Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Severe acute respiratory syndrome (SARS) is a serious respiratory illness caused by a novel human coronavirus. The disease is highly infectious and carries significant mortality and morbidity. There was a major outbreak of SARS in Guangdong, Taiwan, Beijing, Hong Kong and Toronto between March and June 2003. Common presenting features of SARS are high fever, chills, rigor, malaise, nonproductive cough, lymphopenia and pulmonary infiltrates, followed by rapidly progressive respiratory failure in some cases. We describe two patients with systemic lupus erythematosus (SLE) who presented with fever, systemic upset and pulmonary infiltrates between April and June, 2003. One patient was confirmed to have coronavirus pneumonia while the other had active SLE with lung involvement. Our cases illustrate the difficult diagnostic dilemma in the evaluation of febrile SLE patients during the SARS epidemic.
Lupus 2004
PMID:Lupus pneumonitis or severe acute respiratory syndrome? 1535 29

A prospective study was made of 93 patients with antiphospholipid syndrome (APS), including 34 patients with primary APS (PAPS) and 59 with secondary APS (SAPS) associated with exanthematous lupus erythematosus (ELE). According to the clinical outcomes, PAPS is heterogeneous since the clinical and laboratory signs of ELE or lupus-like syndrome occurred in two thirds of the patients with PAPS. The clinical manifestations of PAPS and ELE-associated SAPS differed at the height of disease: the signs of vascular diseases were prevalent in patients with PAPS and ELE-associated systemic manifestations were predominant in those with SAPS. In the patients with APS who further developed ELE, unlike those with PAPS showed a high incidence of transient erythema and arthralgias. The levels of serological markers in patients with APS at its height did not differ. As compared with healthy individuals, patients with APS were found to have the similar changes in the nonspecific immunological parameters manifested by absolute lymphopenia, by the diminished Fc-phagocytosis of monocytes and neutrophils, by the elevated levels of circulating immune complexes, and by the high biocidal activity of neutrophils. There were no significant differences in the immunity of patients with PAPS and in those with SAPS.
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PMID:[Clinical and immunological characteristics of primary and secondary antiphospholipid syndrome]. 1546 24

We have conducted a thorough literature review to evaluate the relative value of the hematologic criterion in making a diagnosis of systemic lupus erythematosus (SLE), its clinical relevance, and its prognostic significance. In the updated 1982 ACR criteria, the presence of one or more of the four elements: 1) hemolytic anemia (with reticulocytosis); 2) leukopenia (<4000/microL on two or more occasions); 3) lymphopenia (< 1500/microL on two or more occasions); or 4) thrombocytopenia (< 100,000/microL in the absence of offending drugs) is now considered as a single hematologic disorder. The sensitivity and specificity of the individual elements of the hematologic criterion range from 18 to 46% and 89 to 99%, respectively. The accuracy of the hematologic criterion requires proper interpretation. For example, many studies reported the presence of anemia that was not clearly defined and likely included anemia from etiologies other than hemolytic anemia, thereby causing an overestimation of the prevalence. In addition, medications such as corticosteroids and cytotoxic agents, and viral infections, can also contribute to a reduction in lymphocyte count. Despite these limitations, the SLICC committee recommends no change in the elements of the hematologic criterion when this criterion is properly interpreted and other causes of cytopenia are excluded.
Lupus 2004
PMID:Review of ACR hematologic criteria in systemic lupus erythematosus. 1558 Sep 84

Previous studies suggest that the forkhead transcription factor Foxj1 inhibits spontaneous autoimmunity in part by antagonizing NF-kappaB activation. To test this hypothesis, we ectopically expressed Foxj1 in the T cells of lupus-prone MRL/lpr mice by backcrossing a CD2-Foxj1 transgene against the MRL/lpr background. Strikingly, CD2-Foxj1-MRL/lpr animals showed a significant reduction in lymphadenopathy, pathogenic autoantibodies, and end-organ disease-but surprisingly, reversion of autoimmunity was not attributable to modulation of NF-kappaB. Instead, CD2-Foxj1 transgenic mice exhibited a peripheral T cell lymphopenia, associated with an accumulation of mature single-positive thymocytes. Transgenic thymocytes demonstrated unimpaired lymphoid organ entry in adoptive transfer studies but demonstrated impaired thymic exodus in response to CCL19, apparently independent of CCR7, S1P1, and NF-kappaB. These findings confirm the importance of Foxj1 in the regulation of T cell tolerance but furthermore suggest a novel and specific role for Foxj1 in regulating thymic egress.
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PMID:Cutting edge: Foxj1 protects against autoimmunity and inhibits thymocyte egress. 1633 15

