Ileal perforation as an initial manifestation of systemic lupus erythematosus: A case report

Introduction and importanceLupus enteritis is uncommon in patients with SLE and usually presents with anorexia, vomiting, and abdominal pain. Intestinal perforation as an initial manifestation of SLE is rare and can have a grave prognosis if not timely diagnosed.Case historyWe report an unusual case of a 22-year-old regularly menstruating female who presented with features of perforation peritonitis as an initial manifestation of lupus enteritis. Intraoperatively, a gangrenous ileal segment with multiple perforations was present. Thus, with an intraoperative diagnosis of perforation peritonitis, a gangrenous segment of the small bowel was resected and a double-barrel jejuno-ileostomy was created.DiscussionLupus enteritis manifesting initially as bowel perforation can be an uncommon cause of acute abdomen. A plain chest X-ray can show gas under the diaphragm suggesting bowel perforation. A contrast-enhanced CT scan of the abdomen is the gold standard in diagnosing lupus enteritis with a good prognosis on steroids.ConclusionPrimary closure, resection, and anastomosis of small gut or diverting stoma are required for management of perforation. A high degree of clinical suspicion is required for early diagnosis thus preventing the grave prognosis of such an entity.     

Primary testicular manifestation of systemic lupus erythematosus

The case of a patient with a hitherto unknown primary testicular manifestation of systemic lupus erythematosus (SLE) is reported. Findings on scrotal palpation and sonography suggested the presence of a testicular tumor. Histological examination of the testicular biopsy and immune status did not confirm the clinical diagnosis but demonstrated SLE.     

End-stage renal disease as the presenting manifestation of renal systemic lupus erythematosus

About one-half of all children with systemic lupus erythematosus have clinical evidence of renal disease at initial presentation, such as proteinuria and acute renal failure. Herein, we report a case of a teenager who presented with end-stage renal disease (ESRD) of uncertain etiology, and who was subsequently determined to have lupus. The purpose of this report is to make health-care professionals aware of this unusual presentation of renal lupus, which has never been reported before. Children presenting in ESRD should be worked-up for autoimmune diseases since the discovery of such a disease process may impact future decision-making, especially with respect to subsequent renal transplantation.     

A case of systemic lupus erythematosus with neurological manifestations as initial symptoms

According to the past reports, neuropsychiatric manifestations have been seen in 10-75% of patients with systemic lupus erythematosus and are second only to renal involvement as a cause of death. The clinical feature is multiple. And cerebrovascular diseases due to systemic lupus erythematosus are detected in 3-16% of the neuropsychiatric manifestations. Occlusion of the intracranial major arteries is less frequently found in other cerebrovascular diseases. And central nervous system involvement usually occurs at some intermediate or terminal stage of systemic lupus erythematosus, so is rarely regarded as one of the initial symptoms. We studied the case of a patient with systemic lupus erythematosus with occlusion of the right middle cerebral artery indicated by angitis and 'string of beads' appearance of the right internal carotid artery indicated by fibromuscular dysplasia. The patient was a 38 year old female and began to feel weakness in the left hand and developed mild-left hemiparesis due to infarction of right temporo-parieto-occipital lesion which was revealed by CT scan. Carotid angiograph showed irregularity at the right middle cerebral artery and 'string of beads' appearance of the right internal carotid artery. Gradually neurological manifestations improved, but a facial 'butterfly' rash, palmar erythema and polyarthritis were detected.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cardiac surgery in patients with systemic lupus erythematosus

Cardiac surgery was infrequently performed in patients with systemic lupus erythematosus (SLE), and its clinical outcome was reported only in small series. We sought to evaluate the clinical outcome of cardiac operation in patients with SLE. Between January 1996 and March 2005, 9 patients with SLE underwent cardiac surgery at the authors' hospital. Six patients underwent coronary artery bypass grafting (three conventional and three on-pump beating heart), two patients underwent valve replacement and 1 patient underwent simultaneous heart-kidney transplantation. All 6 patients with coronary artery bypass grafting had saphenous venous grafts and two of them had additional left internal mammary artery graft. The overall in-hospital mortality rate was 11% (1/9). Major postoperative complications occurred in 4 patients (44%) including profuse postoperative bleeding, ventricular tachycardia and early graft thrombosis. There were two late deaths including sudden cardiac death and sepsis. The median follow-up duration was 23 months (range, 1-110). In conclusion, although the postoperative complication was common, cardiac operation could be performed in patients with SLE.     

