Kikuchi-Fujimoto disease: pediatric case presentation

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare, benign condition, usually self-limited to a six-month period. Classic clinical presentation is characterized by cervical lymphadenopathy, leukopenia and prolonged fever. It affects principally young, Asiatic adult women, although a few cases in the pediatric population have been reported. This disease frequently mimics tuberculous lymphadenitis, malign lymphoma, systemic lupus erythematosus and other benign and malignant conditions. The etiology is unknown, although a viral or autoimmune pathogenesis has been suggested. Laboratory investigations are usually unremarkable, and the most frequent findings are leukopenia, elevated erythrocyte sedimentation rate, anemia and positive antinuclear antibodies. To make an accurate diagnosis, histopathological studies are required. To avoid the use of antibiotics (which have no effect), early diagnosis is necessary. Herein the case of a 10 years old girl is described that presented with high, prolonged fever and marked cervical lymphadenopathy secondary to Kikuchi-Fujimoto disease.     

Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant.

The lupus anticoagulant was found in the plasma of 31 of 60 patients with systemic lupus erythematosus and other connective tissue disorders (mixed connective tissue disease, systemic vasculitis, polyarteritis nodosa, primary sicca syndrome, discoid lupus, Behcet''s syndrome, and systemic sclerosis). Strong associations were found with biological false positive seroreaction for syphilis and thrombocytopenia. The most striking association, however, was with the high prevalence of thrombosis. This tendency to thrombosis was independent of disease activity of systemic lupus erythematosus. The lupus anticoagulant appears to be a useful marker for a subset of patients with systemic lupus erythematosus at risk for the development of thromboembolic complications.     

Fine needle aspiration cytology in systemic lupus erythematosus lymphadenopathy. A case report

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology characterized by inflammation in various organ systems, including lymph nodes, due to the production of antinuclear antibodies. The onset of disease is between ages 13 and 40 years, with a female preponderance. CASE: A 30-year-old female presented with right cervical lymphadenopathy and gave a history of intermittent fever and swollen joints of 2.5 years' duration. The patient was on intermittent corticosteroids. With a suggestion of tuberculous lymphadenitis, the patient underwent fine needle aspiration (FNA). The diagnosis of lupus adenopathy was established by FNA of enlarged right cervical lymph nodes. Smears showed predominantly typical and atypical immunoblasts, plasma cells, occasional Reed-Sternberg-like cells and dispersed hematoxylin bodies. Smears were negative for acid-fast bacilli. CONCLUSION: When SLE patients develop lymphadenopathy, FNA cytology helps differentiate lupus adenopathy from infectious conditions, such as tuberculous adenitis, and from Kikuchi's lymphadenitis.     

Acute acalculous cholecystitis complicating systemic lupus erythematosus: case report and review.

A case of acalculous cholecystitis presented as an acute abdominal emergency in a 22 year old woman with severe systemic lupus erythematosus. At the time of presentation the patient was receiving high doses of prednisone and cyclophosphamide to control her underlying disease. Histological examination of the biopsy specimen from the gall bladder showed lupus vasculitis. This complication of systemic lupus erythematosus has not been reported before. Laboratory studies and changes in lupus activity may fail to predict the onset of cholecystitis.     

Deletion of IL-18 Expression Ameliorates Spontaneous Kidney Failure in MRLlpr Mice

The role of IL-18 in the pathogenesis of systemic lupus erythematosus is still not definitively solved. In this study, we generated MRL^lpr mice, which develop a disease resembling systemic lupus erythematosus, genetically devoid of IL-18 expression. These mice in comparison to IL-18-competent MRL^lpr mice show reduced signs of renal pathogenesis, while other parameters such as mean survival time, lymphadenopathy, constitutive interferon-γ production, and frequency of CD3⁺B220⁺ abnormal T cells were without differences. We conclude that in the systemic lupus erythematosus syndrom IL-18 is involved specifically in the renal pathogenesis.     

