Pericarditis and pleuritis associated with human parvovirus B19 infection in a systemic lupus erythematosus patient

Abstract

Human parvovirus B19 (PVB19) infection sometimes shows systemic lupus erythematosus (SLE)-like symptoms. We present an SLE patient showing pericarditis and pleuritis with a fever and an acute swelling of extremities 2 months after the fist consultation. Initially, a diagnosis of SLE exacerbation was made. Additional laboratory examination showed positive results for immunoglobulin M (IgM) antibody to PVB19 and PVB19 DNA in serum and pleural effusion at that time. After 1 month, PVB19 DNA in serum and IgM antibody to PVB19 was negative. Based on these findings, a final diagnosis of PVB19 infection in an SLE patient was made. PVB19 infection should be taken into consideration for SLE with acute swelling of the extremities and fever, as these symptoms are often observed in adult cases of PVB19 infection. Steroid pulse therapy rapidly improved these symptoms, and later the dose of steroid was reduced to 5 mg/day of prednisolone. Thus, steroids may be one of the choices for severe and/or rapidly progressive symptoms of pericarditis and pleuritis due to PVB19 infection.     

Serum L-ficolin levels in patients with systemic lupus erythematosus

Abstract

Objective L-ficolin plays an important role in innate immunity and is involved in apoptosis. The objective of this study was to investigate the relationship between serum L-ficolin levels and clinical manifestations in patients with systemic lupus erythematosus (SLE).

Methods Serum L-ficolin levels were determined by enzyme-linked immunosorbent assay in 66 SLE patients and 50 healthy controls.

Results Median serum L-ficolin levels were 5.0 and 8.7 μg/ml in SLE patients and controls, respectively (p = 0.0001). There were no significant differences in serum L-ficolin levels between the active disease group [SLE Disease Activity Index (SLEDAI) > 6] and the inactive disease group (SLEDAI < 5). Decreased serum L-ficolin levels were associated with thrombocytopenia (median of with vs. without thrombocytopenia 3.4 vs. 5.3 μg/ml, p = 0.008). There were no correlations between serum L-ficolin levels and SLEDAI, serum C3, or serum C4 levels.

Conclusion The association between L-ficolin and thrombocytopenia suggests a pathogenic role for L-ficolin in thrombocytopenia in SLE.     

Survival in patients with systemic lupus erythematosus in India

Summary Survival in patients with systemic lupus erythematosus (SLE) in developed countries has improved considerably over the last 2 decades. In India, however, survival in patients with SLE reported 5 years ago from this tertiary referral centre was very poor. The present study was conducted to examine trends, if any, towards improvement in the survival of patients with SLE attending the same centre during the period 1981–1990. No statistically significant change in survival was noted. It appears that improvement in the survival of SLE patients would require an overall improvement in the standard of medical care in India.     

系统性红斑狼疮脑病36例临床分析

目的研究系统性红斑狼疮脑病患者临床表现特点。方法分析、归纳2000-01-01~2004-10-10中日友好医院神经内科36例系统性红斑狼疮脑病患者症状、体征、辅助检查。结果36例系统性红斑狼疮脑病患者主诉症状9类,其中头痛、意识障碍、肢体无力居发生率前3位;发现体征13类,其中病理反射、意识障碍、肢体瘫痪居前3位。结论(1)系统性红斑狼疮脑病是活动性系统性红斑狼疮的表现之一。(2)系统性红斑狼疮患者无论病史长短,均可发生系统性红斑狼疮脑病。(3)系统性红斑狼疮脑病表现复杂多样。     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

Abstract

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

Lupus erythematosus (LE) cells in ascites: initial diagnosis of systemic lupus erythematosus by cytological examination: a case report

Systemic lupus erythematosus (SLE) is an autoimmune disease, involving multiple organs. Diverse manifestations may obscure the diagnosis and confuse our thinking process, especially when few clues are present at the beginning. Serositis is one of the various presentations, and the presence of lupus erythematosus (LE) cell in body fluid may be a hint for the final diagnosis of SLE. Herein, we present a young female patient diagnosed of SLE with initial presentation of lupus peritonitis. Finding of LE cell in ascites prompted us for immunologic survey. Diagnosis of SLE was confirmed with high titer of anti-nuclear antibody and antibody to double-stranded DNA. Cytologic examination of body fluid is mainly useful in detecting malignant cells, but high specificity of this marker aids in early diagnosis of SLE.     

