Performance Evaluation and Clinical Associations of Immunoassays That Detect Antibodies to Negatively Charged Phospholipids Other Than Cardiolipin.

OBJECTIVES: We evaluate the performance characteristics of antiphosphatidylserine (anti-PS), antiphosphatidylinositol (anti-PI), and antiphospholipid mixture (APhL) enzyme-linked immunosorbent assays (ELISAs) compared with anticardiolipin (aCL) and anti-ß2 glycoprotein I (anti-ß2GPI) in a large group of patients with antiphospholipid (aPL)-related diseases. METHODS: Serum samples from 548 patients from the Hopkins and Jamaican systemic lupus erythematosus cohorts, the PROMISSE cohort, and the Antiphospholipid Standardization Laboratory were examined for immunoglobulin G (IgG)/immunoglobulin M (IgM) positivity in aCL, anti-ß2GPI, anti-PS, anti-PI, and APhL ELISA assays. RESULTS: All IgG assays were associated with one or more thrombotic and/or obstetric manifestations, with an increased risk associated with higher antibody titers. Analytical performance was similar among assays, but IgG assays performed better than IgM counterparts. CONCLUSIONS: Increasing titers of APhL, anti-PS, and anti-PI antibodies could indicate an increased risk of thrombotic and/or obstetric aPL-related manifestations. These assays may be promising biomarkers for particular APS manifestations.

HLA-DRB alleles and systemic lupus erythematosus in Jamaicans.

BACKGROUND: The human leukocyte antigens (HLA) are associated with susceptibility to systemic lupus erythematosus (SLE) and manifestations of SLE in different ethnic groups. METHODS: A DNA-based HLA-typing method was used to determine alleles of HLA-DRB1, DRB3, DRB4 and DRB5 in Jamaican patients. A total of 70 patients and 100 control subjects were studied. RESULTS: HLA-DRB3*01/03 was significantly associated with susceptibility to SLE, while DRB1*15/16 was associated with the presence of oral ulcers in patients with SLE. The haplotype DRB1*13/14.DRB3*01/03 was also more frequent in SLE patients. No other significant associations were found. CONCLUSION: The SLE HLA associations in Jamaicans differ from those in other black populations.

Abnormal haemorrheological changes in systematic lupus erythematosus (SLE) [abstract]

Vascular complications are frequently associated with SLE. It is hypothesized that haemorrheological abnormalities may play a role in the aetiology of micro-and-macrocirculatory disorders in SLE. The range of rheological abnormality was determined by measuring the concentration of plasma fibrinogen, serum albumin and relating these abnormalities to change in relative plasma viscosity (RPV) and relative serum viscosity (RSV) in 21 SLE patients. The SLE disease activity index (SLEDAI) was used to define the severity of the disease. Eleven patients were "severe" (SLEDAI score> 12) and ten patients were "limited" (SLEDAI score< 12). RPV and RSV were measured by capillary viscometry. Plasma fibrinogen was determined by a clot-weight procedure and serum albumin and globulin by the Biuret method. Results from the SLE patients were compared with those from a randomly selected control group. A significant increase in RPV (p< 0.001) and RSV (p< 0.05) was recorded for the SLE patients. The patients with severe disease had a significant (p<0.001) increase in RSV but neither in fibrinogen nor RPV as compared with patients with limited disease activity. The increased viscosity in SLE patients may constitute an impediment in blood flow. Furthermore, the higher serum viscosity in patients with severe disease suggests that serum viscosity may provide a useful marker for disease activity. (AU)

Photosensitivity and antinuclear antibodies in black patients with systemic lupus erythematosus

Black patients with systemic lupus erythematosus (SLE) have a lower prevalence of photosensitivity rashes than white patients. The reasons for this are unkown, but some studies suggest a correlation between the presence of antinuclear antibodies and protection from phosensitivity. In our study, we determined serum antinuclear-antibody profiles, including anti-dsDNA, anti-Sm, anti-RNP, anti-Ro/SS-A, anti-La/SS-B antibodies, in 91 black Jamaican patients with SLE. All 91 serum samples from SLE patients (100 percent) were positive in the fluorescent antinuclear-antibody test. Using the crithidia luciliae immunofluorescence test, anti-dsDNA was found in 27.5 percent of the samples. By a double immunodiffusion method, anti-Sm antibodies were found in 15.4 percent, and anti-RNP in 18.7 percent, anti-Ro/SS-A in 9.9 percent and anti-La/SS-B in 11.0 percent. However, no statistically significant differences were observed in the seroprevalence of these antinuclear antibodies when sera from patients of the following groups were compared: only photosensitivity rashes (n = 17), photosensitivity and other rashes (n = 23), other rashes without photosensitivity (n = 27), and patients with no skin rash of any type (n = 24). These results suggest that photosensitivity in black Jamaican patients with SLE is not associated with antinuclear-antibody specificity(AU)

