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The aim of this study was to evaluate age-related changes in cortical and trabecular bone mineral density (BMD) in South African subjects, and to develop a local reference database for dual energy X-ray absorptiometry with the QDR 1000 densitometer. A questionnaire was used to recruit volunteers. Age, years since menopause, use of medications and medical diseases were recorded. Men were excluded. Only whites and coloureds were studied. Three hundred and eleven women had single measurements over a 2-year period. Seven sets of subjects were defined according to age (18-44; 45-49; 50-54; 55-59; 60-64; 65-69; > 70 years) (N = 163; 32; 35; 23; 25; 16; 17 respectively). Height and weight did not change significantly with age. There were significant differences in lumbar and femoral BMD (Ward's) compared with those of American subjects in the different age categories. Both the lumbar and total femoral BMD began to fall significantly between the ages of 60 and 65 years (P < 0.01). Ward's triangle showed a significant fall in BMD between 45 and 50 years of age (P < 0.01). Ward's BMD predicted 36% of the variation in lumbar BMD at 45 years but 10% at 70 years. Ward's triangle is a useful predictor of femoral bone loss in later years. The fracture threshold at the lumbar vertebrae was 0.822 g/cm2; at Ward's triangle it was 0.443 g/cm2. This gave a 16% prevalence of osteopenia in the lumbar region and a prevalence of 24% at Ward's area.

A case of non-cirrhotic portal hypertension in a patient with systemic lupus erythematosus, the first of our knowledge, is described. Severe pulmonary hypertension was associated with the portal hypertension and with markers of active auto-immunity. Pulmonary hypertension has not previously been associated with non-cirrhotic portal hypertension. The coexistence of vasculopathy of the portal and pulmonary vascular beds in this patient with active autoimmunity supports the postulate that portal-pulmonary hypertension may be immunologically mediated.

Objective: To evaluate trabecular bone mineral density (BMD) in young ambulatory female patients with systemic lupus erythematosus (SLE)., Methods: Bone mineral density (gm/cm2) at the lumbar vertebrae (L1-L4) and at the left femur (neck, trochanter, intertrochanter, and Ward's triangle) was measured by dual x-ray absorptiometry in 46 SLE patients (mean age 31 years, mean disease duration 76 months) and in 108 healthy female controls (mean age 32 years). Twenty-two of the SLE patients were receiving corticosteroids (CS) at the time of the study., Results: Lumbar BMD in the SLE patients was less severely reduced than was BMD at the femoral sites, but the SLE group was closer to the lumbar fracture threshold of 0.812 gm/cm2 than was the control group (P = 0.0009). There were no significant differences between the SLE patients currently being treated with corticosteroids and those who were not (P > 0.3). BMD at Ward's triangle and at the femoral neck was not significantly reduced in the SLE patients. Total femoral BMD had a sensitivity of 76% and specificity of 62% in differentiating the SLE group from the controls. The positive predictive value was 61% and the negative predictive value was 89%. The prevalence of osteopenia in the SLE patients was 25%., Conclusion: SLE causes significant trabecular bone loss, which is not due to corticosteroid therapy.

We report the prevalence of metacarpal cortical thinning in systemic lupus erythematosus (SLE). Fifty-eight ambulant female patients attending a lupus clinic (mean age 32.4 years), were found to have significant thinning of metacarpal cortices (p < 0.05) when compared with 63 normal females (mean age 34.1 years). However, metacarpal bone mass was within the normal range. Measurements were made at 6 metacarpals of the 2 hands using a computer-aided technique (digitized radiogrammetry). Femoral cortical width and Singh index at the left femur, as well as the vertebral index at L3 were also recorded. The trabecular indices were in the range of normality, but the SLE group had more patients in the immediately pre-osteopenic range. Metacarpal bone loss was not related to disease duration or corticosteroid therapy. The prevalence of osteopenia in SLE is probably underestimated and the pathogenesis is likely to be multifactorial.

Sixty-five patients with rheumatoid arthritis (RA) (mean age 37.2 years) were compared with 71 controls (mean age 33.8 years). Anthropometric measurements included body diameters and skin-fold thickness at multiple sites, while biochemical markers of nutritional status included serum albumin, thyroxine-binding pre-albumin and retinol-binding globulin levels. None of the RA subjects was outside the range that extended 2 standard deviations above and below the normal control values for lean body mass. Discriminant analysis showed that corticosteroid therapy did not significantly influence skinfold thickness in RA. A combination of bi-acromial and bi-ankle diameters had a sensitivity of 70% and a specificity of 72% in differentiating the RA group, in whom disease activity had a greater effect on body diameters than corticosteroid therapy did. Differences related to functional impairment were a manifestation of disease activity rather than a direct effect on skinfold thickness or body diameters. According to anthropometric measurements in ambulant patients, RA does not result in malnutrition in young individuals.

The development of myasthenia gravis (MG) in a patient with juvenile chronic arthritis (JCA) is a rare occurrence. This report concerns a patient with seropositive JCA who developed MG during her second pregnancy.

