Botulinum neurotoxin injections in essential infantile esotropia-a comparative study with surgery in large-angle deviations.

PURPOSE: To compare botulinum neurotoxin (BNT) injections to surgery as first-line therapy in large-angle essential infantile esotropia (IE). PATIENTS AND METHODS: Children between the ages 6 months and 6 years with IE of ≥40 prism dioptres (PD) were randomised to either a maximum of three BNT injections or surgical intervention of bimedial rectus muscle recession for angles ≤60 PD and augmented with BNT injection in angles >60 PD. Time taken for each procedure was documented. Orthophoria or misalignment of ≤10 PD was regarded as a complete response (CR). Follow-up visits were done at 3, 6, 12 and 24 weeks. RESULTS: Mean (SD) age and baseline angle of esotropia were 26.9 (14.5) months and 61.9 PD (12.8), respectively, for the overall cohort. The proportion of children who achieved CR was significantly higher in the surgery arm compared to the BNT injection arm (OR = 4.01, 95% CI 1.74-9.22) but the time taken was six times longer (p < 0.0001). In the BNT arm, 55.2% of children aged ≤24 months and 16% of children >24 months achieved CR. In children with esotropia ≤60 PD, CR was achieved in 50% while those with esotropia >60 PD CR was achieved in 25%. CONCLUSION: Surgery remains the gold standard for treatment of esotropia but BNT injection is a safe and effective alternative in children ≤24 m and with smaller angles of esotropia ≤60 PD in resource-limited centres.

'The feeling of not being entitled to something': fertility, pregnancy, and sexuality among women with systemic lupus erythematosus in South Africa.

Objective: Fertility, pregnancy, and the postpartum period can pose many challenges for patients with systemic lupus erythematosus (SLE) in sub-Saharan Africa. We explored the perceptions and experiences of South African women relating to fertility and pregnancy.Method: In-depth interviews were conducted with 25 consenting women with SLE. We explored their perceptions and experiences on conception, pregnancy, and sexuality. Data were analysed using Nvivo software.Results: Participants had a mean age of 30.9 years (range 22-45 years) and mean disease duration of 4.5 years (range 1-5 years). The majority were black Africans, and the remainder were of mixed racial ancestry. Unemployment, low educational level, and singlehood status were the most predominant sociodemographic features. Most participants had been pregnant and a few reported being sexually inactive. Participants described many negative pregnancy outcomes including lupus flares, miscarriages, premature deliveries, prolonged hospitalization, and unexpected caesarean sections. Conflicting medical advice on conception, together with conflicting personal, cultural, and societal pressures to procreate, resulted in emotional turmoil and pessimism. Participants frequently described intimacy problems, loss of libido, and infidelity by partners leading to sexually transmitted infections. Aesthetic and physical concerns were perceived as the main causes of infidelity. Most participants felt confined to these relationships as they were financially dependent on their partners, which added to their stress.Conclusion: A combination of patient-centred care focusing on safe, effective contraception and medication targeting remission state, constant counselling, consistent information, and a pregnancy managed jointly by an obstetrics and rheumatology team could achieve optimum results.

Spectrum of cutaneous lupus erythematosus in South Africans with systemic lupus erythematosus.

BACKGROUND: Cutaneous involvement is very common in systemic lupus erythematosus. We describe the prevalence and spectrum of lupus-specific (cutaneous lupus erythematosus) and non-specific cutaneous features amongst mostly black South Africans with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective record review of 298 South Africans (262 blacks and 36 non-blacks) with systemic lupus erythematosus was carried out. Cutaneous features were classified according to the Gilliam and Sontheimer classification of cutaneous lupus. RESULTS: Most (81.5%) patients were black African females. The mean (SD) age at diagnosis and follow-up duration were 35.0 (11.8) and 8.0 (5.9) years, respectively. Cutaneous lupus erythematosus was seen in 76.1% of patients, mainly chronic cutaneous lupus erythematosus with the discoid lupus erythematosus subtype seen in 52.1% of patients. Acute cutaneous lupus erythematosus was seen in 30.2% of patients and was more common in non-blacks than blacks (odds ratio = 3.8 (1.9-7.9)); localized acute cutaneous lupus erythematosus was more common than generalized acute cutaneous lupus erythematosus (odds ratio = 2.6 (1.6-4.4)). Non-specific cutaneous features occurred in 77.2%, with oral/nasal ulcers and Raynaud's phenomenon each occurring in approximately 40% of patients. Diffuse melanonychia at initial diagnosis was present in 37.4% of patients and was more common in blacks than non-blacks (odds ratio = 3.1 (1.3-7.3)). Acute cutaneous lupus erythematosus was associated with renal disease (odds ratio = 2.8 (1.6-4.7)) and chronic cutaneous lupus erythematosus with arthritis (odds ratio = 2.02 (1.24-3.29)). Diffuse melanonychia was associated with less renal disease and anti-dsDNA antibody positivity (odds ratio = 0.4 (0.3-0.7) and 0.4 (0.2-0.6), respectively) and significantly lower lupus severity index scores (mean (SD) = 5.99 (1.11) vs 6.56 (1.36) in patients with no melanonychia, p < 0.05)). CONCLUSION: In this study of South Africans with systemic lupus erythematosus, the skin was the most commonly affected organ. In general, cutaneous lupus erythematosus was associated with less severe systemic disease. Acute cutaneous lupus erythematosus was less common in blacks, whereas discoid lupus erythematosus was more common than reported in Caucasians. Diffuse melanonychia was a distinctive finding and was associated with milder systemic disease.