Asymptomatic cardiac involvement in systemic lupus erythematosus

Cardiologic and laboratory parameters were studied in 21 patients with systemic lupus erythematosus (SLE) with cadiopulmonary symptoms (CPS), 20 SLE patients without CPS and 45 age-and sex- matched healthy controls. The most frequent cardiac abnormalities in patients with CPS included pericardial effusion (24 percent), ventricular enlargement (20 percent), mitral regurgitation (19 percent) and tricuspid regurgitation (14 percent). No structural abnormalities were observed in SLE patients without CPS. Mean calculated and derived echocardiacgraphic values in both groups of SLE patients differed significantly from those observed in normal controls (p< 0.004). Patients with CPS had significantly lower mean values of ejection fraction (p< 0.05) and fractional shortening (p< 0.03). However, the frequencies of functional abnormalities in patients with CPS did not differ significantly from those observed in patients without CPS. There were no remarkable laboratory findings in SLE patients with CPS compared to those without. The finding that some SLE patients may have functional cardiac abnormalities in the absence of CPS is an important one. It raises the question as to whether asymptomatic cardiac involvement in SLE is a separate entity or whether it heralds symptomatic cardiopulmonary involvement (AU)

Systemic lupus erythematosus, rheumatoid arthritis and HLA phenotypes in Jamaicans

The HLA phenotypes were investigated in 30 Jamaican patients with systemic lupus erythematosus (SLE), 30 with rheumatoid arthritis (RA) an d forty healthy controls. HLA phenotypes were determined by the microcytotoxicity technique, using commercially prepared typing trays. In this study, the HLA phenotypic associations with SLE (HLA-B14, RR 4.3: HLA-A28, RR 4.3) were not statiscally significant. However, a statistically significant lack of HLA-A9 (p<0.01;CP<0.1) was observed in SLE patients compared to healthy controls. In RA patients, a statistically significant associations was noted with HLA-A2 (RR5.1; CP<0.01). No HLA class 11 associations were noted with SLE. Class 11 associations with RA did not achieve statistical significance but included those previously established in other populations. The preliminary data obtained from this study indicate differences in the patterns of HLA phenotypes in Jamaican patients with SLE and RA compared to those observed in such patients elsewhere. Further studies involving larger groups of patients and typing at the serological, cellular and molecular levels are clearly warranted (AU)

Systemic lupus erythematosus, rheumatoid arthritis and HLA phenotypes in Jamaicans

The HLA phenotypes were investigated in 30 Jamaican patients with systemic lupus erythematosus (SLE), 30 with rheumatoid arthritis (RA) an d forty healthy controls. HLA phenotypes were determined by the microcytotoxicity technique, using commercially prepared typing trays. In this study, the HLA phenotypic associations with SLE (HLA-B14, RR 4.3: HLA-A28, RR 4.3) were not statiscally significant. However, a statistically significant lack of HLA-A9 (p<0.01;CP<0.1) was observed in SLE patients compared to healthy controls. In RA patients, a statistically significant associations was noted with HLA-A2 (RR5.1; CP<0.01). No HLA class 11 associations were noted with SLE. Class 11 associations with RA did not achieve statistical significance but included those previously established in other populations. The preliminary data obtained from this study indicate differences in the patterns of HLA phenotypes in Jamaican patients with SLE and RA compared to those observed in such patients elsewhere. Further studies involving larger groups of patients and typing at the serological, cellular and molecular levels are clearly warranted

Asymptomatic cardiac involvement in systemic lupus erythematosus

Cardiologic and laboratory parameters were studied in 21 patients with systemic lupus erythematosus (SLE) with cadiopulmonary symptoms (CPS), 20 SLE patients without CPS and 45 age-and sex- matched healthy controls. The most frequent cardiac abnormalities in patients with CPS included pericardial effusion (24 per cent), ventricular enlargement (20 per cent), mitral regurgitation (19 per cent) and tricuspid regurgitation (14 per cent). No structural abnormalities were observed in SLE patients without CPS. Mean calculated and derived echocardiacgraphic values in both groups of SLE patients differed significantly from those observed in normal controls (p< 0.004). Patients with CPS had significantly lower mean values of ejection fraction (p< 0.05) and fractional shortening (p< 0.03). However, the frequencies of functional abnormalities in patients with CPS did not differ significantly from those observed in patients without CPS. There were no remarkable laboratory findings in SLE patients with CPS compared to those without. The finding that some SLE patients may have functional cardiac abnormalities in the absence of CPS is an important one. It raises the question as to whether asymptomatic cardiac involvement in SLE is a separate entity or whether it heralds symptomatic cardiopulmonary involvement

