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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.
Lupus 1991 Nov
PMID:Neonatal lupus erythematosus with multisystem organ involvement preceding cutaneous lesions. 184 64

Over the past 50 years, survival has improved in patients with systemic lupus erythematosus and associated nephritis. Yet, there are few long-term outcome studies in patients with well-defined nephropathy. We examined the outcome of 439 patients with lupus nephritis who were seen at the Mayo Clinic between 1964 and 1986 in whom renal biopsies were assessed using the World Health Organization (WHO) classification. There were 341 women and 98 men (mean +/- s.d., age 33.5 +/- 14 years); 200 (46%) patients were hypertensive and 249 (57%) had impaired renal function at renal biopsy. All WHO morphologic classes were represented and 339 (77%) patients had class III, IV and V (the more severe forms of nephritis). Follow-up averaged 10.2 years per patient. At last contact, 286 (65%) patients were alive and 153 (35%) were dead. Overall patient survival was 80%, 69% and 53% at 5, 10 and 20 years after biopsy that was significantly worse than expected survival (P < 0.001). Ten-year cumulative patient survival improved comparing earlier to more recent time spans: 64% in 231 patients seen during 1964-75; 76% in 2089 patients studied during 1976-86 (P = 0.03). Survival free of renal failure was 83%, 74% and 64% at 5, 10 and 20 years, and survival was unfavorably influenced by progressive WHO class, hypertension, impaired renal function, nephrotic range proteinuria, hypoalbuminemia and anemia. Multivariate analysis found impaired renal function, increased urine protein, anemia and younger age to be independent predictors of renal failure. WHO class was not a significant predictor when adjusted for these four factors. Cardiovascular events accounted for 48% of the known deaths and were equally distributed across all WHO classes, followed by infections, renal failure, malignancy, respiratory failure and gastrointestinal bleeding.(ABSTRACT TRUNCATED AT 250 WORDS)
Lupus 1995 Apr
PMID:Prognostic determinants in lupus nephritis: a long-term clinicopathologic study. 779 13

As the co-existence of pernicious anaemia (PA) and systemic lupus erythematosus (SLE) has been repeatedly reported, we have investigated the presence of anti-intrinsic factor antibodies (IFAb), the immunological hallmark of PA, in patients diagnosed with SLE. Serum cobalamin levels and IFAb were determined in 30 women diagnosed with SLE as well as in 45 controls. Cobalamin levels were low in 7/30 patients. IFAb were detected in 3/30 sera from patients but in none of the control sera. The presence of IFAb was associated with a low cobalamin concentration, anaemia and macrocytosis in only one patient. There was no evident relationship between the presence of IFAb and serological markers of SLE. We conclude that IFAb may appear in SLE patients, although the cobalamin deficiency described in SLE seems to be due to the presence of IFAb in only a minority of cases.
Lupus 1993 Apr
PMID:Anti-intrinsic factor antibodies in systemic lupus erythematosus. 833 31

Cytokines are believed to play an important role in the pathogenesis of systemic lupus erythematosus (SLE). However, for tumour necrosis factor alpha (TNF-alpha) both beneficial and deleterious effects have been reported. To obtain information about the involvement of this cytokine in the pathophysiology of SLE, serum levels of TNF-alpha, the soluble forms of the 55 and 75 kDa tumour necrosis factor receptors (TNF-R55 and TNF-R75), and interleukin-6 (IL-6) were measured by ELISA in nine female patients over a period of 2 yr. Compared to healthy controls, levels of TNF-alpha (median 47 pg/ml, range < 15-222 pg/ml), TNF-R55 (median 1.9 ng/ml, range 0.8-10.8 ng/ml), TNF-R75 (median 4.7 ng/ml, range 1.5-15 ng/ml) and IL-6 (median 3.5 pg/ml, range < 3.5-52 pg/ml) were significantly elevated in SLE patients (P < 0.0001 vs controls in all cases). There were strong correlations between TNF-alpha and its soluble receptors (P < 0.0001). Moreover, TNF-alpha and both TNF-Rs strongly correlated with clinical and serological parameters of disease activity, such as the European Consensus Lupus Activity Measurement (ECLAM) score, anti-dsDNA antibodies, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and anaemia (P < 0.0001 for all comparisons). TNF-alpha and TNF-R75 also correlated with IL-6 (P < 0.0001). However, no correlation between IL-6 and ECLAM was found, and the correlation of IL-6 with anti-dsDNA was relatively weak; in contrast, IL-6 correlated strongly with CRP and ESR (P < 0.0001). Although these data do not allow us ultimately to discriminate between beneficial and deleterious effects of TNF-alpha, they nevertheless suggest a central role for the TNF system in the pathophysiology of SLE.
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PMID:Tumour necrosis factor alpha and its soluble receptors parallel clinical disease and autoimmune activity in systemic lupus erythematosus. 894 91

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

Vascular damage is a well known cause of hypopituitarism since Sheehan's report of postpartum pituitary necrosis; it has subsequently been reported that also sickle-cell anemia, eclampsia, pituitary apoplexy and other pathologies may induce failure of the anterior hypophysis through this mechanism. The antiphospholipid syndrome (APS) is characterized by widespread arterial and venous thrombosis with resulting different clinical features; Addison's disease due to adrenal thrombosis is the only endocrine involvement reported so far in this syndrome. We report here a case of global anterior pituitary insufficiency which developed soon after cerebral ischaemic stroke in a 62 year aged woman with Lupus aicoagulant activity (LAC) and large atrial thrombosis; underlying pathologies were excluded by appropriate investigations. Therefore in our opinion this is the first case in which anterior hypopituitarism is reported in the clinical constellation of APS and the second type of endocline involvement.
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PMID:Hypopituitarism and antiphospholipid syndrome. 958 17