Living with systemic lupus erythematosus in South Africa: a bitter pill to swallow.

BACKGROUND: Systemic lupus erythematosus (SLE) often has a profound negative impact on health-related quality of life (HRQoL). In the absence of any qualitative studies in sub-Saharan Africa, we undertook a study to explore living experiences, perceptions and unmet needs of South African patients with SLE. METHODS: Twenty-five women with SLE consented to participate in the study. They underwent individual in-depth interviews exploring their physical concerns, emotional health, sexual well-being and fertility. NVivo software was used for analysis. RESULTS: Participants were either of black ancestry or mixed racial ancestry, mainly indigent with only a quarter gainfully employed. Living with pain was the most common complaint, negatively impacting on activities of daily living (ADL), family expectations, social life, sleep and intimacy. Most participants expressed challenges of living with fatigue, and many felt their fatigue was misconstrued as being 'simply lazy'. This pernicious fatigue had negative consequences on many facets of ADL, including caring for dependants, job sustainability and sexual well-being. All participants experienced low emotional states, often associated with suicidal ideations. Many experienced difficulties with fertility and childbearing and these were exacerbated in many instances by the pessimism of health care providers, resulting in confusion and depression. Physical disfigurements resulting from lupus-associated alopecia and rashes and corticosteroid-induced weight fluctuations were a major concern. These changes often affected self-image and libido, leading to strained personal relationships. Coping mechanisms that participants adopted included intense spiritual beliefs, 'pushing through the difficult times' and use of alternative therapies to relief symptoms was common. A poor understanding of SLE on the part of participant's family and the community, coupled with the unpredictable course of the disease, exacerbated frustration and social exclusion. For most, limited income, lack of basic services, family dependencies, and comorbid diseases, such as human immune deficiency virus (HIV), exacerbated the daily negative SLE experiences. CONCLUSION: In this study of mainly indigent South African women, SLE is associated with complex, chronic and challenging life experiences. The chronic relapsing and unpredictable nature of the disease, poor understanding and acceptance of SLE, compounded by a background of poverty, inadequate social support structures, negatively impact on a range of personal, social and vocational daily life experiences. Improved access to psychosocial services and SLE education might result in better outcomes. TRIAL REGISTRATION: (Ethics Project identification code: 275/2016 and M160633 registered 10 & 29 August 2016).

Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database.

OBJECTIVE: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. METHOD: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses. RESULTS: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's. CONCLUSION: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice.

Mutations of FAM111B gene are not associated with Systemic Sclerosis.

Systemic sclerosis (SSc) is a prototypic systemic fibrotic disease with unclearly characterized genetic basis. We have discovered that mutations in family with sequence similarity 111, member B (FAM111B) gene cause hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis, a multisystem fibrotic condition with clinical similarities to SSc. This observation has established FAM111B as a candidate gene for SSc. PATIENTS AND METHODS: Demographic and clinical characteristics of consenting adults with definite SSc were recorded. Blood DNA analysis was performed using the High-Resolution Melt technique, and samples with abnormal electropherograms were selected for Sanger sequencing to identify mutations. Ethnically-matched controls from the general South African population were used to verify the frequency of variants in FAM111B. Public databases such as 1000 Genomes and ExAC were also used to verify the frequency of variants in FAM111B. RESULTS: Of 131 patients, 118 (90.1%) were female, and 78 (59.5%) were black Africans. Genetic analysis revealed two FAM111B genetic variants. The c.917 A > G variant (rs200497516) was found in one SSc patients, and one control, and was classified as a missense variant of unknown significance. The c.988 C > T variant (rs35732637) occurred in three SSc patients and 42/243 (17.3%) of healthy controls, and is a known polymorphism. CONCLUSION: One rare variant was found in a patient with SSc but has no functional or structural impact on the FAM111B gene. In this cohort, FAM111B gene mutations are not associated with SSc.

