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PubMed1999

[A case report; systemic lupus erythematosus associated with lupus laryngitis].

Systemic lupus erythematosus (SLE) is an autoimmune collagen vascular disease which produces widespread damage to multiple organs. Few studies on laryngeal involvement in SLE have been reported. We report here a case of SLE complicated by lupus laryngitis. A 27-year-old woman was diagnosed as having SLE in October 1996 based on findings of polyarthritis, lymphocytopenia, positive anti-nuclear antibody and anti-Sm antibodies. Polyarthralgia disappeared transiently, and no other clinical symptoms were found. Therefore, she was followed with no medication. She presented hoarseness with high fever and facial erythema in September 1998. Laboratory findings on admission revealed pancytopenia and hypocomplementemia. Anti-nuclear antibody, anti-Sm antibody and anti-RNP antibody were all positive with high titers. Chest X ray examination showed pleural effusion. Laryngoscopy showed a bamboo-joint-like lesion at the middle of the bilateral vocal cords. She was treated with 60 mg of prednisolone (i.v.) which resulted in improvement of hoarseness, pancytopenia, hypocomplementemia and pleuritis. Based on these findings, we diagnosed her hoarseness as the manifestation of laryngitis associated with SLE (lupus laryngitis).

Adult↗

PubMed1998

The effect of race on incidence and clinical course in systemic lupus erythematosus: The Hopkins Lupus Cohort.

Systemic lupus erythematosus, a chronic autoimmune disease of young women, has a higher incidence and prevalence in African Americans. The Hopkins Lupus Cohort, a prospective longitudinal study of SLE outcomes, has shown that race is a major predictor of clinical manifestations, laboratory and serologic tests, and disease-related morbidity. The effect of race on musculoskeletal morbidity remains even after adjustment for education, insurance status, and smoking.

Adult↗

PubMed1995

Cerebellar atrophy in systemic lupus erythematosus.

Central nervous system involvement in systemic lupus erythematosus (SLE) is common; this is especially so in patients with positive antiphospholipid antibody. However, cerebellar involvement is very unusual. In our institution, we have seen 150 cases of SLE over the last 10 years; however, this is the only patient who has cerebellar involvement due to SLE. The patient had no other neurological deficit, and no other cause apart from her SLE to explain her cerebellar ataxia. Her antiphospholipid antibody was negative. The patient showed some improvement on high-dose steroids and azathioprin.

Adult↗

PubMed2000

Hepatitis C virus infection mimicking systemic lupus erythematosus: study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus.

OBJECTIVE: To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in patients with systemic lupus erythematosus (SLE). METHODS: We investigated 134 consecutive SLE patients (121 women and 13 men; mean age 35 years) who fulfilled the 1982 revised criteria for SLE of the American College of Rheumatology. Two hundred consecutive volunteer blood donors were also studied. Serum from all patients and controls was tested for antibodies to HCV (by third generation enzyme-linked immunosorbent assay and confirmed by third generation recombinant immunoblot assay [RIBA-3]). RESULTS: Antibodies to HCV were present in 18 patients with SLE (13%) and in 2 (1%) of the blood donors studied. Among the anti-HCV-positive group, HCV infection was confirmed (by RIBA-3 and polymerase chain reaction) in 15 SLE patients (11%) and in the 2 blood donors (1%) (P < 0.001). We observed a lower frequency of cutaneous SLE features (40% versus 76%; P = 0.01) and positivity for anti-double-stranded DNA (anti-dsDNA) (33% versus 81%; P < 0.001), and a higher frequency of hepatic involvement (93% versus 2%; P < 0.001), low C4 levels (73% versus 39%; P = 0.002), low CH50 levels (73% versus 44%; P = 0.03), and cryoglobulins (60% versus 22%; P = 0.02) in SLE patients with HCV infection compared with SLE patients without infection. CONCLUSION: The prevalence of HCV infection in SLE patients was higher than in blood donors from the same geographic area. SLE HCV-positive patients showed a lower frequency of cutaneous SLE features and anti-dsDNA antibodies, and a higher prevalence of liver involvement, hypocomplementemia, and cryoglobulinemia. HCV testing should be considered in the diagnosis of SLE, especially in patients who lack the typical cutaneous features of SLE or who have low titers of autoantibodies, cryoglobulinemia, or liver involvement.

Adult↗

PubMed1990

Massive hepatic infarction in systemic lupus erythematosus.

