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Patients with systemic lupus erythematosus often assess their disease activity differently from their physicians. We studied the factors associated with this discordance. The data provided by 534 systemic lupus erythematosus patients were analyzed. We compared the physician and patient assessments of lupus activity on a visual-assessment scale from the same visit. We collected clinical data and scores from MOS 36-Item Short-Form Health Survey, Systemic Lupus Erythematosus Quality-of-Life Questionnaire, Rheumatology Attitudes Index, Systemic Lupus Erythematosus Disease Activity Index, and revised Systemic Lupus Activity Measure. Patients tended to score their disease activity higher than do their physicians, when these factors were present: poorer general health assessment, presence of thrombocytopenia, hypertension and urinary sediments, and difficulty in carrying groceries. Physicians tended to score the disease activity higher than do the patients in these circumstances proteinuria, hemolysis, use of azathioprine or cyclophosphamide, tiredness, photosensitivity, higher revised Systemic Lupus Activity Measure score, casturia, and patient report of being more easily ill than are other patients. There was only moderate correlation between the discordance in the baseline and the subsequent visits. The physician assessment of disease activity at baseline correlated better with an objective measure of disease activity (revised Systemic Lupus Activity Measure) in the subsequent visit than the patient assessment. In conclusion, discordance in the perception of disease activity between patients and physicians may be amenable to intervention. [ABSTRACT FROM AUTHOR]

A patient with systemic lupus erythematosus developed pulmonary haemorrhage and pulmonary infarction as rare initial manifestations of her disease. The latter was associated with the presence of the circulating lupus anticoagulant. She recovered with pulse doses of methylprednisolone and plasmapheresis. Anticoagulants were not administered. [ABSTRACT FROM PUBLISHER]

A 50-year-old Chinese woman with a chronic 20-year history of ataxic gait associated with dry eyes and mouth, was admitted to hospital after a single episode of syncope. Magnetic resonance imaging scans showed a large left frontal hypodense lesion suggestive of a glioma. Craniotomy was performed and the lesion excised, with histology showing only infarcted tissue and no malignant cells. Further diagnostic evaluation revealed that the patient had primary Sjogren's syndrome, with demyelinating polyneuropathy. In the absence of risk factors for stroke, it was considered likely that the cerebral infarct was secondary to autoimmune-related vasculitis. Functional neuroimaging, such as magnetic resonance spectroscopy, should be considered in evaluating doubtful or unusual brain lesions in patients with autoimmune disease.

Objective: To examine the relationship between ethnicity and major organ involvement at and after diagnosis in community-based cohorts of Caucasian and Chinese systemic lupus erythematosus (SLE) patients resident in Rochester, Minnesota, and Singapore, respectively., Methods: Clinical manifestations at and after diagnosis were compared in Caucasian and Chinese SLE patients. The association between ethnicity and disease manifestations at and after diagnosis was determined using logistic regression and Cox proportional hazards models, respectively, adjusting for the influence of demographic, socioeconomic, disease-related, and therapy-related factors., Results: At diagnosis, Caucasian SLE patients were 3 times more likely than Chinese SLE patients to have serositis (odds ratio [OR] 3.11, 95% confidence interval [CI] 1.01-9.71), nearly 7 times more likely to have a hematologic disorder (OR 6.95, 95% CI 2.20-21.97), and far less likely to have a malar rash (OR 0.19, 95% CI 0.07-0.54) or positive antinuclear antibodies (OR 0.11, 95% CI 0.03-0.52). Ethnicity was not associated with the prevalence of proteinuria or central nervous system (CSN) and other major organ involvement at diagnosis. After diagnosis, there was a trend toward less development of proteinuria and other major organ involvement in Caucasians (relative risk [RR] 0.47, 95% CI 0.19-1.15, and RR 0.22, 95% CI 0.05-1.04, respectively)., Conclusion: Chinese SLE patients are far less likely to have serositis or a hematologic disorder at diagnosis and may be more likely to develop proteinuria or CNS or other major organ involvement over the course of the disease, compared with Caucasian SLE patients. This may contribute to the increased mortality seen in Chinese SLE patients.