Osteoporosis in RA is mediated by numerous inflammatory substances. This study was undertaken to see if SAARD could modify the rate of metacarpal bone loss in RA. Combined cortical thickness (CCT) measured at the midshaft of the right second metacarpal was used to calculate bone mass (CA%) using a digitizer. Eighty-one subjects were studied, all of whom had at least three sets of hand X-rays, the last of which was approximately 18 months following initiation of SAARD therapy. There were 12 males and 69 females. The mean age at time of starting therapy was 51 (SD 12) years while the mean duration of disease at the time was 7.6 (SD 8) years. The mean time to referral for SAARD from the general clinic was 2.5 (SD 3) years. The percentage fall in bone mass prior to therapy was 2.51%/day compared to a gain of 0.6%/day after therapy (P less than 0.05). Forty-nine patients were aged over 50 years while 32 were 50 years or younger at the time of study. Comparison showed that in the pretreatment period, the rate of change in CCT and CA% was not significantly dependent age (P less than 0.1). During that therapy, the rate of change in CCT and CA% significantly different in the two age groups. Patients aged over 50 years continued to lose bone, but at a slower rate (P less than 0.05). Patients aged 50 years or less either stopped losing or gained metacarpal bone mass during the study period (P less than 0.005). The time to referral for SAARD and disease duration (comparable in the two age groups) did not have a significant effect on changes in CA% during therapy. Change in bone mass could be predicted by change in disease activity. We conclude that SAARD have a significant sparing effect on metacarpal osteoporosis in RA. This positive effect is masked by the overwhelming influence of age (and menopause) and could be missed. Metacarpal osteoporosis seems a pathophysiologically more useful measure of radiological change in RA than erosions or joint space narrowing.

Extensive botryomycosis was diagnosed in a patient with diabetes mellitus with chronic active hepatitis. Peptostreptococcus organisms and Staphylococcus aureus grew from the sulfur granule cultures. The patient's neutrophils showed notable inhibition of chemotaxis toward endotoxin-activated normal serum. The patient's serum also had an inhibitor to neutrophil chemotaxis, This inhibiting substance was probably antistreptolysin O. Results of other tests of neutrophil function and skin tests to evaluate cell-mediated immunity were normal.

Radiogrammetry remains a convenient method of bone mass measurement. It is the only objective means of quantifying metacarpal osteoporosis (OP) in rheumatoid arthritis (RA). An automated technique using a digitizer (interfaced with an IBM PC) for measurement of combined cortical width (CCW) at the mid-shaft of six metacarpals was evaluated in three groups of individuals under 50 years of age (98 normal controls, 96 RA, 63 SLE). Intra-observer, inter-observer, and inter-institution reproducibility was assessed with a 'phantom' embedded in a rectangular mould of wax. Intra-patient variation was also assessed in RA patients seen on two occasions less than a month apart. Two hundred and fifty-seven subjects were studied. The method was found to be reproducible for a single observer, among five different observers and in two separate institutions. The RA subjects seen on two occasions showed no significant differences in CCW. The technique showed significant differences of CCW in the three groups of premenopausal subjects (controls; RA; SLE) studied (p less than 0.001). The six metacarpal bone mass was calculated in less than 5 min. The technique of digitized radiogrammetry is an improvement on the Vernier caliper technique. The method is useful for epidemiological cross-sectional studies and for evaluation of long-term radiological changes in RA.

A cross sectional analysis of the correlation between clinical, laboratory, and radiological markers of disease activity in 98 patients with classical rheumatoid arthritis (RA) is reported. The median age was 38 years, the median age at onset of disease 29 years, and the median duration of disease seven years. The Keitel function test (KFT) showed good correlation with the Ritchie articular index (RAI) (p less than 0.0001; r = 0.5) and the disability questionnaire (DQ) (p less than 0.0001; r = 0.6). The RAI and DQ correlated weakly with laboratory variables, while the KFT showed significant correlation with the erythrocyte sedimentation rate (ESR), C reactive protein (CRP), and plasma viscosity (PV) (p less than 0.001; r = 0.4; 0.3; 0.4). Only the KFT showed significant correlations with bone mass measurements (p less than 0.01; r = -0.3; -0.4), and the Larsen index at the right wrist (p less than 0.0001; r = 0.4). Consensus analysis suggested that the KFT is a useful single clinical test of disease activity in RA. The hand functional index (HFI), a component of the KFT, showed significant correlation with the total KFT (r = 0.9). Prospective drug trials are needed to establish the value of the HFI in the monitoring of patients with RA.

The association between rheumatoid arthritis and HLA was studied in three South African populations: whites (N = 66), blacks (N = 33) and patients of mixed ancestry (Cape Coloureds) (N = 183). The antigens of the HLA-A, B, C, DR and DQ loci were detected by microlymphocytotoxicity assay and their frequencies in the three patient groups were compared with the corresponding frequencies in normal individuals of the same population group. HLA-DR4 was significantly associated with rheumatoid arthritis in whites (P less than 0.0001), blacks (P less than 0.001) and patients of mixed ancestry (P less than 10(-6]. The relative risk for HLA-DR4 was 3,2 in whites, 3,9 in blacks and 3,7 in patients of mixed ancestry. Other significant associations detected were with HLA-A2 in whites and HLA-B8 and DQw3 in patients of mixed ancestry. The constant association with HLA-DR4 seen in the different populations provides support for the suggestion that the HLA genes themselves are responsible for the genetic susceptibility to rheumatoid arthritis.

Avascular necrosis (AVN) of the hips is associated with significant disability, and the majority of established cases require major surgery. In a retrospective analysis of 185 patients with systemic lupus erythematosus (SLE) 13 (7%) were found to have AVN. Of these, six had Raynaud's phenomenon, all had been on corticosteroids, and one had digital vasculitis. The mean duration of corticosteroid therapy was two years (range four months to five years). Five patients developed AVN two to 10 years after discontinuing steroids. The mean duration of disease at the onset of AVN was 6.85 years (range 1-19 years), and the mean age at onset of AVN was 31 years. Ten patients had severe multisystem involvement. None of the patients abused alcohol. Surgery was performed on 11 hips. Three had total hip replacement for stages 3 and 4 and seven had core decompression for stages 1 and 2. AVN progressed in two (28%) of these patients. In another patient core decompression failed for technical reasons. She subsequently required total hip replacement. The early detection of AVN to avoid the need for major surgery is stressed.