Living with systemic lupus erythematosus in the developing world.

Most of our understanding of SLE and its negative impact originates from developed countries. This review aims to collate existing literature on Health-Related Quality of Life (HRQoL) in SLE patients living in developing countries to identify the gaps for the focus of future research. A narrative literature review was compiled using selected MeSH terms to search EBSCOHOST for articles published between January 1975 and February 2018 pertaining to HRQoL in SLE patients in developing countries. 31 studies from 11 countries were included for analysis. Only one longitudinal, one randomized controlled trial (RCT), one qualitative study, and two intervention studies were found. High disease activity and organ damage were associated with poor functional ability, mental health and low socio-economic status (SES). Poor SES is a recurring theme in developing countries, and worsens all SLE outcomes by reducing access to healthcare, mental, social and emotional support systems. In developing countries, SLE has a globally negative impact on patients' HRQoL, similar to that seen in developed countries. There is an urgent need for more HRQoL studies, and in particular, longitudinal, qualitative and interventional studies in these countries to investigate unmet needs, and to explore novel strategies to improve patient outcomes.

Habitual Physical Activity, Sedentary Behaviour and Bone Health in Rheumatoid Arthritis.

Associations between habitual physical activity levels and bone health in rheumatoid arthritis (RA) were assessed. Twenty nine female patients with RA were assessed for bone mineral density (BMD), and classified as having low or normal hip BMD. Habitual physical activity levels were assessed using accelerometry, and disease activity was assessed using the Clinical Disease Activity Index (CDAI). Twenty one patients had normal bone mass, while 8 had low bone mass. There was no difference in age in the normal bone mass group (51(8)) compared to the low bone mass group (57(12)), p=0.19. Patients with normal bone mass spent on average 2 h less per day in sedentary activity (65(4)% vs. 73(2)%, p<0.01), over 70 min more time in light activity (23(1)% vs. 18(2)%, p<0.01), and over 50 min more in moderate activity per day (12(3)% vs. 8(2)%, p<0.01) than did patients with low bone mass, independently of disease activity or duration. Patients with normal bone mass broke up their sedentary time more frequently per day (72(21) vs. 53(18) times per day, p=0.03). The results of this study indicate that higher habitual activity levels may be protective of bone health in patients with RA, and should be encouraged.

Patterns and predictors of joint damage as assessed by the rheumatoid arthritis articular damage (RAAD) score in South Africans with established rheumatoid arthritis.

This study aimed to determine the pattern and predictors of joint damage measured by the rheumatoid arthritis articular damage (RAAD) score and to describe its relationship to functional disability in patients with established rheumatoid arthritis (RA). One hundred Black patients with RA of disease duration ≥5 years were studied cross-sectionally. Data collected included socio-demographics, disease duration, smoking, body mass index (BMI), extraarticular features, rheumatoid factor (RF), haemoglobin (Hb), disease activity (DAS28), delay in disease-modifying antirheumatic drug initiation (DMARD lag) and treatment history. As outcome measures, the RAAD score and modified Health Assessment Questionnaire (mHAQ-DI) were used to assess joint damage and disability, respectively. Data were analysed by univariate and multivariate analyses. The mean RAAD score was 28.2 ± 12.8 for a mean disease duration of 17.5 ± 8.5 years. The majority of patients still had active disease (mean DAS28 4.4) and severe disability (mean mHAQ-DI 1.9), reflected in part by a long mean DMARD lag (9 years). Wrist and ankle joints were commonly involved. Multivariate analysis revealed that longer disease duration, higher RF titres and lower BMI were significant independent predictors of a higher RAAD score. The mHAQ-DI was significantly associated with DAS28, RAAD, education and Hb. Our results provide support for aspects of validity of the RAAD score and for its use in under-resourced settings. Further longitudinal studies are needed to evaluate its sensitivity to change in monitoring joint damage. Patterns of joint involvement and the inverse relationship between BMI and joint damage also merit further investigation in Black RA patients.

