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Neonatal lupus erythematosus (NLE) is an inflammatory syndrome in the fetus or neonate associated with the presence of anti-Ro(SSA) and anti-La(SSB) antibodies in the mother. It is characterized by a combination of dermatologic, hematologic, hepatic, and cardiac manifestations. NLE has been reported in twins; we describe neonatal lupus erythematosus occurring in triplets.
Cutaneous lupus erythematosus (CLE) includes a variety of lupus erythematosus (LE)-specific skin lesions that are subdivided into three categories - chronic CLE (CCLE), subacute CLE (SCLE) and acute CLE (ACLE) - based on clinical morphology, average duration of skin lesions and routine histopathologic examination. This paper describes our personal experience in the management of CLE over the last 30 years, with details on preferential therapeutic options related to clinical, histologic and immunopathologic aspects of each clinical subset of the disease. Effective sunscreening and sun protection are considered the first rule in the management of CLE because of the high degree of photosensitivity of the disease. Antimalarial agents are crucial in the treatment of CLE and are the first-line systemic agents, particularly in discoid LE (DLE) and SCLE. Dapsone is the drug of choice for bullous systemic LE (BSLE) as well as for LE in small dermal vessels (e.g. leukocytoclastic vasculitis). Retinoids, known as second-line drugs for systemic therapy, are sometimes used to treat chronic forms of CLE and are particularly successful in treating hypertrophic LE. Systemic immunosuppressive agents are required to manage the underlying systemic LE disease activity in patients with ACLE. These drugs, especially azathioprine, methotrexate, cyclophosphamide and cyclosporine, together with corticosteroids, constitute third-line systemic therapy of CLE. In our experience, oral prednisone or parenteral 'pulsed' methylprednisolone are useful in exacerbations of disease activity. Thalidomide provides one of the most useful therapeutic alternatives for chronic refractory DLE, although its distribution is limited to a few countries because of the risk of teratogenicity and polyneuropathy. However, medical treatment with local corticosteroids remains the mainstay of CLE treatment, especially for DLE. Patient education regarding the disease is also important in the management of CLE, because it helps relieve undue anxiety and to recruit the patient as an active participant in the treatment regimen.
Patients with systemic lupus erythematosus are at increased risk for premature atherosclerosis. We examined one possible etiologic factor, dyslipoproteinemia, both before and after corticosteroid therapy. We identified 2 distinct patterns of dyslipoproteinemia. One is attributable to active disease; the other is attributable, in part, to corticosteroid therapy. The dyslipoproteinemia of active disease consists of depressed high density lipoprotein cholesterol and apoprotein A-I with elevated very low density lipoprotein cholesterol and triglyceride, while the dyslipoproteinemia after corticosteroid therapy consists of increased total cholesterol, very low density lipoprotein cholesterol, and triglyceride. The possible pathophysiologic mechanisms responsible for these patterns, as well as the possible roles in premature atherosclerosis seen in systemic lupus erythematosus patients, are discussed.
Patients with systemic lupus erythematosus (SLE) have a chance of developing liver involvement in their lifetime. The main cause of liver involvement in SLE patients is previous treatment with hepatotoxic drugs or hepatotropic viral hepatitis. Wilson's disease is a hereditary disorder and is usually diagnosed in patients presenting either neuropsychiatric disorders or manifestations related to chronic liver disease. Fulminant hepatic failure as the initial manifestation of Wilson's disease is rare. The relationship between systemic lupus erythematosus and Wilson's disease has not been established. We report a case of a 12-year-old girl with SLE who presented fulminant hepatic failure as an initial manifestation of Wilson's disease. The diagnosis was established with decreased serum ceruloplasmin level and the presence of Kayser-Fleischer ring. We treated with repeated plasma exchange. Despite repeated plasma exchange she died of multi-organ failure on the 16th hospital day. Considering this case, Wilson's disease should be considered as a cause of fulminant hepatic failure, especially in juvenile age cases.
