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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with myelofibrosis who also demonstrates a lupus anticoagulant is reported. The presence of a circulating anticoagulant adds to the list of potential hemorrhagic diatheses in myelofibrosis and also demonstrates myelofibrosis to involve a system that may be separate from the myeloid elements of the bone marrow.
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PMID:Lupus anticoagulant in myelofibrosis. 100 85

The marrow findings of 23 lupus patients with pancytopenia were reported. The most common findings were dyserythropoiesis and hypoplasia, both occurring in 9/23 (39%) of the cases. Neither feature was definitely related to cytotoxic drug therapy since most cases were treated by steroids only. Three of the hypoplastic marrows also showed gelatinous transformation, a condition characterized by disruption of marrow architecture, fat atrophy, and deposition of hyaluronic acid. Another common finding was lymphocytosis which occurred in 5/23 (22%) of the cases, 2 of which also had associated plasmacytosis. Two cases were associated with hyperplastic marrow, indicating peripheral destruction of blood cells and compensatory marrow hyperplasia. Reports in the literature on bone marrows in systemic lupus erythematosus are conflicting, describing mainly hypoplasia, vasculitis, plasmacytosis, red cell aplasia, and myelofibrosis. In our series, we found hypoplasia and lymphocytosis/plasmacytosis; in addition we described findings previously unreported: gelatinous transformation, dyserythropoiesis, and marrow hyperplasia.
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PMID:Bone marrow findings in lupus patients with pancytopenia. 206 68

Hematologic abnormalities are common in association with collagen diseases, specially Systemic Lupus Erythematosus and include anemia, neutropenia, thrombocytopenia with alterations in lymphocyte subpopulations. On the other hand, patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis or primary myelofibrosis) have clinical and laboratory evidence of immunologic dysfunction. Clinical findings include the presence of arthritis, vasculitis and erythema nodosum. Laboratory abnormalities include the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs test, elevated latex fixation and a circulating lupus type anticoagulant. Total hemolytic complement markedly depressed has also been reported. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis. A review of the literature revealed that myelofibrosis occurring in the setting of collagen diseases is rare. However, a role for immunologic factors in the pathogenesis of myelofibrosis is also supported by the patients with coincident well defined collagen disease and myelofibrosis. In this report, we present two patients with such an association. Case 1 was a 58-year-old male with a two year duration history of rheumatic arthritis. He had bone erosions on hands, splenomegaly and myelofibrosis. Rheumatoid factor (latex) was positive: 1:2560. He had positive LE cells and hypocomplementemia: 37 CH50/ml (NV 70-150). The patient did not meet criteria for SLE. Case 2 was a 36-year-old female admitted because of dyspnea and fever. Diagnosis of myeloid metaplasia with myelofibrosis and progressive systemic sclerosis had been made four years before hand.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Coexistence of myelofibrosis and collagen diseases]. 213 Feb 12

Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were studied for clinical and laboratory evidence of immunologic dysfunction. Clinical findings included the presence of arthritis, vasculitis, and erythema nodosum. Laboratory abnormalities included the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs tests, elevated latex fixations, and a circulating lupus type anticoagulant. Total hemolytic complement was markedly depressed in four patients. Analysis of complement (C) components C1-C9 and factor B demonstrated significant reduction of only C3 and factor B. By crossed-immunoelectrophoresis, both C3 and factor B, but not C4, were cleaved, indicating that C activation was occurring predominantly via the alternative pathway. The control proteins beta 1H and C3b inactivator were decreased in three of four patients with hypocomplementemia. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis.
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PMID:Immunologic abnormalities in myelofibrosis with activation of the complement system. 691 10

We describe an 18 yr old female with systemic lupus erythematosus presenting with Evans' syndrome (autoimmune hemolytic anemia and immune thrombocytopenia) and dense myelofibrosis. Her clinical course and response to treatment were monitored with regular blood counts, serial measurements of serum procollagen I and III and periodic bone marrow examinations. This report brings to 9 the number of well-documented cases of systemic lupus erythematosis and myelofibrosis in the literature; we discuss the pathogenesis and management of patients with this apparently rare disorder, with particular reference to the role of serum procollagen measurements as markers of myelofibrosis.
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PMID:Evans' syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosis. 853 92