Exaggerated circulating Th-1 cytokine response in early rheumatoid arthritis patients with nodules.

BACKGROUND: Immunohistochemical studies of the rheumatoid nodule (RN) suggest it is a Th1 granuloma, with focal vasculitis, yet the pathogenesis remains unclear and little is known about circulating cytokines in these patients. OBJECTIVE: We studied circulating cytokines in DMARD-naïve RA patients to investigate associations with subcutaneous RN. METHODS: 149 DMARD-naïve adults with early RA (symptom duration ≤ 2 years) were assessed using the Simplified Disease Activity Index (SDAI), and hand and feet radiographs were scored using the modified Larsen method. Circulating cytokines and growth factors representative of T-helper cell 1(Th1) and Th2 cell, macrophages, and fibroblasts were measured using the Bio-Plex® suspension array system. RESULTS: Of 149 patients, 34 (22.8%) had subcutaneous RN, and these patients had more severe disease with higher mean swollen joint counts (p=0.02), SDAI (p=0.04) and modified Larsen scores (p=0.004). There were no differences in Rheumatoid Factor or anti-cyclic citrullinated peptide antibody positivity between patients with RN and those without RN. Patients with RN showed significantly higher levels of circulating IL-12 (p=0.02), IL-2 (p=0.048), and VEGF (p=0.033) levels, with a trend towards higher levels of IL-7 (p=0.056), IFN-γ (p=0.059) and IL-8 (p=0.074) compared to those without RN. CONCLUSIONS: DMARD-naïve early RA patients with RN had more severe disease than those without RN, and showed an exaggerated circulating Th1 and macrophage cytokine profile.

Functional disability and health-related quality of life in South Africans with early rheumatoid arthritis.

BACKGROUND: The severity and predictors of functional disability and health-related quality of life (HRQoL) in a cohort of South Africans with early rheumatoid arthritis (RA) were investigated. METHODS: Changes in the Health Assessment Questionnaire Disability Index (HAQ) and the 36-Item Short Form Health Survey (SF-36) following 12 months of traditional disease-modifying anti-rheumatic drugs (DMARDs) were studied in previously DMARD-naïve adults with disease duration ≤ 2 years. RESULTS: The majority of the 171 patients were female (82%), Black Africans (89%) with a mean (SD) symptom duration of 11.6 (7.0) months. In the 134 patients seen at 12 months, there were significant improvements in the HAQ and all domains of the SF-36 but 92 (69%) still had substantial functional disability (HAQ > 0.5) and 89 (66%) had suboptimal mental health [SF-36 mental composite score (MCS) < 66.6]. Multivariate analysis showed that female sex (p = 0.05) and high baseline HAQ score (p < 0.01) predicted substantial functional disability at 12 months. Unemployment (p = 0.03), high baseline pain (p = 0.02), and HAQ score (p = 0.04) predicted suboptimal mental health, with a trend towards a low level of schooling being significant (p = 0.08). CONCLUSIONS: Early RA has a broad impact on HRQoL in indigent South Africans, with a large proportion of patients still showing substantial functional disability and suboptimal mental health despite 12 months of DMARD therapy. Further research is needed to establish the role of interventions including psychosocial support, rehabilitation programmes, and biological therapy to improve physical function and HRQoL in this population.

Response to traditional disease-modifying anti-rheumatic drugs in indigent South Africans with early rheumatoid arthritis.

The clinical response to traditional disease-modifying anti-rheumatic drugs (DMARDs) in indigent South Africans with early rheumatoid arthritis was investigated. A cohort of patients with early (≤2 years) RA who were DMARD-naïve at inception were prospectively assessed for response to DMARDs using the Simplified Disease Activity Index (SDAI) over a 12-month period. Patients with low disease activity (LDA) at 12 months were compared to those with moderate and high disease activity with respect to demographic, clinical, autoantibody and radiographic features. The 171 patients (140 females) had a mean (SD) age of 47.1 (12.4) years, symptom duration of 11.7 (7.1) months and baseline SDAI of 39.4 (16.2). There was a significant overall improvement in the SDAI and its components in the 134 (78.4%) patients who completed the 12 months visit, but only 28.4% of them achieved LDA. The majority of patients (91%) were treated with methotrexate as monotherapy or in combination with chloroquine and/or sulphasalazine. Baseline features that independently predicted a LDA state at 12 months were lower Health Assessment Questionnaire Disability Index (p = 0.023) and a higher haemoglobin level (p = 0.048). Receiver operating characteristic curve analysis showed that the 6-month SDAI was better than the baseline SDAI in predicting the 12-month SDAI (area under the curve of 0.69 vs. 0.52, respectively, p = 0.008). In conclusion, less than a third of the patients achieved a low disease activity at 12 months on traditional DMARDs. Patients who have an inadequate response to traditional DMARDs at 6 months are unlikely to show further improvement on traditional DMARDs at 12 months. These findings underscore the need for better disease control by an aggressive tight control strategy, including intense patient education and biologic therapy.

