Thirty-six patients with lupus nephritis (LN) attending the Nephrology Clinic, Hospital Kuala Lumpur were studied for the prevalence of anticardiolipin antibody (ACA) isotypes (IgG and IgM) and other associated antibodies, antinuclear antibody (ANA) and anti-ds DNA antibody and to determine the possible association between serological and clinical parameters. The study population consisted of 20 (55.6%) Malays, 15 (41.7%) Chinese and 1 (2.8%) Indian with a mean age of 31.4 +/- 11.3 years, range 14 to 60 years. The female to male ratio was 11:1. The average time between diagnosis and blood sampling was 4.4 years (range 0.25 to 15 years). Increased ACA levels were found in 20 (55.6%) patients where raised IgG ACA and IgM ACA were observed in 20 (55.6%) and 2 (5.6%) cases respectively. ANA and anti-ds DNA antibodies were detected in 22 (61.1%) and 4 (11.1%) individuals respectively, with the majority (82%) showing a speckled pattern of nuclear staining. However, neither the IgM ACA nor IgG ACA showed any significant association with thrombosis or any other clinical parametres. Our preliminary study indicates that ACA is a frequent finding in lupus nephritis and that the IgG isotype is more prevalent.
Study site: nephrology Clinic, Hospital Kuala Lumpu
From 1981 to 1989, 12 patients of the University Hospital, Kuala Lumpur, were diagnosed to have Evans syndrome based on direct antiglobulin test (DAT) positive haemolytic anaemia and immune thrombocytopenia occurring either simultaneously (7 patients) or consecutively (5 patients). Their mean age at presentation was 24.8 years with a marked female preponderance. All 12 patients were given high dose steroid after diagnosis. Subsequently, other modalities including intravenous immunoglobulin (1 patient) and high dose methylprednisolone (1 patient) were given. Three patients died of intracranial haemorrhage during the first admission while 1 patient died of pulmonary embolism six months after diagnosis. Three patients had splenectomy because of thrombocytopenia. Six patients tested positive for antinuclear factor and antibodies to double stranded DNA and four of them died. Positive serology appeared to be associated with a poorer prognosis. Follow up observations indicate that patients who survive the acute attacks fare reasonably well.
Systemic lupus erythematosus (SLE) is highly prevalent in Malaysia, which has a mixed population of Malays, Chinese, and Indians. A quantitative enzyme linked immunosorbent assay (ELISA) was used to determine anticardiolipin antibody (aCL) levels (total immunoglobulin, IgG, and IgM) in 200 patients with SLE (164 Chinese, 26 Malay, and 10 Indian) attending the University Hospital of Kuala Lumpur, Malaysia, and 103 matched controls. Only 33 (16.5%) of the patients had raised aCL levels; 26 had raised IgG aCL, five IgM aCL, and two both IgG and IgM aCL. There was a low prevalence of raised levels of aCL in the population studied, which was seen in conjunction with a rare occurrence of thrombosis. The classical association of high aCL levels with thrombocytopenia and recurrent abortions was noted, though not with cerebral disease. The low prevalence of aCL in this study population of mixed racial origin contrasts with findings in European patients with SLE and lends support to the influence of local factors, be they genetic or environmental, on the clinical manifestations of this disease.
Patients with autoimmune connective tissue disease may manifest as overlap syndrome with features of systemic lupus erythematosus (SLE), systemic sclerosis, rheumatoid arthritis and myositis. Those presenting with active SLE can present with immune thrombocytopenia (IT) and may be complicated with subdural hematoma which, though rare, is potentially life-threatening. We report here a patient with overlap syndrome who had recurrent spontaneous subdural hematoma due to severe thrombocytopenia which did not respond to corticosteroids and azathioprine. Her platelet count became normal with three doses of low-dose intravenous cyclophosphamide (IV CYC) given at 3-weekly intervals. She remained in remission with maintenance therapy with azathioprine.