Myelofibrosis has been reported as a rare cause of pancytopenia in patients with autoimmune diseases. We describe a 54y old female patient who was admitted with severe anemia subsequently found to be due to marrow fibrosis. During the course of her hospitalization, relying both on her clinical symptoms as well as the results of a wide range of laboratory tests and diagnostic procedures, the diagnosis of systemic lupus erythematosus was established. The patient was treated with high dose steroids, but improvement of her clinical symptoms as well as normalization of her peripheral blood count were achieved only after high dose intravenous therapy with gamma globulin (IVIG) was instituted. Along with the improvement in the peripheral blood parameters normalization of the bone marrow architecture was recorded on a repeated bone marrow biopsy. IVIG therapy should be considered in extreme cases of bone marrow suppression in SLE.
Lupus 1997
PMID:Successful treatment of early secondary myelofibrosis in SLE with IVIG. 917 29

The purpose of this study was to determine the prevalence and correlates of fibromyalgia (FM) in a prospective, multiethnic systemic lupus (SLE) cohort. A total of 266 SLE patients with disease duration of < or = 5 years at study entry were evaluated longitudinally for the presence of FM (per ACR criteria). Sociodemographic factors, behavioral/psychological variables, clinical features, serologic factors (autoantibodies), and self-reported functioning (MOS SF-36) were ascertained in all patients. Subjects were evaluated at study entry and annually thereafter. The prevalence of FM was then calculated, as was the prevalence of FM-like manifestations (widespread pain with at least 6, but fewer than 11/18 tender points). Variables were evaluated for association with FM or FM-like manifestations by univariate and stepwise logistic regression analyses. FM was present in 14 patients (5%; 9/92 Caucasians (C), 4/109 African Americans (AA), 1/65 Hispanics (H)) and FM/FM-like manifestations in 35 (13%; 16 C, 9 AA, 10 H). There was no difference noted between those with and without FM with respect to gender, education level, income below poverty level, disease activity or damage. By stepwise logistic regression analyses, the strongest association with both FM and FM/FM-like manifestations was a self-reported history of anxiety or affective disorder (P = 0.0237, OR = 4.6 and P = 0.0068, OR = 3.4, respectively). Caucasian ethnicity was strongly associated with FM (P = 0.0066, OR = 7.5) and African American ethnicity was negatively associated with FM/FM-like (P = 0.0204, OR = 0.3). Poorer self-reported physical functioning was associated with FM/FM-like (P = 0.0443, OR = 0.96). FM and FM-like manifestations correlate best with the presence of Caucasian ethnicity, concomitant anxiety or affective disorder, and to a lesser extent with poorer self-reported physical functioning. African American ethnicity is negatively associated with the combination of FM and FM-like manifestations. Clinical measures of disease activity, disease damage, specific organ dysfunction, sociodemographic factors and serologic features are not correlated with FM in this early SLE cohort.
Lupus 2003
PMID:Systemic lupus erythematosus in three ethnic groups: XV. Prevalence and correlates of fibromyalgia. 1272 50