Allergic diseases,drug adverse reactions and total immunoglobulin E levels in lupus erythematosus patients

BACKGROUND: The association of allergic diseases, drug adverse reactions and elevated total immunoglobulin E (IgE) concentration in systemic lupus erythematosus patients remains controversial. The aim of the study was to investigate the prevalence of those features in active and inactive systemic lupus erythematosus patients, and in the control group as well. METHODS: Total IgE concentration was evaluated by enzyme-linked immunosorbent assay. RESULTS AND CONCLUSIONS: The results of our study revealed that concomitant allergic diseases were not more frequent in systemic lupus erythematosus patients than in the general population. Total IgE concentration was significantly higher during the active stage of the disease. Drug reactions were very frequent but not connected with IgE elevation. Our results indicate that IgE may play a role in lupus pathogenesis, especially in the active phase of the disease.     

Patients with Systemic Lupus Erythematosus Have Higher Prevalence of Thyroid Autoantibodies: A Systematic Review and Meta-Analysis

Background

Thyroid autoimmunity is considered the most common type of organ-specific autoimmune disorder and can be associated with other autoimmune endocrine disorders or non-endocrine diseases. Systemic lupus erythematosus is a prototypical autoimmune disorder with multifactorial etiology. The pathogenesis and development of the disease may result from a loss of immune tolerance and the resulting synthesis of autoantibodies against nuclear antigens. Autoimmune factors may be common features of both thyroid autoimmunity and systemic lupus erythematosus, making it likely that both conditions may coexist within some patients.

Methods and Findings

A number of studies have investigated whether an association between thyroid autoimmunity and systemic lupus erythematosus exists. However, the results of these studies have been inconsistent. Furthermore, most of these studies have had relatively small sample sizes, which have rendered them insufficiently powerful to determine whether there is an association between systemic lupus erythematosus and thyroid autoimmunity. The main objective of this meta-analysis is to provide reliable estimates of the extent of any association between thyroid autoimmunity and systemic lupus erythematosus by combining the primary data from all relevant studies. Literature databases were searched, including the Medline, Embase, Web of Science, Chinese Wanfang and CBM databases, from January 1970 to May 2014. A total of 1076 systemic lupus erythematosus cases and 1661 healthy controls were included in this study. From these data, the odds ratio (OR) and the corresponding 95% confidence interval (95% CI) were calculated. The meta-analysis results showed that the prevalence of thyroid autoantibody positivity in patients with systemic lupus erythematosus was higher than in healthy controls (TgAb: OR = 2.99, 95% CI = 1.83–4.89; TPOAb: OR = 2.20, 95% CI = 1.27–3.82, respectively).

Conclusion

The results of this meta-analysis suggest that thyroid autoimmunity is more prevalent in patients with systemic lupus erythematosus than in a control group.     

Cardiovascular disease in systemic lupus erythematosus: has the time for action come?

PURPOSE OF REVIEW: The recognition that inflammation is a hallmark of atherosclerotic disease has led to a series of studies reporting accelerated atherogenesis in chronic inflammatory diseases. Indeed, systemic lupus erythematosus is associated with an increased incidence of cardiovascular disease and the etiology thereof deserves closer attention. RECENT FINDINGS: The association between systemic lupus erythematosus and accelerated atherosclerosis has recently been confirmed by surrogate-marker studies for cardiovascular disease in patients with systemic lupus erythematosus. Since the propensity towards cardiovascular disease cannot solely be explained by classical risk factors, disease-specific pathways have been put forward as additional risk factors. SUMMARY: In the present review, we will discuss several of these factors as well as their potential impact for future prevention strategies in systemic lupus erythematosus.     