Cardiac tamponade as the first manifestation of bronchogenic carcinoma

A case of cardiac tamponade as the first manifestation of bronchogenic adenocarcinoma is presented. Diagnosis of metastatic pericarditis with effusion was made by echocardiography and cytology of the pericarditis fluid. Because of rapid recurrence of the effusion, a pericardial window was created. This operation was well tolerated by the patient. A short discussion of metastatic pericarditis is given.     

Cardiac tamponade as the initial manifestation of metastatic adenocarcinoma from the colon: a case report

Metastatic cardiac malignancies mainly come from the lung, breast, and the lymphoreticular system by direct invasion or hematogenous or lymphatic spread. Metastasis from colorectal cancer to the heart or pericardium is seldom reported and only sporadic antemortem cases have been reported. We report an unusual case of malignant pericardial effusion caused by metastatic adenocarcinoma of colon. Malignant pericardial effusion and subsequent tamponade was the earliest manifestation without any other confirmed clinical metastases. Pericardiotomy was performed to relieve the life-threatening cardiac tamponade. We report this rare case and review the literature.     

Pulmonary thrombosis as the first manifestation of systemic lupus erythematosus in a 14-year-old boy

We describe the case of a 14-year-old boy who presented with pulmonary embolism and who was subsequently found to have nephrotic syndrome due to lupus membranous nephropathy. He had no other signs of nephrotic syndrome or of systemic lupus erythematosus, such as edema or circulating lupus anticoagulants (antiphospholipid or anticardiolipin antibodies), and no hereditary coagulopathy. This case contributes to our understanding of unusual clinical presentations of systemic lupus erythematosus in children.     

Neuropsychiatric systemic lupus erythematosus

Opinion statement The treatment of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) can be difficult and complex owing to the variety of nervous system manifestations that can occur, which include peripheral nerve disease, headaches, seizures, cerebrovascular disease, chorea, transverse myelitis, and psychiatric and cognitive disorders. Many of these manifestations can result from metabolic abnormalities or infection or as side effects of medications. Thus, in any patient with suspected NPSLE, it is crucial to exclude secondary causes of the presenting symptoms before assuming that they are due to NPSLE. It is especially important to exclude infection because this is a common cause of both morbidity and mortality in patients with systemic lupus erythematosus (SLE). Symptoms such as anxiety and depression may or may not be related to disease activity. Treatment decisions are based on accurate diagnosis of the specific NPSLE manifestation, which is usually made using tools such as brain imaging, electroencephalography, cerebrospinal fluid analysis, nerve conduction studies, or special serologic tests (eg, determination of antiphospholipid or antiribosomal P antibody levels). It is also important to assess the degree of other SLEmediated systemic disease activity in a patient with neurologic manifestations to determine if activation of systemic disease activity is also occurring. This is done by measuring complement levels, anti-double-stranded DNA levels, complete blood count, and urinalysis. For some NPSLE manifestations (eg, infrequent seizures, headaches, depression, anxiety, or peripheral neuropathy) that appear without activation of systemic disease, symptomatic treatment is appropriate. For others (eg, psychosis, delirium, or transverse myelopathy without other obvious cause), treatment with high-dose glucocorticoids with or without cyclophosphamide is appropriate whether there is evidence of other systemic disease activity or not. In general, the activity and severity of the leading organ manifestations dictate pharmacologic treatment.     

Monogenic systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a rare autoimmune disease, which is more severe in case of pediatric onset. This may be due to greater involvement of genetic factors in comparison to adult forms. SLE is a multifactorial disease and is thought to be secondary to a combination of genetic, environmental factors as well as immunological defects. Studies on early-onset SLE and both familial and syndromic cases have led to the discovery of monogenic form of SLE, with autosomal or recessive inheritance. Related genes are responsible for complement deficiency and excessive production of interferon-alpha, both mechanisms studied in SLE pathogenesis. Apoptosis defect described in autoimmunity and lymphoproliferation syndromes is the human counterpart of lpr/lpr mouse model, deeply investigated as a murine SLE model. In this review, we discuss all monogenic Mendelian forms of SLE and underline the impact of this gene discovery on better understanding of SLE pathogenesis.     

