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Two cases of fatal systemic lupus erythematosus (SLE) with cerebral involvement are described. Both showed widespread microinfarction of the brain secondary to platelet and fibrin thrombi. There was no evidence of vasculitis. Anticoagulants and antiplatelet drugs may have an important role in the management of cerebral SLE.
OBJECTIVE: Disease activity in systemic lupus erythematosus (SLE) is usually assessed with complex disease activity scores comprising a variety of different parameters. In order to determine whether SLE disease activity correlates with abnormal B lymphocyte activity, B cell subsets were analyzed, and their relationship to clinical and humoral measures of disease activity was assessed. METHODS: The distribution of B cell subsets was determined by fluorescence-activated cell sorting analysis and assessed in relation to the autoantibody profile, disease activity measured by the SLE Disease Activity Index (SLEDAI) and the European Consensus Lupus Activity Measure scores, disease duration, and therapy. RESULTS: The number and frequency of CD27(high) plasma cells were significantly correlated with the SLE disease activity indices and with the titer of anti-double-stranded DNA (anti-dsDNA) autoantibodies. Circulating B cell subsets were not influenced by age or sex, but appeared to relate to the duration of disease and the therapeutic regimen, with the number and frequency of CD27(high) plasma cells increasing and those of CD27- naive B cells decreasing over time. Patients were divided into those with a SLEDAI score of 0-8 (low disease activity) and those with SLEDAI score >8 (high disease activity). Patients with high disease activity had an increased frequency of both CD19+ B cells and CD27(high) plasma cells. By using a nonparametric data sieving algorithm, we observed that these B cell abnormalities provided predictive values for nonactive and active disease of 78.0% and 78.9%, respectively. The predictive value of the B cell abnormalities (78.9%) was greater than that of the humoral/clinical data pattern (71.4%), including anti-dsDNA antibody levels, circulating immune complexes, increased erythrocyte sedimentation rate, mucocutaneous involvement, and acute renal involvement. CONCLUSION: Flow cytometric monitoring of B cell subsets in the peripheral blood provides new insights into abnormalities of B cell function in SLE and may also be a diagnostically valuable option for monitoring the activity of this autoimmune disease.
While mild thrombocytopenia in systemic lupus erythematosus (SLE) is frequently seen in the context of active disease, severe thrombocytopenia causing significant bleeding is not that common. Corticosteroids are considered the first line therapy for severe thrombocytopenia in SLE. Second-line therapeutic agents or splenectomy have been reported to be effective for patients who fail to respond to steroids or those who require moderate doses of steroids to maintain the platelet counts. Recent randomized controlled studies have shown that mycophenolate mofetil (MMF) is an efficacious and safe therapeutic agent in patients with proliferative forms of lupus nephritis. However, little information has been available regarding the role of MMF in the treatment of immune thrombocytopenia complicated with SLE. Hereby I describe a patient with SLE in whom thrombocytopenia was refractory to corticosteroids, intermittent intravenous cyclophosphamide, azathioprine, cyclosporine, intravenous gamma globulin, danazol, and splenectomy, and whose platelet counts eventually normalized during therapy with MMF. In this patient, thrombocytopenia is initially thought to be associated with active SLE involving major organ. However, after immunosuppressive agents were given, the refractory nature of thrombocytopenia seems to be an isolated phenomenon, independently of SLE activity.
A young woman with systemic lupus erythematosus developed a rapidly fatal pneumonia from which no visible or culturable organisms were found. Subsequent stains disclosed typical findings of Legionnaires' disease. A cutaneous portal of entry was suspected and a fulminant lung abscess developed, neither of which has been previously reported in Legionnaires' disease.
A young woman with systemic lupus erythematosus (SLE) had clinical evidence of acute cor pulmonale. Autopsy disclosed vascular lesions in the lungs resembling those seen in advanced pulmonary hypertension. This case illustrates that severe pulmonary vascular disease may complicate SLE and mimic pulmonary thromboembolic disease.
INTRODUCTION: Neonatal lupus erythematosus (NLE) presents clinically with either cutaneous lesions or cardiac involvement and is related to the transplacental passage of anti-Ro antibodies from mother to foetus. We report a series of 10 cases seen at the National Skin Centre between 1990 and 1998 including a pair of siblings. CLINICAL PICTURE: The female to male ratio was 2.3:1 and most presented with rash between 3 to 5 weeks of life while 4 had lesions at birth. Four of the 9 mothers had SLE, one was diagnosed to have a lupus-like illness, 2 had Sjogren's syndrome and the remaining two had very high ANA titres but the final diagnosis was uncertain. OUTCOME: The rash resolved spontaneously by 3 to 6 months of age.