Systemic lupus erythematosus is a chronic autoimmune disease of unknown etiology with multisystem involvement. Pulmonary hemorrhage is a major life-threatening manifestation in children and adolescents with systemic lupus erythematosus, as well as in adults. Treatment has traditionally been with high-dose corticosteroids, with or without the addition of cytotoxic agents. We report the response of a patient with childhood systemic lupus erythematosus with recurrent pulmonary hemorrhage to treatment with mycophenolate mofetil.
The clinical heterogeneity of systemic lupus erythematosus (SLE) is associated with a multiplicity of autoimmune responses to both cellular and soluble antigens. At least part of the clinical heterogeneity is associated with serological heterogeneity and this is particularly striking among patients with SLE who produce anti-Ro(SSA) and anti-La(SSB). Certain subsets within the lupus spectrum are closely associated with the anti-Ro(SSA) response. These include subacute cutaneous lupus erythematosus, the lupus like syndrome associated with homozygous C2 and C4 deficiency, and neonatal lupus erythematosus. Moreover, among the patients with SLE who produce anti-Ro(SSA) are 2 stable groups, on which produces anti-Ro(SSA) alone and one which produces both anti-Ro(SSA) and anti-La(SSB). Clear clinical and serological differences distinguish these groups and it is hypothesized that these differences are due to genetic differences in the HLA-D region. Exact identification of the genes involved and how they control immune responses should lead to a clearer understanding of the clinical phenomena seen in these patients.
Lupus erythematosus (LE) was first described as a clinical dermatological entity in 1851. The possibility of serious systemic manifestations became recognized by 1872 as the result of the work of M. Kaposi. Since then, it took a long time before LE was recognized to be an immunological disease. Recognition of antinuclear antibodies and their deposition at the basal membrane region in skin resulted in two concepts of LE pathogenesis. In one, antibody complexing and complement activation with generation of the membrane attack complex (C5-C9) is thought to be the origin of the chronic inflammatory reaction. In the other, antibody deposition enables antibody dependent cellular cytotoxicity, leading to hydropic degeneration of the basal epidermal layer and subsequent chronic inflammation. Norris postulated in 1993, that the epidermis acts as a pro-inflammatory organ, in which an UVB-induced increase in cytokine production is followed by increased expression of adhesion molecules on keratinocytes as well as dermal endothelial cells. Translocation of certain antigens (i.e. Ro & La) to which circulating auto-antibodies exist in LE, enables recognition by adhesion molecule directed skin-invading T cells with subsequent cytotoxic effector activity. A persistent chronic inflammatory reaction then ensues. A similar development in knowledge may be seen in the history of immunodermatology. Originally, lupus erythematosus could not be recognized as an immune disease, since concepts of immunology were virtually non-existent when LE was first described. Immunology, In the first half of this century, was mainly antibody-oriented and thus came the concept of (S)LE as an antibody-mediated disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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INTRODUCTION: Diffuse brain disease in systemic lupus erythematous (SLE) can be difficult to assess because of the sparse biological expression of the disorder, resulting in usually normal neuroimaging and laboratory findings. For this reason, it is likely that patients without a previous SLE diagnosis that presented initially with a pure psychiatric disorder, as psychosis or depression, can remain undiagnosed, and, in fact, they are exceptionally reported. As a biological marker, antiribosomal P protein antibodies have been closely related to SLE psychosis although their pathogenic role and specificity is under discussion. CLINICAL CASE: A young woman, without relevant medical history, presented with an acute psychotic catatonic picture, suspected organic in nature due to the existence of persistent mild CSF pleocytosis, while neuroimaging and laboratory studies were negative, including antinuclear and antiribosomal antibodies. Finally, a SLE diagnosis could be established because of a strong lupic band phenomenon in skin biopsy. CONCLUSIONS: It is important to maintain a high suspicion of SLE in acute psychotic patients, especially if atypical features are present. As the biological expression of the disorder in such cases may be elusive, we propose the study of lupus band in skin biopsy for these patients, regardless the absence of antiribosomal antibodies.