Liver disease in systemic lupus erythematosus, as demonstrated by abnormal histopathology, is rare and usually mild; typically, this hepatic disease is of chronic nature and not related to a hypercoagulable state. A patient is described in whom life-threatening hypercoagulability in association with systemic lupus erythematosus resulted in extensive liver infarction. Follow-up radionuclide liver scintigraphy suggested that regenerative recovery in the infarcted areas of the liver may be delayed or absent, but there was no evident functional hepatic impairment.

Adult↗

PubMed1988

Gluten-sensitive enteropathy and systemic lupus erythematosus.

A patient with systemic lupus erythematosus developed gluten-sensitive enteropathy, or celiac sprue. The patient's histocompatibility antigens included HLA-B8 and HLA-DR3, previously found to have a high frequency in gluten-sensitive enteropathy and possibility increased as well in systemic lupus erythematosus. Such histocompatibility antigens are common to a variety of autoimmune disorders. An immune basis for the association is discussed herein.

Celiac Disease↗

PubMed2001

An update on the genetics of systemic lupus erythematosus.

The development of systemic lupus erythematosus has a strong genetic basis. The techniques to study the genetics of SLE have improved, and family-based studies have been gaining importance due to advances in the human genome project. Recently, complete genome scans with microsatellite markers and linkage analysis have been performed in human systemic lupus erythematosus in various populations. These studies represent the first step of a process towards the identification of susceptibility genes in SLE. We review here the results of the human genome scans performed until now and provide an update of the latest advances on the genetics of SLE in linkage studies and association analyses of candidate genes.

Female↗

PubMedDate not supplied

[Pancreatitis and hepatitis associated with systemic lupus erythematosus].

A patient with systemic lupus erythematosus presenting abdominal pain, nausea, vomiting and severe mucocutaneous vasculitis had significant elevation of serum amylase and hepatic enzymes levels during a flare-up of the disease. Clinical and laboratory alterations disappeared after therapeutic increase of corticosteroids given to the patient a fact that suggested etiologic correlation between systemic lupus erythematosus, hepatitis and pancreatitis.

Acute Disease↗

PubMed1984

Oral discoid lupus erythematosus. II. Skin lesions and systemic lupus erythematosus in sixty-six patients with 6-year follow-up.

Sixty-six patients with oral discoid lesions and either discoid (DLE) or systemic (SLE) lupus erythematosus were followed during an average period of 6 years (range, 0.1 to 25 years). At time of initial examination, thirty-seven patients (56%) had discoid skin lesions and the remaining 29 (44%) had isolated oral discoid lesions. Among the latter, seven patients developed discoid skin lesions during the observation period. Ten of the sixty-six patients (15%) had SLE at the time of initial examination, and SLE developed in another five (8%) who had DLE initially. In six of fifteen SLE patients the oral discoid lesions were the first manifestation of SLE. Presence of ulceration of the oral discoid lesions at time of initial examination was of predictive value with regard to the presence or development of SLE. It is concluded that patients with oral discoid lesions should be seen at least yearly in order to secure an early diagnosis of eventually developing signs of cutaneous DLE and SLE. Furthermore, no connection was noted between the presence of skin lesions and the development or presence of SLE.

Adult↗

PubMed1999

Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus.

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.

Adrenal Cortex Hormones↗

PubMed1986

Progressive multifocal leukoencephalopathy complicating systemic lupus erythematosus.

Two patients with systemic lupus erythematosus who developed progressive multifocal leukoencephalopathy (PML) are described. Although the time of PML onset could not be determined with certainty, it probably developed after steroid-induced immunosuppression. However, high-dose corticosteroids appeared to produce transient amelioration in the neurologic disorder. We suggest that PML is an important addition to the list of conditions to be considered in the differential diagnosis when a patient with systemic lupus erythematosus develops neurologic abnormalities.

Adult↗

PubMed2006

Lymphopenia at presentation is associated with increased risk of infections in patients with systemic lupus erythematosus.

BACKGROUND: Patients with systemic lupus erythematosus (SLE) frequently suffer from infections, but the predisposing risk factors, as well as the exact frequency and nature of such infections, are not fully understood. AIM: To describe the frequency, types and risk factors for infections in a group of Chinese patients in the early stage of SLE in Hong Kong. DESIGN: Retrospective record study. METHODS: We reviewed the case records of 91 Chinese SLE patients, presenting <12 months after SLE diagnosis. Details of major infections (requiring intravenous antimicrobial therapy, or any confirmed mycobacterial infection) and minor infections were reviewed. Clinical and laboratory features, the systemic lupus erythematosus disease activity index (SLEDAI) at presentation and drug treatment were recorded and analysed. RESULTS: There were 48 major infections and 62 minor infections during 260 patient-years of follow-up. A lymphocyte count < or =1.0 x 10(9)/l at presentation was independently associated with an increased risk for major infection: hazard ratio 4.7 (95%CI 1.6-13.7), p = 0.005. SLEDAI, use of corticosteroids and immunosuppressive therapy were all not associated with increased risk of infection. DISCUSSION: Lymphopenia was an important risk factor for major infections in this group of Chinese patients in the early stages of SLE. SLE patients with lymphopenia at presentation should be closely monitored for the development of infective complications.