Of 319 patients with systemic lupus erythematosus (SLE), nine had thyrotoxicosis, three had hypothyroidism, and two had thyroiditis. This prevalence seems greater than that of similar thyroid disorders seen in the general population. It is suggested that patients with autoimmune thyroid disorders may develop SLE or vice versa. This association requires confirmation by prospective study.
One hundred patients with systemic lupus erythematosus (SLE) were investigated for serological markers of hepatitis B virus (HBV) and had liver function evaluated. One patient had hepatitis B surface antigen and 25 had antibodies to HBsAg which is not significantly different from normal blood donor controls. Minor liver function abnormalities were found in 35 patients and there was no difference between those with and without anti-HBs. A single patient without HBV markers had more severe liver enzyme elevation. Our data do not show a relationship between SLE and HBV and support the contention that liver diseases in lupus is mild.
We report the case of a patient with discoid lupus erythematosus who developed a severe skin reaction whilst undergoing mantle irradiation for non-Hodgkin's lymphoma. Widespread moist desquamation occurred after a skin dose of only 17 Gy and was associated with an abscopal response outside the treatment area. The case illustrates the need for extreme caution when administering radiotherapy to patients with discoid or systemic lupus erythematosus.
In a patient with systemic lupus erythematosus, anticoagulant activity directed against factor XI was found together with thrombocytopenia. In the serum globulin fraction, antiplatelet antibodies and an activity-inhibiting platelet aggregation could also be found. A possible correlation between the inhibition of platelet aggregation and the anticoagulant activity directed against factor XI is discussed.
Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) can produce a broad range of disease-specific neuropsychiatric manifestations that must be differentiated from infections, metabolic complications, and drug-induced toxicity. Despite the development of classification criteria by the American College of Rheumatology, the prevalence of neuropsychiatric systemic lupus erythematosus (NPSLE) varies widely across studies. Some of the neuropsychiatric manifestations are extremely rare, indicating a need for multicenter studies. Mechanisms that can lead to neuropsychiatric manifestations include intracranial vascular lesions (vasculitis and thrombosis); production of autoantibodies to neuronal antigens, ribosomes, and phospholipids; and inflammation related to local cytokine production. As a rule, no reference standard is available for establishing the diagnosis of NPSLE. Several investigations can be used to assist in the clinical diagnosis and to evaluate severity. Treatment remains largely empirical, given the absence of controlled studies. Variable combinations of corticosteroids, immunosuppressants, and symptomatic drugs are used according to the presumptive main pathogenic mechanism.
Eye involvement in chronic cutaneous lupus erythematosus (CCLE) has been reported infrequently. We describe two cases of disseminated CCLE, a 50-year-old woman and a 45-year-old man, with eyelid involvement and disease duration of 4 and 20 years respectively. The first patient showed an excellent response to chloroquine while there was lack of response in the second patient.
Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.
It has long been observed that sun exposure can induce or exacerbate skin lesions in patients with certain forms of lupus erythematosus. Despite the frequency of photosensitivity in these patients, the mechanism by which ultraviolet radiation alters the pathogenic course of this disease remains poorly understood. After development of standardized test methods, our group demonstrated in 1986 that skin lesions in patients with lupus erythematosus can be experimentally reproduced by UVA and UVB irradiation. In the following years, phototesting has received much attention as a valid model to study photosensitivity of different forms of lupus erythematosus and the pathogenetic mechanism of this disease. Further investigations have also made it possible to find genetic and immunologic factors associated with photosensitivity and have helped to identify the pathophysiologic steps involved in the induction of such skin lesions. We present phototesting results and clinical correlations of more than 400 patients with different forms of lupus erythematosus and discuss the recent advances in provocative phototesting.
We report two cases of lupus erythematosus (LE) in which a truncal papulonodular eruption predominated. Histologically the lesions were characterized by diffuse dermal mucin without the usual inflammatory or epidermal changes of LE. It is uncommon in LE for dermal mucin to be present in a sufficient quantity to produce a papulonodular eruption in the absence of typical epidermal changes.
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A patient with systemic lupus erythematosus and a three-month history of bilateral follicular conjunctivitis had both a superficial and deep culture-negative keratitis. Although SLE interstitial keratitis is uncommon, it must be recognized as a definite clinical entity. Its prompt recognition and treatment with corticosteroids may quickly bring about improvement, whereas if it is unrecognized, corneal scarring and loss of vision may follow.