Fcgamma receptors in the initiation and progression of systemic lupus erythematosus

Systemic lupus erythematosus, a systemic autoimmune disorder, is characterized by the production of autoantibodies to nuclear constituents and inflammatory lesions in multiple organ systems. Although the pathogenesis of the disease is largely unknown, recent studies have suggested that disturbances in apoptosis and/or clearance of apoptotic cells may play an important role in the induction and perpetuation of autoantibody production. When autoantibodies subsequently complex to autoantigens present on apoptotic cells, ligation of Fcgamma receptor will result in inflammation and disease development. Indeed, mice deficient in activating Fcgamma receptors were protected against inflammation in models of immune complex-mediated autoimmune disease, whereas deletion of the inhibitory Fcgamma receptors increased autoantibody production and susceptibility to immune complex-induced inflammation. Additionally, functional polymorphisms in Fcgamma receptors were shown to be associated with development of human systemic lupus erythematosus. This review focuses on the role of Fcgamma receptors in the initiation of autoantibody production, inflammatory handling of immune complexes, and disease development in systemic lupus erythematosus.     

PSYCHOSIS AND SYSTEMIC LUPUS ERYTHEMATOSUS—A Review of the Literature and Case Reports

In a review of the literature of the last 60 years concerning the association between psychosis and systemic lupus erythematosus, reports of 227 cases of this association were found. The average incidence of psychosis in the various series of systemic lupus erythematosus that were reviewed was 22 per cent. In only 25 per cent of the cases in which the information was given was the psychosis associated with steroid therapy.The psychiatric manifestations are variable and may be associated with a neurological disorder. Psychosis may antedate by many years other features of lupus. The psychosis due to lupus may respond to steroid therapy. Since systemic lupus erythematosus sometimes may be an important differential diagnosis of functional psychosis, appropriate diagnostic studies should be carried out in psychotic patients who have an accelerated sedimentation rate or positive serological test for syphilis without apparent reason.     

Prevention of systemic lupus erythematosus in children born to mothers treated for this disease

Genetic predisposition and environmental factors (physical, chemical, hormonal and drugs inducing collagen-like syndrome) play an important role in the pathogenesis of the systemic lupus erythematosus. Elimination of these factors from the environment of the genetically predisposed individuals may prevent part of them against the disease. Basing on the above assumption, a chart of prophylaxis has been constructed and distributed among the mothers with the systemic lupus erythematosus, recommending prophylactic measures in both mothers and children. Within 1977-1987, 50 children were examined from time to time. Basic laboratory tests, phenomenon LE, antinuclear antibodies and antibodies anti-DNA have been determined. Transient presence of antinuclear antibodies was seen in 23 children. A tendency to an increase in the antibody titre was observed in girls of this group whereas a decrease in the titre was noted in the boys with the time lapse. Systemic lupus erythematosus prophylaxis in both mothers and children is uncomplicated and favourable for children. Regular determination of antibodies enables early diagnosis of the disease.     

Autoimmune response to U1 small nuclear ribonucleoprotein (U1 snRNP) associated with cytomegalovirus infection

The induction of autoantibodies to U1 small nuclear ribonucleoprotein (U1 snRNP) complexes is not well understood. We present evidence that healthy individuals with cytomegalovirus (CMV) infection have an increased frequency and quantity of antibodies to ribonucleoprotein, directed primarily against the U1-70k protein. A significant association between the presence of antibodies to CMV and antibodies to the total RNP targeted by the immune response to the spliceosome (to both the Sm and RNP; Sm/RNP) was found for patients with systemic lupus erythematosus (SLE) but not those with mixed connective-tissue disease. CMV thus may play a role in inducing autoimmune responses in a subset of patients with systemic lupus erythematosus.     

Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant.

A woman presented with multiple cerebral thromboses and skin lesions characteristic of Degos'' disease. Her serum contained high titres of anticardiolipin antibodies and showed lupus anticoagulant activity, both known to be strong markers of a thrombotic tendency in systemic lupus erythematosus. This finding may have therapeutic implications for this usually fatal disease.     

Editorial: Islet cell tumours of the pancreas.