The diaphragm is an unusual localization for disseminated erythematous lupus and is generally not recognized. We present the case of a 34-year-old woman who developed bilateral pain in the base of the thorax 15 days before hospitalization without any bronchial signs and NYHA stage II dyspnea. The patient's general health status remained satisfactory and the physical examination was normal except for diffuse inflammatory joint pain and cutaneous photosensitivization. The chest x-ray disclosed ascension of both hemidiaphragms with retracted lungs associated with bilateral basal atalectasia. The diagnosis of lupus with diaphragmatic involvement was retained due to the clinical presentation with diffuse joint pain, photosensitization with facial erythema, pericardial effusion and elevated antinuclear antibody and lymphopenia (1 100/mm3). Lung function tests revealed a restrictive syndrome. Oral corticosteroids 1 mg/kg/d enabled clinical and functional improvement. In light of this observation we discuss the pathogenic mechanisms of this uncommon localization of lupus and the difficulty of establishing a sure diagnosis.
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PMID:[Disseminated erythematous lupus with bilateral diaphragmatic involvement]. 1684 Sep 98

CD55 and CD59 are glycophosphatidylinositol-anchored proteins with complement inhibitory properties. Lymphopenia in systemic lupus erythematosus (SLE) has been associated with autoantibodies targeting nuclear antigens. The aim of this study was to evaluate the surface density of CD55 and CD59 in T and B lymphocytes from patients with SLE and lymphopenia and its possible correlation with the presence of common SLE autoantibodies. Flow cytometric analyses were performed on CD55 and CD59 stained CD3+ and CD19+ cells from 40 SLE patients, 30 with lymphopenia and 10 without it, and 25 healthy controls. Autoantibodies were detected in the sera by enzyme linked immunosorbent assay. The mean fluorescence intensity of CD55 and CD59 in T and B cells was significantly diminished in SLE patients with lymphopenia when compared with healthy subjects. Interestingly, the opposite was found in T and B cells from non-lymphopenic SLE patients. Although there was no correlation between CD55 and CD59 surface density and the presence of any specificity of the autoantibodies tested, higher titres of anti-dsDNA, anti-SM and anti-ribosomal p antibodies were significantly associated with lymphopenia. The deficiency of CD55 and CD59 expression may play a role in the pathophysiology of lymphopenia, most likely by increasing the susceptibility of cells to complement mediated cytolysis.
Lupus 2006
PMID:Diminished expression of complement regulatory proteins (CD55 and CD59) in lymphocytes from systemic lupus erythematosus patients with lymphopenia. 1708 Sep 16

Thirty silent lupus nephritis (SLN) patients were compared to 16 individuals bearing overt lupus nephritis (OLN). Results included: years of systemic lupus erythematosus (SLE) diagnosis were significantly earlier (4.6 +/- 2.8 years) in SLN than in OLN (7.18 +/- 3.61) (P < 0.05). Neurological compromise, hypertension, normocitic anemia and lymphopenia were significantly prevalent in OLN than in SLN (P < 0.05). Beside normal urinary sediment and renal function tests, the SLN group showed a moderate increase of both activity (AI) and chronicity (CI) renal pathology index when compared to highly increased AI and CI in OLN (P < 0.05). Seventy percent of SLN patients were ISN/RPS Classes I (6.6%) and II (63.3%) while 81% of OLN cases were Classes III, IV (37.5%) and V. IgG, IgA, IgM, lambda chain, C3 and fibrinogen immune deposits were found in 90% or over in both SLN and OLN individuals while in 60% or over, both groups also showed kappa chain, Clq and C4 deposits. While prevalence of ANA, anti-dsDNA and anti-C1q antibodies were similar in both groups, anti-histone, anti-RNP, CIC and CH50 serum levels were significantly different in OLN versus SLN (P < 0.05). We strongly suggest that indeed SLN is the earliest stage in the natural history of lupus nephritis.
Lupus 2006
PMID:Further description of early clinically silent lupus nephritis. 1721 89

