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PubMed1981

Noninflammatory renal microangiopathy of systemic lupus erythematosus ('lupus vasculitis').

5 (group 1) of 9 patients with systemic lupus erythematosus (SLE) who underwent a kidney biopsy during a 1-year period had a noninflammatory renal microangiopathy which significantly narrowed or occluded the lumens of arterioles and terminal segments of interlobular arteries. Though these lesions resemble those of malignant hypertension and the thrombotic microangiopathies, their distinctive features, revealed by light, immunofluorescence and electron microscopy, differentiate them from the others. 4 of these 5 (group 1) patients developed hypertension; in 3 the microangiopathy predated the hypertension. None of the remaining 4 (group 2) patients without these lesions has become hypertensive. One group-1 patient with extensive lesions involving 40% of intrarenal arterioles at biopsy developed renal failure shortly thereafter with increasing arteriolar involvement but unchanged glomerular histology.

Adult↗
PubMed1995

Bullous systemic lupus erythematosus: revised criteria for diagnosis.

Blistering in systemic lupus erythematosus has been divided into three groups. A specific subgroup of 'bullous systemic lupus erythematosus' has been defined by Gammon et al. on the basis of a number of criteria. From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistological (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride-split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VII collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.

Adult↗
PubMed1995

Proton magnetic resonance spectroscopy of systemic lupus erythematosus involving the central nervous system.

We examined 13 patients with neurological manifestations of systemic lupus erythematosus (SLE) based on previous and/or current neurological or psychotic episodes by magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (MRS) together with psychiatric and cognitive assessment. MRI was abnormal in 7 patients, showing high signal lesions in the white matter and/or cerebral atrophy. Proton MRS centred on white matter lesions in 5 patients showed a reduction in the N-acetyl aspartate creatine ratio compared with normal appearing white matter in the SLE group and in 10 healthy controls. This pattern of abnormality does not allow differentiation of SLE lesions from the chronic plaques occurring in multiple sclerosis. There was a very high incidence of current psychiatric morbidity in the SLE group, namely in 12 of the 13 patients. There was no correlation between the presence of current psychiatric involvement and/or cognitive dysfunction and abnormalities detected with MRI or MRS.

Adult↗
PubMed1996

Central serous chorioretinopathy in patients with systemic lupus erythematosus.

PURPOSE: To describe three patients with systemic lupus erythematosus in whom ophthalmoscopic and fluorescein angiographic evidence of central serous chorioretinopathy developed. METHODS: The authors retrospectively reviewed the clinical and photographic records of three patients with systemic lupus erythematous in whom central serous chorioretinopathy developed. RESULTS: Ophthalmoscopic changes observed in these patients with systemic lupus erythematosus included discrete areas of clumping and mottling of the retinal pigment epithelium (RPE), focal RPE detachments, serous elevations of the neurosensory retina, and late subretinal fibrosis with scar formation. Fluorescein angiographic findings included transmission hypofluorescence and hyperfluorescence corresponding to focal RPE alterations, early punctate intense hyperfluorescence corresponding to RPE leaks with progressive filling of sub-RPE detachment spaces, and slow late filing of subretinal detachment spaces. CONCLUSION: Patients with systemic lupus erythematosus are at increased risk to have central serous chorioretinopathy develop. The pathogenetic implications for an association between systemic lupus erythematosus and central serous chorioretinopathy as well as the similarity to the chorioretinopathy seen with accelerated hypertension, pregnancy, hemodialysis, organ transplantation, and exogenous and endogenous hypercortisolism are discussed. Focal choroidal vasculature compromise with secondary dysfunction of overlying RPE cells is the proposed common mechanism.

Adult↗
PubMed1995

Detection of circulating adhesion molecules ICAM-1, VCAM-1 and E-selectin in Wegener's granulomatosis, systemic lupus erythematosus and chronic renal failure.