Aim of Study: To determine the spectrum and prevalence of ophthalmic manifestations of systemic lupus erythematosus (SLE) in Asian patients., Methods: We performed a standardised ophthalmic examination on consecutive Asian patients with SLE referred from a tertiary rheumatology unit to an ophthalmology department., Results: Seventy patients were included in the study. There were 66 females (94%) and 4 males (6%). The mean (range) age of the patients was 32.9 (9-67) years. Five patients (7%) had ophthalmic symptoms while 65 (93%) were asymptomatic. Eighty-three eyes of 45 patients had abnormal Schirmer's #1 test and 27 of these eyes of 17 patients also had concomitant rose bengal staining of the cornea and/or conjunctiva. Seventeen eyes of 9 patients had retinal vascular lesions. Fourteen of these eyes had mild microangiopathic retinopathy with best-corrected visual acuity (BCVA) 6/12 or better and 3 had retinal vaso-occlusive disease with BCVA worse than 6/12. Twenty-eight eyes of 14 patients had cataract and 3 eyes of 2 patients had raised intraocular pressure. Twelve eyes of 7 patients had BCVA worse than 6/12 because of optic neuropathy (4 eyes), posterior subscapsular cataract (4 eyes), retinal vaso-occlusive disease (3 eyes) and phthisis bulbi (1 eye). None had any eyelid lesion, extraocular motility disorder or retrochiasmal disorder of vision., Conclusions: Asymptomatic dry eye is the most common ocular finding in patients with SLE. Sight-threatening complications of SLE include retinal vaso-occlusive disease and optic neuropathy.

Objective: The Health Assessment Questionnaire - Disability Index (HAQ), used as a disability and outcome measurement in rheumatoid arthritis (RA), has been validated in several languages, but not in Chinese. Our aim was to validate the Chinese version of HAQ (Chinese-HAQ) to suit the needs of Chinese speaking patients with RA in an Asian setting., Methods: The original HAQ was modified in the context of Chinese culture and translated into Chinese by 2 translators aware of the objective of the questionnaire. The Chinese HAQ was self-administered by 42 patients with RA during their routine followup visit and one week later., Results: The test-retest reliability assessed using Spearman's correlation coefficient was 0.84. Between dimensions measured in the HAQ, the highest test-retest reliability was observed for walking (Spearman correlation coefficient rs=0.80) and the lowest was for eating (rs=0.54). The internal consistency of the scale using Cronbach's alpha was high at 0.86. In terms of criterion validity, the Chinese-HAQ score was found to correlate well with American College of Rheumatology functional status (rs=0.501, p=0.01). The Chinese-HAQ scores also correlated well with markers of disease activity such as patient's perception of pain measured on a visual analog scale (rs=0.55, p < 0.001), grip strength in mm Hg (rs=-0.55. p < 0.001 ), and physician's assessment of disease activity (rs=0.59, p < 0.001)., Conclusion: The Chinese HAQ is a reliable and valid instrument for studies measuring disability of patients with RA in Singapore.

Objective: To determine the effects of ethnicity on disease manifestations in Oriental patients with systemic lupus erythematosus (SLE) and to describe the risk of developing renal or central nervous system (CNS) involvement with time., Methods: A retrospective study of 472 patients with SLE seen at the only Rheumatology Unit in Singapore. The effect of ethnicity on selected disease manifestations at diagnosis was assessed after adjusting for demographic variables using multiple logistic regression. The probability of developing selected disease manifestations with time was determined using the Kaplan-Meier product limit method., Results: At diagnosis, Malays had a higher risk of renal or CNS involvement than Chinese (OR 2.26, 95% CI 1.21 to 4.21, and OR 3.07, 95% CI 1.01 to 9.34, respectively), and Indians a lower risk of malar rash and a higher risk of oral ulcers than Chinese (OR 0.30, 95% CI 0.13 to 0.68, and OR 2.90, 95% CI 1.45 to 7.34, respectively). The prevalence of renal or CNS involvement in the entire cohort increased with time, reaching 75.6% (95% CI 66.1% to 85.0%) and 16.7% (95% CI 11.7% to 21.6%), respectively, after 18 years of disease., Conclusion: Ethnicity influenced disease manifestations at diagnosis in this cohort of Oriental patients with SLE. Renal or CNS involvement developed in previously unaffected patients up to 18 years after diagnosis, highlighting the need for continued vigilance in patients with lupus.

Rheumatoid arthritis, a common chronic inflammatory arthritis, tends to run a more benign course in patients from the community than those seen at hospitals. The aetiology is unknown but the disease is postulated to result from the interaction between genetic and environmental factors. The strongest genetic association is with the major histocompatibility complex (MHC) Class II antigen HLA DR 4, although not more than 50% of the genetic susceptibility is due to MHC genes. In early disease the pathogenesis is thought to be T cell mediated whereas in late disease the mechanisms are T cell independent, with destruction contributed to by autonomous fibroblast like synoviocytes. The diagnosis is made on clinical groups. Management is directed at controlling active synovitis so that joint damage is limited. The early use of a slow acting anti-rheumatic drug is now advocated, as studies have shown that they can potentially prevent or limit the progression of disease. Biological therapies are still experimental but may hold promise for the future.