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Although an acute abdomen often occurs in lupus patients, definitive treatment is commonly delayed due to the difficulty in diagnosis. We performed an abdominal ultrasound (US) in two patients with severe abdominal pain and recognized mural thickenings of intestinal walls. In the first case high dose prednisone was given for severe abdominal pain, with subsequent perforation of sigmoid colon. Pathological specimens showed a thrombotic vasculitis in the mesenteric artery and arteriole of intestinal walls. In the second case physical examination elicited a rebound tenderness. An abdominal X-ray demonstrated a gasless ileus and US also showed marked thickening of intestinal walls, 16 mm in thickness. A CT scan revealed the same findings in the entire bowel. After high dose methylprednisolone was administered, her symptoms markedly improved. The mural thickenings disappeared within 10 days as seen by abdominal ultrasound examination. The US is very useful in order to make an early diagnosis of intestinal vasculitis, showing as edema (but non-specific) of intestinal wall and we can use it safely and repetitively without exposure to radiation, compared with other examinations, such as CT scan and gastrointestinal contrast study etc. The US is not only useful in diagnosis, but also helpful for follow up of patients after the initiation of treatment.
BACKGROUND: Neuropsychiatric involvement in systemic lupus erythematosus is complex and several clinical presentations are related to this disease such as: convulsions, chronic headache, transverse myelitis, vascular brain disease, psychosis and neural cognitive dysfunction. OBJECTIVES: To assess the efficacy and safety of cyclophosphamide and methylprednisolone in the treatment of neuropsychiatric manifestations of systemic lupus erythematosus on mortality and side effects. SEARCH STRATEGY: We searched EMBASE, LILACS, Cochrane Controlled Trials Register and MEDLINE up to and including December 1999, additional articles were sought through handsearching in relevant journals, using the search strategy described in the Cochrane Handbook [Dickersin 1994]. There were no language restrictions. SELECTION CRITERIA: All randomized controlled trials which compared cyclophosphamide to methylprednisolone were to be included. Patients of any age and gender were included if they fulfilled the criterion of the American Rheumatology Association for the diagnosis of systemic lupus erythematosus and presented with any one of the following neuropsychiatric events; convulsions, organic brain syndrome; cranial neuropathy. Outcome measures included the following: a) Overall mortality (primary event); b) Motor and psychiatric deficit (primary event); c) Clinical improvement (secondary event). DATA COLLECTION AND ANALYSIS: The analysis planned was to do the following: Data would be independently extracted by the two reviewers and cross-checked. The methodological quality of each trial would be assessed by the same two reviewers. Details of the randomisation (generation and concealment), blinding, and the number of patients lost on follow-up would be recorded. The results of each RCT would be summarised on an intention-to-treat basis in 2 x 2 tables for each outcome. External validity would be defined by characteristics of the participants, the interventions and the outcomes. If appropriate, RCTs would be stratified based on control group and category of disease in accordance to the clinical homogeneity (external validity). The results obtained from these different methods are very similar, and therefore, only the results from the Risk Difference method, with the corresponding 95% confidence interval would be presented in this review. The fixed effects model would be used if there was no significant statistical heterogeneity. MAIN RESULTS: We found no randomised controlled trials comparing cyclophosphamide versus methylprednisolone for the treatment of neuropsychiatric involvement in the systemic lupus erythematosus. REVIEWER'S CONCLUSIONS: Cyclophosphamide regimen treatment is a form of care in neuropsychiatric involvement in systemic lupus erythematosus with no evidence to prove better effectiveness and safety when compared with methylprednisolone. This systematic review found no randomised controlled trials and its findings must be interpreted as 'no evidence of effect' and not as 'evidence of no effect'.
Cutaneous lupus erythematosus can be induced or precipitated by a variety of drugs. Among the cutaneous variants of lupus, subacute cutaneous lupus erythematosus is the one most often associated with drug intake. The time lag between drug intake and skin eruption makes the clinical association less obvious, and the condition is often overlooked. We report on a 50-year-old woman with previously diagnosed systemic lupus erythematosus who had a severe cutaneous flare-up seven weeks after starting treatment with terbinafine for suspected onychomycosis.
In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded DNA ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. Patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.