The adhesion molecules ICAM-1, VCAM-1 and E-Selectin are regulated by proinflammatory cytokines and play an important role in the binding and activation of leukocytes in inflammatory diseases. This study measured the serum concentrations of circulating adhesion molecules (cICAM-1, cVCAM-1, cE-Selectin) by sandwich ELISA in systemic vasculitis with renal involvement (Wegener's granulomatosis WG, n = 25; systemic lupus erythematosus SLE, n = 50) in comparison to chronic glomerulonephritis (n = 10), stable renal allograft function and end-stage renal disease (CAPD/hemodialysis, each n = 10). Both cICAM-1 and cVCAM-1 levels, but not cE-Selectin, were significantly increased (p < 0.001) in active WG compared to healthy controls. cICAM-1, not cVCAM-1 differed significantly between active and inactive WG. Only cVCAM-1 was significantly elevated in active/inactive SLE (p < 0.01). WG with rapidly progressive glomerulonephritis had significantly raised levels of cICAM-1 and cVCAM-1, but not cE-Selectin, compared to controls (p < 0.005). In lupus nephritis only cVCAM-1 was significantly elevated (p < 0.01). cICAM-1 and cVCAM-1 levels were significantly raised in WG patients, that were hemodialysed and in patients with hemodialysis because of different reasons when compared to controls. However, cVCAM-1 but not cICAM-1 was significantly higher in WG with HD than without HD. Patients with chronic glomerulonephritis, renal allografts or CAPD also had significantly raised cVCAM-1 concentrations. These data suggest, that levels of circulating adhesion molecules might reflect different pathophysiologic processes in systemic vasculitides and endothelial/immune activation in non-inflammatory renal diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2004

Systemic lupus erythematosus in Kuwaiti children: organ system involvement and serological findings.

The course and severity of systemic lupus erythematosus (SLE) in children is generally similar to the adult form with potential serious organ system involvement, there are, however, factors that influence the prevalence and clinical behavior of the disease. Our objective was to analyse the organ system involvement and immunological findings in Kuwaiti children with SLE in relation to gender and age of onset and compare these findings to that in published reports. Organ system involvement and serologic profiles were analysed in 35 children with SLE. The major organ systems studied were: renal, hematological, cardiac, pulmonary, hepatic and the central nervous system. The prevalence of ANA, anti-dsDNA, anti-Sm, SSA, SSB and anti-cardiolipin antibodies were studied in addition to complement C3 and C4 levels. The results showed that a high percentage of children had hematological involvement (34%); thrombocytopenia (23%) and hemolytic anemia (20%). Renal involvement was proven by biopsy in only 10 children (29%). Neuropsychiatric manifestations were seen in five (14%) of patients. Males had a tendency for major organ involvement relative to females. All patients had positive ANA tests. All males had positive anti-dsDNA tests compared to 86% of female patients. The most significant finding in this study is the high frequency of hematological manifestations and the relatively low incidence of renal disease and neuropsychiatric abnormalities in Kuwaiti children with SLE.

Adolescent↗
PubMed1992

[Systemic lupus erythematosus and pregnancy].

In patients with systemic lupus erythematosus in remission pregnancy has a good prognosis for mother and foetus. Pregnancy does not have a negative effect on systemic lupus erythematosus in remission, either in the short or the long term. Immuno-regulatory drugs should not be withdrawn before or after start of pregnancy, and flares during pregnancy can be treated with steroids and/or azathioprine. The risk of flare is very high in the puerperium, and can be prevented by steroids. All patients are at high risk of losing the foetus, particularly patients with antiphospholipid antibodies. Patients at high risk should be given low doses of aspirin (80 mg daily) from the first day of pregnancy. Neonatal lupus and congenital heart block are rare, and are associated with the presence of anti-SSA and anti-SSB antibodies in the maternal blood.

Autoantibodies↗
PubMed1999

Systemic lupus erythematosus and myoclonic epileptic manifestations.