We identified clinical predictors of lupus nephritis presenting more than 6 months after the diagnosis of lupus in a cohort of 335 lupus patients. Almost 24% (80/335) of patients developed nephritis more than 6 months after the diagnosis of lupus. Using a Cox proportional hazards model, we found hypertension, thrombocytopaenia and leukopaenia to be associated with lupus nephritis presenting more than 6 months after diagnosis, with adjusted relative risks of 2.5 (95% CI 1.3 to 4.7), 4.3 (95% CI 1.7 to 10.8) and 3.2 (95% CI 1.7 to 6.2) respectively. In this cohort, hypertension, thrombocytopaenia and leukopaenia were associated with lupus nephritis presenting more than 6 months after the diagnosis of lupus.

Vibrio vulnificus is an emerging opportunistic pathogen that is responsible for one of the most fulminant food-borne diseases in immunocompromised hosts. The infection should be suspected in patients with underlying liver disorders who present with a prominent skin rash, hypotension and septic parameters especially if they give a history of seafood or shellfish ingestion. Awareness and early recognition of this infection will result in a better outcome for the patient.

We studied the initial manifestations and late features in our lupus patients. The clinical data of patients fulfilling the American College of Rheumatology criteria for systemic lupus erythematosus (SLE) were entered prospectively for newly diagnosed patients and cumulatively for those with at least 10 years of disease duration. Ninety-seven Group A (newly diagnosed; 86 females and 11 males; mean age 31 years; 83 Chinese, 11 Malays, and 3 Indians) and 58 Group B (more than 10 years disease duration; 56 females and 2 males; mean age 41 years; 50 Chinese, 5 Malays, and 3 Indians) lupus patients were studied. The commonest clinical features in Group A were: haematological (73%), arthritis (57%), malar rash (43%), renal disorder (31%) and photosensitivity (30%). Group B patients had haematological (78%), malar rash (73%), arthritis (69%), renal disorder (59%) and photosensitivity (33%). Renal disorder was significantly increased over the years (P < 0.001). Hypertension was present in 18% (Group A) and 59% (Group B) (P < 0.00001), diabetes mellitus in 5% (Group A) and 10% (Group B) (P = ns), atherosclerosis in 2% (Group A) and 7% (Group B) (P = ns). Cataract formation was not present in Group A patients but was present in 10% of Group B patients. Renal disorders and morbidity factors like hypertension and cataracts increased significantly over the years. Optimum treatment of lupus patients should therefore include close attention to these factors.

We describe a 27y old female systemic lupus erythematosus (SLE) patient with salmonella bacteraemia who presented with fever, back pain and an enlarging heart size. A two dimensional echocardiogram (2D Echo) showed a mass in the right atrium. Subsequent computer tomographic (CT) and magnetic resonance imaging (MRI) studies showed that this had become a ring shaped lesion at the posterior end of the interventricular septum with an area communicating with the right atrial cavity. At operation a ruptured mycotic aneurysm of the right coronary artery was found. This is the first report of an SLE patient with a coronary artery mycotic aneurysm due to salmonella and the first reported case of survival following rupture of such aneurysm.

A single-base change in the regulatory region of the tumour necrosis factor alpha (TNF-alpha) gene and the TNF2 allele said to be associated with Caucasian lupus patients has been reported. We studied this genetic polymorphism in 89 normal individuals and 67 Chinese lupus patients, 45% of whom has renal involvement. The allelic frequencies of TNF1 and TNF2 alleles in our male controls (n = 49) were 0.86 and 0.14 respectively, in the female controls (n = 40) they were 0.91 and 0.09 respectively, and as a combined group they were 0.88 and 0.12 respectively. The corresponding frequencies in our lupus patients were 0.81 and 0.19 respectively. Comparing the allelic frequencies of the combined control and patient group as well as only between the female control and female lupus patients, we did not find any association between the TNF2 allele and systemic lupus erythematosus in our cohort of Chinese patients. The TNF2 gene may be in linkage disequilibrium with the DR3 allele.