The aim of this study was to determine the association between lupus autoantibodies and the clinical manifestations and outcome in a cohort of Puerto Ricans patients with systemic lupus erythematosus (SLE). All patients fulfilled the American College of Rheumatology classification criteria for SLE. Demographic parameters, clinical manifestations over time and damage accrual were obtained at the last study visit. Disease damage was assessed with the Systemic Lupus International Collaborating Clinics Damage Index (SDI). ANA, ANA pattern, and anti-dsDNA, anti-Smith, anti-Ro (SSA), anti-La (SSB) and anti-snRNP antibodies were measured at the time of SLE diagnosis. Chi-square test, Fisher exact test, ANOVA, logistic regression and general lineal model analyses were used to evaluate these associations. Ninety-six percent of patients were females. The cohort had a mean age of 40.2 +/- 12.0 years and mean disease duration of 9.6 +/- 7.0 years. Patients with elevated anti-dsDNA antibodies were more likely to have vasculitis, pericardial effusion, renal involvement, anaemia, leukopenia, lymphopenia and thrombocytopenia. Anti-Smith antibodies were positively associated with skin ulcerations, elevated liver enzymes, renal involvement and thrombocytopenia. Anti-Ro antibodies were related with the presence of discoid lupus, serositis, pneumonitis, elevated liver enzymes, hemolytic anaemia, leukopenia and lymphopenia. No positive associations were found for anti-snRNP or anti-La antibodies. The presence of anti-dsDNA, anti-Smith and anti-Ro antibodies was associated with higher SDI scores. In conclusion, anti-dsDNA, anti-Smith and anti-Ro antibodies are associated with several clinical manifestations and more damage accrual in Puerto Ricans with SLE. These findings provide valuable clinical and prognostic information for this ethnic population.
Lupus 2006
PMID:Clinical and prognostic value of autoantibodies in puerto Ricans with systemic lupus erythematosus. 1721 98

We investigated whether premature telomeric loss occurred in peripheral polymorphonuclear neutrophils (PMN) as well as mononuclear cells (MNC) from patients with systemic lupus erythematosus (SLE). We measured the telomere length of MNC and PMN in 60 SLE patients and 26 sex-, race- and age-matched healthy volunteers by Southern blotting with chemiluminescence method. The possible predisposing factors associated with telomere change were also analysed. We found the telomere length of MNC and PMN shortened with age in different degrees in both SLE and control groups. Compared to the control group, the telomere length was shortened in both SLE-MNC (6.08 kb in SLE versus 6.71 kb in control, P = 0.0008) and PMN (6.24 kb in SLE versus 6.75 kb in control, P = 0.0025). The average reduction in telomere length in SLE patients was equivalent to a premature senescence of 16.5 years in MNC and 13.4 years in PMN. In addition, the accelerated telomere shortening was more prominent in SLE patients younger than 45 years old. SLE disease activity (SLEDAI) contributed remarkably to the accelerated telomere erosion, at least in PMN. Moreover, the telomere length of MNC was significantly shorter than PMN in the same SLE patients with leukopenia and lymphopenia. These data suggested that MNC and PMN from patients with SLE displayed premature and accelerated telomere shortening that SLE is an independent factor for it.
Lupus 2007
PMID:Premature telomere shortening in polymorphonuclear neutrophils from patients with systemic lupus erythematosus is related to the lupus disease activity. 1743 33

We describe a 13-year-old systemic lupus erythematous (SLE) patient who presented with severe headache. The diagnosis of pseudotumor cerebri (PTC) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, including magnetic resonance (MR) venography. She later developed a Coombs positive anemia, lymphopenia, positive tests for antinuclear antibody (ANA) and anti-dsDNA and a migratory polyarthritis confirming the diagnosis of SLE. IgM type anticardiolipin antibodies were positive in low titer. Since she did not have a demonstrable thromboembolic phenomenon in neuroimaging studies, a diagnosis of antiphospholipid antibody syndrome could not be made and anticoagulant treatment was not given. Treatment with pulse i.v. methylprednisolone followed by oral treatment along with azathioprine produced a rapid and dramatic resolution of the clinical symptoms. PTC may also be a neurological manifestation of childhood SLE and should be considered in the differential diagnosis. We suggest that pulse steroids and azathioprine is an effective treatment for this feature.
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PMID:Systemic lupus erythematosus presenting with pseudotumor cerebri: a rare association. 1747 54


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