Osteoarticular tuberculosis in patients with systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) patients are at increased risk of developing tuberculosis (TB), particularly extrapulmonary TB (ExP-TB). AIM: The present study was undertaken to investigate whether SLE patients showed increased susceptibility to develop osteoarticular TB (OA-TB). DESIGN AND METHODS: We retrospectively reviewed and compared the frequency of ExP-TB, in particular OA-TB, in patients with SLE at a tertiary hospital in South Africa, to a non-SLE control TB group seen at the same hospital. RESULTS: TB was diagnosed 111 times in 97 (17%) of the 568 SLE patients. The relative frequency of ExP-TB in the SLE group (25.2%) was significantly lower than in the control group (38.5%) (OR = 1.9, P = 0.006). In contrast, OA-TB was diagnosed in the SLE group in nine (8.1%) patients (seven with peripheral arthritis and two with TB spine) compared to 54 (0.4%) in the overall control group (OR = 20.8, P < 0.001) and 13 (0.2%) in the subgroup of known HIV positive patients in the control group (OR = 44.4, P < 0.001). Within the SLE group, Black ethnicity (P = 0.003), lymphopaenia (P = 0.001), C3/C4 hypocomplementaemia (P = 0.05), corticosteroids [maximum dose (P = 0.002) and duration of treatment (P = 0.02)] and immunosuppressive agents (P = 0.02) were risk factors for TB. Duration of corticosteroid therapy was the only risk factor for OA-TB (P = 0.04). CONCLUSION: While the relative frequency of ExP-TB was lower in the SLE group compared to the control group, our findings suggest that SLE patients are at particular risk of developing OA-TB. Further prospective studies are needed to better understand the mechanisms that predispose SLE patients to OA-TB.

Causes and predictors of death in South Africans with systemic lupus erythematosus.

OBJECTIVES: Little is known about the long-term outcome and mortality patterns in systemic lupus erythematosus (SLE) in sub-Saharan Africa. We undertook a retrospective study of SLE in mainly black, unemployed patients, seen at a tertiary institution in Soweto, South Africa, to determine the causes and predictors of death. METHODS: Demographic, clinical and laboratory data and outcome were extracted from the case records of patients attending the Lupus Clinic at Chris Hani Baragwanath Hospital. RESULTS: Of the 270 case records with a diagnosis of SLE, 226 met the American College of Rheumatology classification criteria for SLE. The female to male ratio was 18 : 1. The mean (s.d.) age at presentation was 34 (12.5) yrs. Arthritis, nephritis and neuropsychiatric disease had a cumulative frequency of 70.4, 43.8 and 15.9% of patients, respectively. During the course of a mean follow-up period of 54.9 months, 193 (85.3%) and 89 (39.3%) patients were treated with oral corticosteroids and immunosuppressive agents, respectively. There were 55 (24.5%) known deaths and 64 (28.6%) patients were lost to follow-up. The estimated 5 yr survival rates were between 57 and 72%, depending on whether the group of patients lost to follow-up was classified in the analysis as either alive or dead. Infection (32.7%) was the commonest cause of death followed by renal failure (16.4%). Univariate analysis revealed that nephritis, neuropsychiatric disease and hypocomplementaemia were associated with an increased mortality, but multivariate analysis showed nephritis as the only significant predictor of mortality. CONCLUSION: Our findings suggest that SLE in indigent South Africans not only carries a poorer prognosis but also the main cause of death, infection and renal failure differ from those reported recently in industrialized Western countries. Nephritis is common in our patients and is the only independent predictor of poor outcome.

Clinical and serological correlates of antinucleosome antibodies in South Africans with systemic lupus erythematosus.

