细胞变化对ＳＬＥ作进一步探讨。结果发现ＳＬＥ活动期ＣＤ＿（１１ａ）、ＣＤ＿（１８）表达随ＣＤ＿４细胞减少而降低、ＣＤ＿８细胞增多而增高，ＣＤ＿（５４）在ＣＤ＿（２０）细胞上亦增高。此外，ＣＤ＿８细胞的ＣＤ＿（１８）增高与ＣＤ＿４ＣＤ＿（４５）ＲＡ￣＋细胞降低呈负相关（Ｐ＜０．０５），而与ＣＤ＿（２０）细胞的ＣＤ＿（５４）增高呈正相关（Ｐ＜０．０１）。提示粘附分子可能在ＳＬＥ发病机理中具有重要意义。     

THE RELATIONSHIP OF ANTICARDIOLIPIN ANTIBODIES TO DISEASE ACTIVITY IN SYSTEMIC LUPUS ERYTHEMATOSUS

Sera from 124 blood donors, 60 rheumatoid arthritis (RA) and57 SLE patients were measured for anticardiolipin antibodiesby ELISA. Significantly raised IgG (aCLG) and IgM (aCLM) anticardiolipinantibody levels were found in RA and SLE (p<0.0005). However,in SLE, both aCLG and aCLM levels were significantly higherthan in RA (p<0.0025). We then conducted a transectionalstudy to evaluate aCL levels and disease activity in SLE. Therewas a good positive predictive value (70%) between aCL and overalldisease activity, but not for individual systems. A strong associationbetween aCL and renal involvement irrespective of activity wasalso found (80%). Nine SLE patients fulfilled both the clinicaland serological criteria for the antiphospholipid syndrome (APS)and a further 18 patients fulfilled the serological criteriafor APS. Results indicate that aCL levels are of value in predictingoverall disease activity in SLE and in monitoring those patientswho fulfil or partially fulfil the criteria for APS. KEY WORDS: Anticardiolipin antibodies, Systemic lupus erythematosus, Disease activity     

PREVALENCE AND CLINICAL SIGNIFICANCE OF ANTIBODIES TO RIBONUCLEOPROTEINS IN SYSTEMIC LUPUS ERYTHEMATOSUS IN MALAYSIA

One hundred and seventy patients with systemic lupus erytbematosus(SLE) were studied for the prevalence of antibodies to the smallRNA-associated proteins Ro/SSA, La/SSB, Sm, UIRNP and Sm. Therelationship of these autoantibodies to different races, sexesand clinical manifestations of SLE was evaluated. Passive immunodiffusionwas employed using human spleen extract as antigen source forRo and rabbit thymus extract for La, Sm and UIRNP. We foundthe prevalence of antibodies to be as follows: anti-Ro/SSA,36%; anti-La/SSB, 8%; anti-Sm, 15%; anti-UIRNP, 21%. Exceptfor a low prevalence of anti-La, the prevalence of these antibodieswas similar to that in Western studies. The prevalence of anti-Ro/SSAis similar to that reported in the Western studies, but lowerthan that reported in other Oriental patients from Singaporeand Hong Kong. Linkages of anti-Ro with anti-La antibodies wereusual; however, although anti-Sm antibodies were usually associatedwith anti-UIRNP, they were more frequently associated with anti-Roantibodies. The Malay patients had a high prevalence of anti-UIRNPcompared to other races. No gender difference was detected.Anti-Sm antibody was associated with scrositis and anti-UIRNPantibodies with Raynaud's phenomenon. No association was foundbetween the presence of skin, renal or cerebral manifestationsand any specific antibodies or combination of antibodies. KEY WORDS: Systemic lupus erythematosus, Racial comparison, Ro/SSA, La/SSB, Sm, UIRNP