Disease specific measures like systemic lupus erythematosus (SLE) Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics (SLICC) and Systemic Lupus Activity Measure (SLAM) are primarily based on physician assessment of disease severity along with blood tests pertinent to SLE. These are primarily used for research purposes and do not quantify the social impact of the disease or measure function and well being from the patients perspective. We wish to study the degree of correlation between the SLEDAI, SLICC and patient reported health related quality of life (HRQOL), to see if these measures can be used to gauge the disease impact from the patient's perspective. The aim of the study was, therefore, to assess the correlation between SLICC, SLEDAI and HRQOL in patients with systemic lupus erythematosus. We utilized the University of Chicago SLE database to obtain SLEDAI, SLICC and MOS SF-36 scores. A physician not directly involved in their care assessed SLEDAI, SLICC and SF-36 at the same visit. STATA-7SE software was used to obtain the two summary scores [physical component summary (PCS) and mental component summary (MCS)]. Multiple linear regression and correlation coefficients were obtained to assess the direction and relationship between HRQOL and these disease specific measures. On multivariate regression models, both SLICC and SLEDAI were predictive of the PCS scores (beta SLICC = - 1.036, P = 0.025, 95% CI - 1.9, -0.13: beta SLEDAI = - 0.322, P = 0.012, 95% CI -0.57, -0.07. R2= 0.14). Neither SLICC nor SLEDAI were predictive of MCS scores (beta SLICC =-0.015, P=0.97, 95% CI -1.03, 1.001: beta SLEDAI=-0.19, P=0.174, 95% CI -0.47, 0.08. R2 = 0.02). The correlation coefficients between SLEDAI and PCS, MCS were -0.29 and -0.15, respectively. The correlation coefficients between SLICC and PCS, MCS were -0.27 and -0.02, respectively. The conclusions are that SLEDAI and SLICC are poor indicators of HRQOL of patients with SLE.
Lupus 2004
PMID:Can disease specific measures for systemic lupus erythematosus predict patients health related quality of life? 1564 47

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse manifestations. We suggest that intravenous immunoglobulin (IVIg) therapy may be beneficial and safe for various manifestations in SLE. A structured literature search of articles published on the efficacy of IVIg in the treatment of SLE between 1983 and 2005 was conducted. We searched the terms "IVIg," "intravenous immunoglobulin," "lupus," "SLE," and "systemic lupus erythematosus." The various clinical manifestations of SLE that were reported to be successfully treated by IVIg in case reports include autoimmune hemolytic anemia, acquired factor VIII inhibitors, acquired von Willebrand disease, pure red cell aplasia, thrombocytopenia, pancytopenia, myelofibrosis, pneumonitis, pleural effusion, pericarditis, myocarditis, cardiogenic shock, nephritis, end-stage renal disease, encephalitis, neuropsychiatric lupus, psychosis, peripheral neuropathy, polyradiculoneuropathy, and vasculitis. The most extensive experience is with lupus nephritis. There are only a few case series of IVIg use in patients with SLE with various manifestations, in which the response rate to IVIg therapy ranged from 33 to 100%. We suggest that IVIg devoid of sucrose, at a dose of 2 g/kg over a 5-d period given uniformly and at a slow infusion rate in patients without an increased risk for thromboembolic events or renal failure, is a safe and beneficial adjunct therapy for cases of SLE that are resistant to or refuse conventional treatment. The duration of therapy is yet to be established. Controlled trials are warranted.
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PMID:Intravenous immunoglobulin therapy and systemic lupus erythematosus. 1639 97

This study aims to develop a self-administered needs assessment questionnaire for people with systemic lupus erythematosus (SLE), assess its face, content and construct validity and test the reliability of the instrument. Eighty-four people with SLE, registered with a Lupus Resource Centre in New South Wales, Australia participated in a series of focus groups and pre and pilot testing phases in the development of a needs instrument and 594 people from a SLE support association were sent the SLE needs questionnaire (SLENQ) and the MOS-SF-36 and asked to complete both. Face and content validity were found to be high following pre and pilot testing. Principal components analysis identified seven factors with eigenvalues greater than 1, which together accounted for 53% of the total variance (psychological/spiritual/existential, health services, health information, physical, social, daily living and employment/financial needs). Internal reliability coefficients (Cronbach's alpha) of all seven factor-based scales were found to be substantial, ranging from 0.77 to 0.96. Moderately significant correlation between the domains of the SLENQ and the MOS SF-36 were supportive in ascertaining concurrent validity. These findings suggest that the SLENQ provides a reliable and valid index of the global needs of people with SLE.
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PMID:Development and psychometric analysis of the systemic lupus erythematosus needs questionnaire (SLENQ). 1709 57


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