Antinucleosome antibodies (AnuA) are increasingly recognized as an important biomarker in the diagnosis and subset stratification of patients with systemic lupus erythematosus (SLE). The aim of the study was to determine the sensitivity, specificity, and clinico-serological correlates of AnuA in black South Africans with SLE. We performed a cross-sectional study of 86 SLE patients attending a tertiary center and 87 control subjects. AnuA were tested using a second-generation enzyme-linked immunosorbent assay (ELISA). The sensitivity, specificity, positive predictive value, and negative predictive value of AnuA were 45.3, 94.3, 88.6, and 63.6%, respectively. The presence of AnuA were strongly associated with the co-presence of anti-dsDNA antibodies (OR = 3.4, p < 0.0005) and antihistone antibodies (OR = 15.7, p < 0.00001). Patients who were seropositive for AnuA were more likely to have skin involvement (discoid lupus and/or malar rash) and had higher SLE disease activity index (SLEDAI) scores and Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) damage scores (p < 0.05). IgG anticardiolipin antibody (aCL) levels showed a significant correlation with AnuA ratios (p < 0.01). Our findings provide further evidence that AnuA are a sensitive and specific diagnostic biomarker in SLE. Moreover, our finding that the presence of AnuA, but not anti-dsDNA antibodies, are associated with worse SLICC/ACR damage scores suggest that AnuA may have a role in predicting disease outcome. The correlation between IgG aCL and AnuA is a novel finding that merits further studies to determine possible common peptide specificities of the antibodies.

Prevalence and clinical correlates of anti-phospholipid antibodies in South Africans with systemic lupus erythematosus.

OBJECTIVE: To determine the prevalence and clinical correlates of anti-phospholipid antibodies (aPL), including anti-cardiolipin antibodies (aCL), lupus anti-coagulant (LA), anti-beta2-glycoprotein 1 (abeta2GP1), and anti-prothrombin (aPT) antibodies, in Black South African patients with systemic lupus erythematosus (SLE). METHODS: A cross-sectional study of 100 SLE patients in whom clinical characteristics, including features of the anti-phospholipid syndrome (APS), disease activity, and damage were documented, and sera tested for aCL, abeta2GP, and aPT of all isotypes, and LA. RESULTS: Positive aCL, abeta2GPI, aPT, and LA were found in 53, 84, 20, and 2 patients, respectively. Immunoglobulin (Ig)A aCL and IgG abeta2GPI were the commonest aCL (49.1%) and abeta2GPI (47%) isotypes, respectively. IgA abeta2GPI were associated with both a history of thrombosis alone (p<0.05) and a history of any clinical feature, thrombosis, and/or spontaneous abortion of the APS (p<0.05); IgA aCL were associated with a history of any clinical APS event (p<0.05); and abeta2GPI of any isotype were associated with a history of arthritis (p<0.001). CONCLUSION: Our findings provide further evidence that screening for abeta2GPI and IgA aCL isotypes may improve the risk assessment for APS in SLE patients of African extraction. Further prospective studies are warranted to determine the clinical utility of these tests and to elucidate the genetic basis for the increased IgA aPL response in SLE patients of African extraction.

Human leukocyte antigen class II associations with systemic sclerosis in South Africans.

Human leukocyte antigen class II typing, using polymerase chain reaction-sequence-specific primers, was performed in 52 Black South Africans with systemic sclerosis (SSc) and 112 controls. Increased frequencies of DR2 in the overall SSc group (OR = 2.4), DRB1*0301 in the limited cutaneous SSc (lcSSc) subset (OR = 9.0), and DQB1*0301/4 in the diffuse cutaneous SSc (dcSSc) subset (OR = 9.0) were observed. Pulmonary fibrosis was associated with DRB1*11 and anti-topoisomerase I antibodies were associated with DPB1*1301 and DRB1*15. Patients with anti-fibrillarin antibodies (AFAs) had increased the frequencies of DRB1*1101 allele group (OR = 16) and DQB1*0603/14 (OR = 13.6). These findings provide new serological and immunogenetic data on a previously unreported population. The association of AFAs with class II alleles merits further investigation.

Systemic lupus erythematosus in a patient with human immunodeficiency virus infection--challenges in diagnosis and management.

Systemic lupus erythematosus (SLE) is mainly a disease of young women. The problem of coexistent human immunodeficiency virus (HIV) infection is a growing problem in sub-Saharan Africa. We describe a case of active life-threatening neuropsychiatric lupus in a patient with underlying HIV infection. The challenges of interpreting physical signs and laboratory features that are common to both conditions, and the dilemma of treating active SLE with cytotoxics in the immunocompromised patient, are discussed.