Cardiac consequences of the systemic lupus erythematosus therapy with corticosteroids morphological study

It is presented the case of a fifty years old women, diagnosed 3 years ago with systemic lupus erythematosus, under therapy with prednisone and cyclophosphamid therapy. She was admitted in our hospital for right decompensated heart disease and the presence of an apical right ventricular mass occluding part of the right ventricular cavity. The endomyocardial biopsy was made to clearify the nature of this mass. After processing the specimen, the histological study evidenciated an organizing apical thrombotic mass formed in a large right ventricular cavity in conditions of pulmonary hypertention. There are presented data concerning the adverse effects of the systemic lupus erythematosus drug therapy, as well. In these circumstances, we demonstrated histologically, that both conditions could alter the heart morphology.     

Atheroma and systemic lupus erythematosus

Epidemiologic data indicate a large increase in cardiovascular risk in patients with systemic lupus erythematosus (SLE). Non-invasive investigations show increases in intima-media thickness, carotid plaque, and coronary artery calcifications in patients with SLE, compared to controls. Conventional cardiovascular risk factors may fail to fully explain the high cardiovascular risk in SLE patients. Immunological disturbances and inflammation may indirectly contribute to the risk of cardiovascular disease by inducing dyslipidemia and/or insulin resistance. The potential role for glucocorticoid therapy is controversial. Effective control of the disease would be expected to decrease the cardiovascular morbidity and mortality rates. Careful attention should be given to controlling conventional risk factors such as obesity, smoking, and physical inactivity. Hypertension and/or dyslipidemia should be treated optimally. The appropriateness of antiplatelet therapy should be assessed.     

Urine chemokines as biomarkers of human systemic lupus erythematosus activity

The purpose of this study was to evaluate urine monocyte chemoattractant protein-1 (MCP-1) and IL-8 as biomarkers of SLE flare. Urine was collected every 2 mo from patients who were followed prospectively in the Ohio SLE Study. Renal and nonrenal flares were identified and MCP-1 and IL-8 were measured by specific ELISA in samples that were collected at flare. When available, MCP-1 and IL-8 were also measured in urine samples before and after flare. For comparison, MCP-1 and IL-8 were measured in the urine of healthy individuals and in renal and nonrenal SLE patients with stable disease activity (disease controls). Most patients were receiving maintenance immunosuppressive therapy before flare. At renal flare, mean urine MCP-1 (uMCP-1) was significantly greater than uMCP-1 at nonrenal flare and from healthy volunteers and renal disease controls. The level of uMCP-1 correlated with the increase in proteinuria at flare and was higher in patients with proliferative glomerulonephritis and in patients with impaired renal function. Urine MCP-1 was increased beginning 2 to 4 mo before flare. Patients who responded to therapy showed a slow decline in uMCP-1 over several months, whereas nonresponders had persistently high uMCP-1. In contrast, uIL-8 did not change with disease activity and was not elevated at renal or nonrenal flare compared with disease controls. In conclusion, uMCP-1 but not uIL-8 is a sensitive and specific biomarker of renal SLE flare and its severity, even in patients who receive significant immunosuppressive therapy. Persistently elevated uMCP-1 after treatment may indicate ongoing kidney injury that may adversely affect renal prognosis.     

Hypoparathyroidism in systemic lupus erythematosus

Hypoparathyroidism is a rare disease. The main cause of hypoparathyroidism is postsurgical hypoparathyroidism. However, cases of hypoparathyroidism in patients suffering from SLE exist although it is uncommon. Only three previous cases have been reported. We present the case of a woman suffering both from systemic lupus erythematosus and hypoparathyroidism. This reported association of hypoparathyroidism with lupus expands the spectrum of endocrine disorders seen in this disease. We suggest that there may be a common underlying pathophysiological process linking these diseases.     

Autoantibodies in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multifactorial disorder with multigenic inheritance and various environmental factors implicated in its aetiopathogenesis. Despite the multiple mechanisms involved in the aetiology of SLE being elusive, recent studies have made progress in our understanding of the pathogenic mechanisms via abnormal regulation of cell-mediated and humoral immunity that lead to tissue damage. The heterogeneity of the clinical manifestations probably reflects the complexity of the disease pathogenesis itself. The immune system in SLE is characterised by a complex interplay between overactive B cells, abnormally activated T cells and antigen-presenting cells. This interplay leads to the production of an array of inflammatory cytokines, apoptotic cells, diverse autoantibodies and immune complexes that in turn activate effector cells and the complement system, leading to tissue injury and damage which are the hallmarks of the clinical manifestations. SLE patients have dysregulation of inflammatory cytokines, chemokines and immune response-related genes, as well as of the genes involved in apoptosis, signal transduction and the cell cycle.