The painful crisis of homozygous sickle cell disease: clinical features

The details of onset, perceived precipitating factors, associated symptoms, and pain distribution in the painful crisis of homozygous sickle cell (SS) disease have been prospectively recorded in 183 painful crises in 118 patients admitted to a day-care centre in Kingston, Jamaica. Painful crises developed most frequently between 3 p.m. and midnight, most commonly affected patients aged 15-29 years, affected the sexes equally, and were not obviously influenced by menstrual cycle. Of the perceived precipitating factors, skin cooling occurred in 34 percent, emotional stress in 10 percent, physical exertion in 7 percent, and pregnancy in 5 percent of women of child-bearing age. Cold as a precipitant was not less common in patients with more subcutaneous fat. Pain affected the lumbar spine in 49 percent, abdomen in 32 percent, femoral shaft in 30 percent, and knees in 21 percent. There was a highly significant excess of bilateral involvement in limb and rib pain. Recurrent painful crises occurred in 40 patients but showed no evidence of involving similar sites on successive occasions. Abdominal painful crises were associated with abdominal distention in 18 ( 31 percent) and with referred rib pain in a further 15 (26 percent) of crises. Fever was common even in apparently uncomplicated painful crises, suggesting that fever is characteristic of the painful crisis itself after and not necessarily indicative of infection. Following investigation and treatment in a day-care centre, over 90 percent of patients returned home (AU)

Primary antiphospholipid syndrome: a case report

A case of the primary antiphospholipid syndrome (PAPS) in a 21-year-old Jamaican female is described. Recurrent abortions, thrombocytopenia and neurological complications as well as lupus anticoagulant positivity in the absence of features of systemic lupus erythematosus (SLE) were the main clinical findings. Diagnostic criteria, treatment and prognosis are discussed. When the antiphospholipid syndrome (APS) is present in the primary form, the diagnosis may be difficult but its recognition may prevent those vascular events which can lead to significant morbidity and foetal wastage (AU)

Primary antiphospholipid syndrome: a case report

A case of the primary antiphospholipid syndrome (PAPS) in a 21-year-old Jamaican female is described. Recurrent abortions, thrombocytopenia and neurological complications as well as lupus anticoagulant positivity in the absence of features of systemic lupus erythematosus (SLE) were the main clinical findings. Diagnostic criteria, treatment and prognosis are discussed. When the antiphospholipid syndrome (APS) is present in the primary form, the diagnosis may be difficult but its recognition may prevent those vascular events which can lead to significant morbidity and foetal wastage.

The heart and systemic lupus erythematosus - abstract

A prospective clinical, serological and echocardiographic study of consecutive patients with systemic lupus erythematosus (SLE) and cardiopulmonary symptoms (Group 1) and 10 asymptomatic SLE patients (Group 11) was carried out. Both groups were then subsequently compared to 45, age and sex matched controls taken from the general population. The most common cardiac manifestation of SLE was pericardial effusion (5 patients; 24 percent). Other echocardiographic findings were: enlarged left ventricle (3 patients; 14 percent), large right ventricle (2 patients; 10 percent) myocarditis (2 patients; 10 percent), Libman Sacks endocarditis (1 patient; 5 percent). Doppler studies showed a 19 percent incidence of mitral regurgitation (4 patients), 14 percent tricuspic regurgitation (3 patients), 10 percent pulmonary hypertension (2 patients). Echocardiographic measurements in Groups 1 and 11 showed no statistically significant difference in left ventricular diastolic (LVDD) and systolic (LSVD) dimensions, and in end diastolic (EDV) and end sytolic volumes (ESV). There was increased heart rate and decreased stroke volume and cardiac index in Group 1. Indeed, there was no echocardiographic abnormality in Group 11. When both groups were compared to Group 111, there was stastically significantly lower ejection fraction and fractional shortening, greater septal and posterior wall thickness and smaller right ventricular diastolic dimension (RVDD) in the combined SLE group. The left ventricular mass in the SLE group was not increased but there were decreased stroke output and systolic function compared to the normal controls. Only one patient was positive for antiphospholipid antibody and she was in group 1. All SLE patients were antinuclear antigen positive (ANA) and antidouble stranded DNA antibodies were the most frequently found ANA in both groups 1 and 11. There was no statiscally significant difference in the prevalence of any ANA specificity between groups or between those patients with valvular abnormality and those without. Echocardiography was a more sensitive indicator of pericarditis/pericardial effusion than chest roentgenogram or electrocardiogram (AU)