Adolescent↗

PubMed1985

Embolic stroke complicating systemic lupus erythematosus.

Embolic stroke complicating systemic lupus erythematosus has been infrequently reported. We examined a 25-year-old woman who suddenly became hemiplegic. Two-dimensional echocardiography identified a source of emboli. At cardiac surgery, friable thrombotic vegetations were found adhering to the mitral valve leaflets, left ventricular septal wall, and chordae tendineae. At autopsy several weeks later, careful pathologic examination of the heart failed to reveal evidence of thrombus formation or endocarditis. An embolus identical in appearance to the thrombotic vegetations described at cardiac surgery was found lodged in the left middle cerebral artery underlying the recent brain infarction. To our knowledge, this is the first report of embolic stroke in systemic lupus erythematosus caused by extensive cardiac thrombus formation in the absence of underlying endocarditis.

Adult↗

PubMed1985

Splenectomy does not cure the thrombocytopenia of systemic lupus erythematosus.

Fourteen patients with systemic lupus erythematosus had splenectomies done between 1960 and 1982 for treatment of severe thrombocytopenia. Thrombocytopenia persisted or recurred within 1 month postoperatively in five patients and within 6 months in three others. Three patients had late recurrence (18, 30, and 54 months after splenectomy); in two it was probably related to withdrawal of immunosuppressive agents or corticosteroids. Median lowest platelet count before splenectomy and median platelet count at relapse or failure of splenectomy were both 8000/microL. Only two patients maintained normal platelet counts without need for corticosteroids or other treatment. These results differ from those in patients with idiopathic thrombocytopenic purpura. Other treatments should be tried before splenectomy is done for thrombocytopenia in patients with systemic lupus erythematosus.

Adult↗

PubMed2006

Lupus-related advanced liver involvement as the initial presentation of systemic lupus erythematosus.

BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE), a prototype of systemic autoimmune disease characterized by multiorgan involvement with diverse clinical and serological manifestations, principally affects women in their child-bearing years. Clinically significant hepatic abnormality as the initial presentation of SLE has rarely been reported. METHODS: Eleven patients with lupus with initial presentation of lupus-related hepatitis were included in this retrospective review. Clinical manifestation, immunological profiles, and risk factors for poor prognosis were analyzed. RESULTS: The most commonly associated clinical manifestations were found to be thrombocytopenia, leukopenia, advancing age, and presence of anti-SSA/Ro antibody and anti-thyroid antibodies. The diagnosis of SLE was delayed due to dominant hepatic abnormalities. Age greater than 50 years and marked hepatic decompensation in accordance with Child classification B and C might suggest poor prognosis (p=0.06). However, the p value was not statistically significant because of the small sample size. CONCLUSIONS: Lupus-related hepatitis, particularly in late-onset lupus, is common. In addition, the presence of anti-SSA, anti-thyroglobulin, and anti-microsomal antibodies is indicative of hepatic involvement in patients with SLE.

Adult↗

PubMedDate not supplied

Altered signaling triggered by ligation of the TCR/CD3 receptor in T lymphocytes from patients with systemic lupus erythematosus: the road from anergy to autoimmunity.

Some of the signaling phenotypic abnormalities observed in peripheral blood T cells from patients with systemic lupus erythematosus resemble those seen in cells anergized by stimulation through the TCR/CD3 complex, in the absence of costimulatory signals, or by stimulation with altered ligands. The potential association of this "anergic" phenotype and the triggering of autoimmune responses in human lupus is discussed.

Autoimmunity↗

PubMed2006

Neuropsychiatric manifestations of systemic lupus erythematosus.

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.

Humans↗

PubMedDate not supplied

[Psychiatric manifestations in patients with erythematosus systemic lupus].

Psychiatric diagnostics were examined in 34 patients with Systemic lupus erythematosus (SLE) sent to Psychiatric Unit of La Paz Hospital. At the moment of interview, the Adaptative disorders were the most frequent (50%), in second place were the Organic psychiatric disorders (38.2). In 24 patients with a follow-up period, all patients had chronic psychiatric disorders and the adaptative disorders were the most frequent too. Twenty patients had neuropsychiatric manifestations. We analyse the difference in SEL with and without nervous system involvement.

Adaptation, Psychological↗