To gain a better understanding of systemic lupus erythematosus (SLE) in Puerto Ricans we studied the clinical and serologic manifestations in a cohort of 134 patients. The female to male ratio was 18:1. Mean age at diagnosis was 32 +/- 12 y. The mean duration of disease and follow-up were 7.4 +/- 6.0 and 5.8 +/- 6.0 years respectively. Mortality was 3%. Photosensitivity (76.9%) and malar rash (71.9%) were the most common clinical manifestations. Arthritis was observed in 67.5% of patients. Anemia was seen in 67.2% of patients, but only 12.7% had autoimmune hemolytic anemia. Leukopenia (41.8%) and lymphopenia (64.9%) were also common. Serositis was observed in only 28%. Severe kidney damage such as nephrotic syndrome (14.2%) or renal failure (4%) was infrequent. Cardiovascular (12.7%) and neurologic (9.0%) manifestations were also uncommon. Antinuclear antibodies (ANA) were detected in 93.3%, anti-dsDNA antibodies in 54.5%, anti-Ro antibodies in 30.1% and anti-La antibodies in 14.2%. Low C3 and low C4 were observed in 38.3% and 35.7% respectively. This study suggests that Puerto Ricans with SLE present a mild form of disease predominantly manifested by cutaneous, musculoskeletal and hematologic involvement, but low prevalence of major organ damage and low mortality.
Lupus 1999
PMID:Clinical and immunological manifestations in 134 Puerto Rican patients with systemic lupus erythematosus. 1041 6

Kikuchi's disease (KD) can occur in association with systemic lupus erythematosus (SLE). The treatment of concomitant diseases, however, is unclear. We describe a case of a 45-y-old man who presented with generalized histiocytic necrotizing lymphadenitis, fever, malaise and weight loss. Ten months later he also developed arthritis, serositis, anemia, leukopenia and lymphopenia. ANA, anti-Smith, anti-snRNP and anti-Ro antibodies were positive. He responded rapidly and favorably to mid-dose prednisone. Hydroxychloroquine, added 5 months later, allowed tapering down and discontinuation of prednisone treatment. He has remained in complete remission for 5 years.
Lupus 2001
PMID:Therapeutic response and long-term follow-up in a systemic lupus erythematosus patient presenting with Kikuchi's disease. 1123 25

Data related to the disease course of patients with systemic lupus erythematosus (SLE) with special attention to the persistence of disease activity in the long term are scarce. At this moment reliable figures are only known about the survival rate as a measure of outcome. The aim of this multicenter study was to describe the outcome of SLE patients with a disease duration of greater than 10 y. Outcome parameters were two disease activity-scoring systems (SLEDAI and ECLAM), the end organ damage (SLICC/ACR damage index) and treatment. Our results are derived from 187 SLE patients followed at 10 different centres in Europe over a period of 1 y. Serious clinical signs or exacerbations, defined by the occurrence or detoriation of already existing symptoms of renal and cerebral nervous systems were observed in 2-11% of the patients, seizures and psychosis in 3%, proteinuria in 11% and an increase in serum creatinine in 5% of the patients. No change took place in the overall damage index. Yet, the disease course in most patients was characterized by periods of tiredness (42-60%), arthritis (20-25%), skin involvement such as malar rash (32-40%), migraine (15-20%), anaemia (15%) and leucopenia (17-19%). Summarizing these results it is shown that patients, still under care after such a long time of having this disease, do have a disease that is far from extinguished.
Lupus 2001
PMID:Systemic lupus erythematosus. Disease outcome in patients with a disease duration of at least 10 years: second evaluation. 1124 10

Recent accumulated evidence suggests that prolactin (PRL) is an important immunomodulator and plays a part in the pathogenesis of systemic lupus erythematosus (SLE). The current study assessed the frequency of hyperprolactinaemia in patients with SLE and its association with defined clinical manifestations or serological abnormalities. PRL levels were analysed in 60 patients with SLE including a follow-up of 20 patients, 18 patients with rheumatic autoimmune diseases other than SLE (AID) and in 47 normal healthy subjects (NHS) using ELISA. Clinical manifestations and disease activity (ECLAM) were recorded. Autoantibodies (anti-dsDNA, anti-CL) were determined by standard techniques. In all, 28.3% of the patients with SLE had raised serum PRL. Their PRL levels (17.4+/-15.1 ng/ml, P<0.0001) and those of patients with AID (13.1+/-10.3 ng/ml, P<0.001) were significantly higher compared to NHS (6.3+/-3.2 ng/ml). Anti-dsDNA (r(s) = 0.3, P = 0.04) and anti-CL antibody titres (IgG; r(s) = 0.3, P = 0.03) correlated with PRL level. Furthermore, elevated erytthrocyte sedimentation rate (ESR), anaemia, decrease in C3, fatigue, fever and renal involvement were associated with hyperprolactinaemia. These results were confirmed by follow-up examinations. Moderate hyperprolactinaemia is present in a subset of patients with SLE and serum PRL correlates with clinical and serological disease activity.
Lupus 2001
PMID:Enhanced serum prolactin (PRL) in patients with systemic lupus erythematosus: PRL levels are related to the disease activity. 1153 Sep 97


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