The haematology of homozygous sickle-cell disease - abstract

The purpose of the study was to investigate age- and sex-related variations in the haematology of older patients with SS disease, in order to determine haematological characteristics possibly favouring survival. Steady state haematology was available in 181 patients aged 40-73 years. There appeared to be no consistent sex differences in any of the indices examined. Longitudinal analyses were performed for the 133 patients with at least two observations, using analysis of covariance (ANCOVA) methods. Highly significant declines in total haemoglobin (Hb), platelet counts and absolute reticulocyte count were displayed in both sexes. Overall, Hb levels decreased by approximately 0.076 gm/dl/year in females and 0.113 gm/dl/year in males. Significant increases occurred in HbA, HbF and MCV in females only. The total nucleated count (NBC) fell with age, although the decline was only significant in females. These observations are consistent with a progressive bone marrow failure which is not explained by the commonly occurring renal impairment in older SS patients since the changes persisted in analyses confined to the 84 patients with normal creatinine levels (C=120 æmol/l). The mechanism for this bone marrow failure is currently unknown. The prevalence of homozygous alpha thalassaemia in the study group (4.4 percent) was similar to that in the overall SS population, providing no evidence that this may lead to improved survival, as has been suggested (AU)

Infective arthritis due to Blastocystis hominis

A patient with rheumatoid arthritis taking prednisone developed Blastocystis hominis acute diarrhoea, which was associated with increased inflammation and effusion of the left knee. B. hominis organisms were found in synovial fluid from the left knee. The patient responded dramatically to metronidazole treatment. B. hominis may become disseminated in immunosuppressed patients with diarrhoea and may cause infective arthritis. (AU)

Arthritis in childhood: a report on 65 consecutive cases observed at the University Hospital of the West Indies

In a two-year period (October 1985 - September 1987), 65 children presented to the Child Health Department and/or rheumatology clinic at the University Hospital with arthritis. Eighteen children (28 percent) had juvenile chronic arthritis, ten (15 percent) rheumatic fever, eight (12 percent) systemic lupus erythematosus and thirteen (20 percent) had self-limiting arthritis. Systemic onset of juvenile chronic arthritis occurred only in one child; nine children has polyarticular and eight pauci-articular onset of disease. The self-limiting arthritis was difficult to differentiate from juvenile chronic arthritis; therefore serological testing for bacterial and viral infections should be performed before anti-rheumatic therapy is undertaken. Arthritis in childhood is not uncommon in Jamaica. However, the outcome appears to be generally favourable except in a few cases of juvenile chronic arthritis (AU)

Mortality of Jamaican patients with systematic lupus erythematosus

A retrospective study of all patients with systemic lupus erythematosus (SLE) who died at the University Hospital of the West Indies over a 14-year period is presented. The major cause of death was infection followed by renal failure. Gram-negative organisms were the major microbiological agents causing infections. Side-effects of therapy were common, in particular bone marrow depression and haemorrhage related to anticoagulants. It appears that controlling severe lupus activity without increasing the risk of life-threatening complications remains an important goal in the treatment of SLE.(AU)

Arthritis in Jamaica - abstract

Presented are data on 241 patients who attended the Rheumatology Clinic U.H.W.I. over a 5-month period. Two hundred and eight (86 percent) of the patients were female. The ages ranged from 7 to 87 years (mean 41 years). Seventy-eight (32 percent) had rheumatoid arthritis. Their mean age was 47 years (range 18-84 years). Only 2 patients were male. Twenty-one (27 percent) were negative for the rheumatoid factor, and 7 (9 percent) had anti-nuclear antibodies. Twenty-seven patients (11 percent), all female, were diagnosed as having systemic lupus erythematosus. Their mean age was 30 years (range 10-50 years). Two patients were consistently negative for anti-nuclear antibodies. Other diseases frequently diagnosed were: degenerative joint disease in 26, juvenile rheumatoid arthritis in 12, mixed connective tissue disease in 8, spondylitis in 7. Forty-one patients were seen with arthralgia and/or myalgia without evidence of an inflammatory process (AU)