Joint fluid findings in systemic lupus erythematosus (SLE) have been described in only a few series and systematic electron microscopic study of the synovial fluid (SF) cells has not been reported. We describe the evaluation of 17 SF in patients with SLE with routine analysis and electron microscopy. Joint effusions had a wider range of leukocyte counts than often appreciated, with counts varying from 875 to 39,250 cells per mm3. LE inclusions were seen in eight fluids and have been shown to contain chromatin-like filaments by electron microscopy. There was little associated electron dense immunoglobulin-like material. Tubuloreticular structures (TRS) found in seven SF were mostly in mononuclear cells including some LE cells. The known association of TRS with alpha interferon and viral infections may have important implications for pathogenesis.

The clinical characteristics of 38 local Chinese ankylosing spondylitis patients were studied by interview, clinical examination and review of casenotes. The sex ratio was 3.2:1 in favour of males. The average duration from onset of symptoms till diagnosis of disease was 7.25 years. Peripheral joint involvement occurred in 71% of the patients. Extra-articular complications were uncommon; only three patients had a history of uveitis and one patient had biopsy proven IgA nephropathy. 26.3% of patients had significant disability due to the disease. Early diagnosis of the disease should be made as it may improve the prognosis of the patients.

The HLA-A, B, C, DR and DQ antigens were determined in 50 Singapore Chinese patients with rheumatoid arthritis (RA). There was a significant increase in the prevalence of HLA-Bw46, DRw53 and DQ3 in patients with RA. The linkage disequilibrium between Bw46 and DRw53 explains this association. This major histocompatibility complex association differs from the HLA-DR4 link in Caucasian populations and suggests that RA is an immunogenetically heterogeneous disease.

39 patients who received pulse methylprednisolone for disease manifestations of systemic lupus erythematosus were studied for zero to twenty-four weeks following therapy. Pulse methylprednisolone was given as intravenous infusions of methylprednisolone (10 mg/kg body weight) over one hour each day for three consecutive days. 27 (69.2%) patients were treated for lupus nephritis, 12 (30.8%) patients for non-renal manifestations of lupus. 17 (63.0%) of the renal lupus patients and 7 (58.3%) of the non-renal lupus patients showed clinical response. 11 (28.2%) patients had infections from which 7 (63.6%) died. Overall, 15 (38.5%) patients died. Early deaths (occurring within the first two weeks) were mainly due to disease activity while later deaths were mainly due to infection. In conclusion, the majority of lupus patients appeared to have had a beneficial response to pulse methylprednisolone therapy.

The clinico-pathological features of 5 patients with malignant histiocytosis aged 18 years to 73 years seen over a three year period from 1980 to 1983 at the Department of Haematology Singapore General Hospital were studied. Haematological, cytological and histopathological findings of the disease are described. Diagnosis of malignant histiocytosis was established from biopsies of bone marrow, liver, spleen or lymph nodes. Three out of the 5 patients died rapidly after diagnosis despite cytotoxic chemotherapy. One patient was lost to follow up while another is alive surviving so far for 3 months since diagnosis. The differential diagnosis of malignant histiocytosis is also discussed.

Autoantibodies to the three extractable nuclear antigens (ENA), Anti-SSA (Ro), Anti-Sm, Anti-RNP and antinuclear antibodies were determined in 150 patients with SLE. Seventy patients (46.7%) had Anti-SSA (Ro), 40 (26.7%) Anti-Sm and 25 (16.7%) Anti-RNP antibodies. Ninety four percent patients had a positive Fluorescent anti-nuclear antibody (FANA) test. The commonest FANA pattern is the speckled pattern. Subclinical keratoconjunctivitis sicca (KCS) was present in 60% patients. No correlation could be demonstrated between the presence of ENA autoantibodies and the clinical features of patients.

20 patients with moderately severe bacterial infections were studied to determine the clinical efficacy and safety of parenteral sulbactam/ampicillin. There were 9 female and 11 male patients. Their mean age was 51 years. 8 patients had pneumonia, 5 urinary tract infection, 4 cellulitis of the leg and 3 had pustular tonsillitis. 85% of patients had resolution of fever and symptoms within 48 hours of commencing treatment. 95% had successful treatment outcome. The organisms isolated included E. Coli, Klebsiella sp, Branhamella catarrhalis and Bacillus species. In 2 patients, the organisms isolated demonstrated in-vitro ampicillin resistance. However, they recovered fully with sulbactam/ampicillin therapy. No adverse side-effects were reported and dosage adjustment was not required in the elderly.