Systemic lupus erythematosus (SLE) frequently involves the central nervous system (CNS) and, in fact, epileptic manifestations may be one of the earliest symptoms of SLE. These early occurrences of epilepsy, however, can easily be misdiagnosed as indication of pure epileptic syndrome when the SLE diagnosis is still largely incomplete. We present a young girl who developed myoclonic photosensitive seizures at the onset of the illness, erroneously diagnosed as manifestation of a "pure" epileptic syndrome. Shortly after the onset of an anticonvulsant therapy (lamotrigine), there was a remarkable impairment of the general clinical condition: at that time a diagnosis of SLE was made and a specific treatment began. However, the seizures persisted and evolved toward status epilepticus which needed pentobarbitone therapy in an intensive care unit (ICU). After recovery, the girl gradually got better and during the 23 months of follow-up she received only corticosteroid therapy and did not experience seizures nor SLE relapses.

Anti-Inflammatory Agents↗
PubMed1985

[Systemic lupus erythematosus. A new look].

The systemic lupus erythematosus is a chronic inflammatory condition in which many abnormalities of the immune system have been described. Recent work has shown a subset of antiphospholipid antibodies which are apparently independent of the classical antinuclear antibodies. Their association with the false positive VDRL and the lupus "anticoagulant" is statistically significant. Clinically, these antibodies are associated with thrombosis, thrombocytopenia, thrombotic and demyelinating neurological features.

Autoantibodies↗
PubMed2000

Nonsteroidal anti-inflammatory drugs in systemic lupus erythematosus.

Up to 80% of patients with systemic lupus erythematosus (SLE) are treated with nonsteroidal anti-inflammatory drugs (NSAID) for musculoskeletal symptoms, serositis and headache. This survey reviews the literature on non-selective and selective inhibitors of cyclooxygenases, with an emphasis on the efficacy and safety profile reported in SLE patients. No lupus-specific data on gastro-intestinal side effects of NSAID exist. Both non-selective Cox inhibitors and selective Cox-2 inhibitors induce renal side effects, including sodium retention and reduction of the glomerular filtration rate. Lupus nephritis is a risk factor for NSAID-induced acute renal failure, but not for rare idiosyncratic toxic renal reactions to NSAID. In refractory nephrotic syndrome, NSAID have been used successfully. Cutaneous and allergic reactions to NSAID are increased in SLE patients as well as hepatotoxic effects, particularly with high dose aspirin. Whereas a variety of central nervous system side effects of NSAID are probably no more common in SLE patients than others, aseptic meningitis has been reported more frequently. Ovulation and pregnancy can be adversely affected by Cox inhibitors. The antiplatelet effect of aspirin and non-selective Cox inhibitors has a therapeutic potential in patients with antiphospholipid syndrome (APS). In summary, treatment of SLE with NSAID requires awareness for the increased frequency of some side effects and close monitoring of toxicity.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1978

Oral contraceptive therapy and systemic lupus erythematosus.

The relationship between oral contraceptive therapy and systemic lupus erythematosus is not well defined. It has been reported that oral contraceptives may induce lupus: they may also exacerbate pre-existing disease. This report concerns a young woman in whom systemic lupus erythematosus developed three weeks after the commencement of oral contraceptive therapy, but who was shown to have had a chronic biological false positive serological test for syphilis for eight months prior to this. The significance of false positive serological tests for syphilis and the effect of female sex hormones on disease activity in systemic lupus erythematosus is discussed.

Adult↗
PubMed1993

Disseminated intravascular coagulation in a patient with systemic lupus erythematosus with lupus anticoagulant.

A 19-year-old female patient with systemic lupus erythematosus (SLE) who had lupus anticoagulant (LA) and concurrently developed disseminated intravascular coagulation (DIC) during an exacerbation of "central nervous system (CNS) lupus". Since no other indications or causes for DIC could be demonstrated, she was treated with prednisolone and anticoagulants, which rapidly alleviated her DIC condition as well as "CNS lupus". Although abnormalities of coagulation are frequently reported in SLE patients, DIC rarely occurs in patients with SLE without associated complications. A review of the pathogenesis of DIC in patients with SLE is discussed.

Adolescent↗