This report documents a case of systemic lupus erythematosus in which the major presenting feature was pericarditis and in which the diagnosis was made after pericardiocentesis by the demonstration of typical LE cells in pericardial fluid stained with Wright''s stain. Review of the literature indicates that pericarditis in lupus is more common than is generally suspected, and when it is the initial manifestation it is often misdiagnosed as “benign viral pericarditis”.Screening for collagen disease should be a part of the work-up of any patient presenting with pericarditis, especially a woman. Occasionally lupus may be present even when the serologic tests have given negative results. Under such circumstances, or when the patient manifests an atypical or prolonged course, a diagnostic pericardiocentesis is in order, provided an effusion can be confirmed on scan. A search for LE cells should be included in the analysis of the aspirated fluid.     

Immunologic findings,thrombocytopenia and disease activity in lupus nephritis.

Twenty patients with nephritis due to systemic lupus erythematosus were followed up for a mean of 34 months after renal biopsy with serial determinations of total serum complement and C3 and C4 concentrations, binding of deoxyribonucleic acid (DNA), antinuclear antibody pattern and platelet count. There were 25 episodes of nonhematologic observed disease activity in 16 of the 20 patients; elevated DNA binding and thrombocytopenia correlated well with these episodes. The mean platelet count during episodes of observed disease activity was 96 +/- 42 X 10(9)/L, which was significantly different from the mean count of 248 +/- 90 X 10(9)/L during disease quiescence. The proportion of false-positive results with the immunologic tests varied from 25% to 67% and with platelet counts it was 11%. It is suggested that thrombocytopenia may be a simple and accurate index of disease activity in lupus nephritis.     

Disturbances of apoptotic cell clearance in systemic lupus erythematosus

Systemic lupus erythematosus is a multifactorial autoimmune disease with an as yet unknown etiopathogenesis. It is widely thought that self-immunization in systemic lupus is driven by defective clearance of dead and dying cells. In lupus patients, large numbers of apoptotic cells accumulate in various tissues including germinal centers. In the present review, we discuss the danger signals released by apoptotic cells, their triggering of inflammatory responses, and the breakdown of B-cell tolerance. We also review the pathogenic role of apoptotic cell clearance in systemic lupus erythematosus.     

Elevated urinary VCAM-1, P-selectin, soluble TNF receptor-1, and CXC chemokine ligand 16 in multiple murine lupus strains and human lupus nephritis

In an effort to identify potential biomarkers in lupus nephritis, urine from mice with spontaneous lupus nephritis was screened for the presence of VCAM-1, P-selectin, TNFR-1, and CXCL16, four molecules that had previously been shown to be elevated in experimental immune nephritis, particularly at the peak of disease. Interestingly, all four molecules were elevated approximately 2- to 4-fold in the urine of several strains of mice with spontaneous lupus nephritis, including the MRL/lpr, NZM2410, and B6.Sle1.lpr strains, correlating well with proteinuria. VCAM-1, P-selectin, TNFR-1, and CXCL16 were enriched in the urine compared with the serum particularly in active disease, and were shown to be expressed within the diseased kidneys. Finally, all four molecules were also elevated in the urine of patients with lupus nephritis, correlating well with urine protein levels and systemic lupus erythematosus disease activity index scores. In particular, urinary VCAM-1 and CXCL16 showed superior specificity and sensitivity in distinguishing subjects with active renal disease from the other systemic lupus erythematosus patients. These studies uncover VCAM-1, P-selectin, TNFR-1, and CXCL16 as a quartet of molecules that may have potential diagnostic significance in lupus nephritis. Longitudinal studies are warranted to establish the clinical use of these potential biomarkers.     

Reversal of aplastic anaemia secondary to systemic lupus erythematosus by high-dose intravenous cyclophosphamide

Aplastic anaemia is rare as a primary feature of systemic lupus erythematosus and is more commonly a complication of treatment with cytotoxic drugs. Three years after starting treatment for systemic lupus erythematosus a 22-year-old woman developed bone-marrow depression. Azathioprine was thought to be responsible and was withdrawn. The aplastic anaemia worsened despite treatment with prednisolone. In view of clinical and serological evidence of lupus disease activity the patient was given high-dose intravenous cyclophosphamide and the aplastic anaemia responded in a sustained manner.In such cases of continued disease activity high-dose immunosuppressive agents may prove effective.