In several cases of lupus erythematosus (SLE, SCLE, DLE), the Koebner phenomenon could be seen in the apparently normal skin of patients following traumas, scratching effects, operation scars, contact dermatitis, pressure from sock tops, application of liquid nitrogen, exposure to strong sun light, etc. The interval between exposure to such provocation factors and appearance of the isomorphic reactions varied from case to case. In SLE patients receiving systemic treatment with glucocorticosteroids, the Koebner phenomenon could not be found, while it was detected in patients with generalized DLE who had never received any systemic glucocorticoid hormones. It was not seen in patients with localized DLE. There was no direct relationship between the appearance of humoral autoantibodies and onset of the Koebner phenomenon. With regard to the histopathogenesis of the Koebner phenomenon, it is thought that disturbances of the local cellular repair processes and of the cytokine network, with expression of adhesion molecules, might be involved. The Koebner phenomenon may be a valuable model for the investigation of lupus erythematosus.
BACKGROUND: Systemic lupus erythematosus (SLE), an autoimmune disorder, is linked to a heightened risk of multiple malignancies, including thyroid cancer. Thyroid cancer is the most prevalent malignancy of the endocrine system, and its autoimmune-related pathological features render it an optimal subject for investigating the mechanisms of their comorbidity. The molecular mechanisms underlying this comorbidity are still ambiguous. The accurate diagnosis and treatment of thyroid cancer urgently necessitate innovative molecular targets that extend beyond conventional pathological characteristics. This study seeks to employ integrated bioinformatics approaches to elucidate potential shared molecular mechanisms and immunological features between thyroid cancer and systemic lupus erythematosus (SLE), aiming to enhance understanding of their comorbidity and identify novel intervention targets. METHODS: This study initially acquired gene expression data for TC and SLE from the GEO database and subsequently screened and identified differentially expressed genes (DEGs) shared by both diseases. Subsequently, we conducted Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG), and Reactome functional enrichment analyses on these 46 shared differentially expressed genes (DEGs) and further assessed the activation status of pertinent pathways using Gene Set Enrichment Analysis (GSEA). Subsequently, we employed CIBERSORTx to examine immune infiltration patterns and developed protein-protein interaction networks utilising the STRING database. We identified hub genes utilising the MCODE and cytoHubba plugins and visualised the findings with Cytoscape software. We additionally assessed the diagnostic efficacy of these core hub genes in an independent dataset utilising ROC curves and investigated their prognostic relevance in thyroid cancer through Kaplan-Meier survival analysis and multivariate Cox proportional hazards regression. Ultimately, we employed the Network Analyst platform to forecast transcription factor-gene and miRNA-gene regulatory networks and identified potential targeted therapeutic compounds utilising the DSigDB database. RESULTS: This study identified 46 differentially expressed genes (DEGs) commonly linked to thyroid cancer and systemic lupus erythematosus (SLE), which were significantly enriched in signalling pathways associated with immune-inflammatory activation, type I interferon responses, and complement pathway activation. Moreover, GSEA findings validated that immune-inflammatory and autoimmune-related pathways are markedly activated in both conditions. Twelve hub genes were discerned through protein-protein interaction networks. Analysis of immune infiltration indicated that thyroid cancer and systemic lupus erythematosus exhibit a shared characteristic of innate immune dysregulation, marked by the infiltration of myeloid cells (neutrophils, M0/M2 macrophages). Receiver operating characteristic (ROC) curve analysis identified six significant core hub genes with substantial diagnostic value: C1QB, LCN2, C1QC, LTF, VSIG4, and C3AR1. Univariate survival analysis indicated that elevated expression of C1QC and C3AR1 significantly enhances overall survival in thyroid cancer patients; however, multivariate COX regression analysis revealed that their independent prognostic significance necessitates further validation. This study predicted the interaction networks of transcription factors and miRNAs regulating key genes, with LCN2 demonstrating the highest connectivity to miRNAs, and identified candidate therapeutic compounds linked to it. CONCLUSION: This study employed bioinformatics analysis to identify critical shared hub genes and molecular pathways connecting thyroid cancer and systemic lupus erythematosus, offering novel insights into their shared pathogenesis and the advancement of targeted biomarkers and therapeutic strategies.