Prevalence and classification of headache in patients with systemic lupus erythematosus

Studies on the prevalence of headache in systemic lupus erythematosus (SLE) have shown that it varies from 32 to 78%. The purpose of our study was to determine the prevalence and characteristics of headache in SLE compared with patients with different types of diffuse connective tissue diseases (DCTD) and its relationship with clinical and laboratory manifestations of SLE. We studied patients with SLE (SLE group) and patients with DCTD (control group). All patients were made to answer questionnaire to assess the presence of headache, characterized by at least five episodes of headache during the last year, which was classified according to the International Headache Society criteria. A total of 207 patients were studied, 115 in SLE group and 92 in the control group. The 1-year prevalence of headache was 75.7% in SLE group and 66% in the control group. When the groups were analyzed, 66.1% met the diagnostic criteria for migraine in the SLE group compared with 52.2% in the control group (p=0.04) and 13.9% for tension-type headache in SLE group compared with 16.3% in the control group. The former was the only variable that reached statistical significance comparing the two groups. Both headache and migraine were associated with Raynaud’s phenomenon in SLE patients (odds ratio of 2.80, 95% confidence interval: 1.11–7.05, p=0.02 and odds ratio of 2.34, 95% confidence interval: 1.04–5.23, p=0.03, respectively). These results suggest that headache is a common manifestation in SLE and in other DCTD and we cannot exclude the possibility that it may be related to the emotional stress induced by such clinical situations.     

Nailfold videocapillaroscopy in patients with systemic lupus erythematosus

Nailfold videocapillaroscopy was performed in 21 controls (C) and 21 patients (P) with systemic lupus erythematosus (SLE) classified according to the American College of Rheumatology, with, at least, 1 year of diagnosed disease and having low activity (MEX-SLEDAI) and sequel (SLICC) indexes at the time of the examination, paired by sex and age. Red blood cell velocity (RBCV, mm/s) at rest and after the release of 60s arterial occlusion (RBCV_max, mm/s), time to reach it (TRBCV_max, s), functional capillary density (FCD, number of capillaries /mm²), afferent, apical and efferent capillary diameters (μm) (DAF, DAP and DEF, respectively) were obtained from videotapes analyzed by the CapImage software. The results did not show any significant difference between the groups that were analyzed, suggesting that morphological (capillary diameters) and functional (RBCV, RBCV_max, TRBCV_max and FCD) parameters are not affected by SLE when low activity and sequel indexes of the disease are present.     

IgG therapy in systemic lupus erythematosus

Summary Two paediatric patients with systemic lupus erythematosus were treated with immunoglobulin G (IgG). In the first case treatment resulted in regression of the most acute symptoms and a long remission was achieved. In the second patient, who was treated during the chronic stage of the disease, there was no significant effect on the course of the SLE.Abbreviations ESR Erythrocyte sedimentation rate - T Thoracic vertebra - DNA Deoxyribonucleic acid - HLA HLA system (of histocompatibility loci) - IgG Immunoglobulin G - RES Reticuloendothelial system - SLE Systemic lupus erythematosus - CNS Central nervous system     

Genetic variation in the interleukin-10 gene promoter in Polish patients with systemic lupus erythematosus

Identification of susceptibility genes in systemic lupus erythematosus (SLE) has recently become a topic of interest. The IL-10 promoter contains three single base-pair substitutions at −627C > A, −854C > T and −1117G > A. These single base-pair substitutions produce three different haplotypes, GCC, ACC and ATA, which affect IL-10 expression. We examined the distribution of −627C > A, −854C > T and −1117G > A IL-10 promoter polymorphisms in patients with SLE (n = 103, women only) and matched controls (n = 300). Despite the higher prevalence of the GCC/GCC, GCC/ATA and ATA/ATA genotypes in SLE patients than in controls, we observed that only GCC/GCC genotype frequency distribution was significant between these groups. We observed that women with the GCC/GCC genotype displayed an approximately twofold increased risk of SLE OR = 2.245 (95% CI = 1.354–3.721, P = 0.0022). We did not find any associations between various genotypes of IL-10 promoter haplotypes and clinical manifestations or autoantibody production in patients with SLE. Our observations indicate that the GCC/GCC promoter genotype may contribute to SLE incidence in Polish patients.     

Anorexia nervosa in a patient with systemic lupus erythematosus

Summary Anorexia nervosa has not been described in patients with systemic lupus erythematosus (SLE). The case of a patient with SLE who developed the classical features of anorexia nervosa is described. Although an impressive interplay between psychosocial and cultural factors was present, the possibility that this syndrome was produced by active CNS vasculitis remained.     

Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias

We studied 21 bone marrow specimens from 21 patients with systemic lupus erythematosus (SLE) and peripheral cytopenias: anaemia (Hb<10 g/dl), and/or leucopenia (white blood cell count <4×10⁹/l), and/or thrombocytopenia (platelets <150×10⁹/l). None of the patients had used immunosuppressive drugs in the 2 months before the study, and 11 (52.4%) had never used these drugs. The global and specific series cellularity, degree of fibrosis and necrosis were evaluated by bone marrow trephine; morphological abnormalities and iron stores were evaluated by cytological smears. The most important abnormalities viewed in bone marrow biopsies were: global hypocellularity (47.6%), increased reticulin proliferation (76.2%) with myelofibrosis in one patient, and necrosis (19.0%). The marrow aspirates were difficult to obtain in four patients, who showed an increased reticulin proliferation on histological analysis. Plasmocytosis was present in 26.7% of cases and in one there was a serum monoclonal component (IgG kappa). Iron stores were normal or increased in 26.7% of specimens and decreased or absent in 73.3%. The most frequent peripheral abnormality was leucopenia in 90.4% (19/21) and granulocytic hypoplasia was observed in 47.3% (9/19) of these patients. We conclude that the bone marrow may be a target organ in SLE with cytopenias.     

Short-term add-on tocilizumab and intravenous cyclophosphamide exhibited a remission-inducing effect in a patient with systemic lupus erythematosus with refractory multiorgan involvements including massive pericarditis and glomerulonephritis

We report on a 41-year-old woman with refractory systemic lupus erythematosus with massive pericarditis, macrophage activation syndrome, and glomerulonephritis despite high-dose glucocorticoids and tacrolimus. Tocilizumab dramatically improved pericarditis, and glomerulonephritis was controlled after adding cyclophosphamide. We had to halt tocilizumab and cyclophosphamide due to possible pneumocystis infection after five and three infusions of tocilizumab and intravenous cyclophosphamide, respectively. Nevertheless, no lupus flare had been observed on glucocorticoid monotherapy and enabled further rapid tapering prednisolone.     

Treatment adherence in patients with rheumatoid arthritis and systemic lupus erythematosus

This study assessed self-reported adherence in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) from underserved healthcare settings. We conducted a cross-sectional survey of 102 ethnically diverse patients--70 with RA and 32 with SLE--attending rheumatology clinics at publicly funded hospitals in Houston, Texas; 43% were Hispanic, 32% African-American, and 25% White. Treatment adherence was evaluated using the compliance questionnaire rheumatology (CQR; 0, low adherence and 100, high adherence) and the questionnaire of the Adult AIDS Clinical Trials Group (AACTG). The patients were also asked how often they forgot to take their prescribed medications or discontinued them on their own. Mean patient age was 48.5 years; 75% were female, 32% were African-American, 43% Hispanic, and 25% White. Only one third reported never forgetting to take their medications; 40% reported having stopped their medications on their own because of side effects, and 20% because of lack of efficacy. Mean CQR score was 69.1 +/- 10.5, suggesting moderate adherence overall. Differences were also observed across ethnic groups: 23% of ethnic minority patients had problems taking their medications at specified times compared to 11% of Whites (p = 0.03). Lower education and side effects were associated with lower adherence. No differences were observed between RA and SLE patients. Many patients with RA and SLE report problems with treatment adherence. These appear to be more prevalent in African Americans and Hispanics than Whites; the impact of decreased adherence on outcomes could be significant and should be considered when treating patients with RA and SLE.     

Myocarditis in systemic lupus erythematosus

Although clinical manifestations of myocarditis in systemic lupus erythematosus are uncommon, noninvasive cardiac testing may detect subclinical cases. The pathogenesis of myocarditis in systemic lupus erythematosus has been ascribed to many factors, including autoimmunity, medications, and coexisting diseases. Lupus myocarditis merits urgent clinical attention because of the likely progression to arrhythmias, conduction disturbances and heart block, dilated cardiomyopathy, and heart failure. Endomyocardial biopsy can be used to identify the underlying inflammatory histopathology. Usual therapy includes high-dose corticosteroids, in addition to standard cardiac medications.     

Pilot clinical study of Adacolumn cytapheresis in patients with systemic lupus erythematosus

The aim of this study is to investigate the clinical effects of cytapheresis using the Adacolumn system (selective removal of circulating monocytes and granulocytes by means of an extracorporeal type column) in patients with active systemic lupus erythematosus (SLE). An open uncontrolled multicenter pilot study was conducted in 18 SLE patients who were showing a SLEDAI score of 8 or more under conventional medication. Patients with lupus nephritis (>class 1, WHO classification) were excluded. Extracorporeal cytapheresis with the Adacolumn system was administered once a week for five consecutive weeks. The efficacy of the treatment was evaluated using the SLEDAI for 10 weeks after the first cytapheresis session. The median SLEDAI decreased from 16 at baseline to six at week 11 (10 weeks after the first apheresis) (p<0.001). Significant improvements in musculoskeletal and dermal systems were observed. Arthritis and alopecia were present in 14 and nine patients at baseline and this number decreased to five and one patients, respectively by week 11.Three mild and one moderate adverse events out of the 42 reported events were judged ‘probably related’ to the treatment; no serious adverse events were reported. Selective removal of monocytes and granulocytes from the blood in an extracorporeal circulation system was associated with clinical improvement in this small series of patients with SLE. Since this approach seems not to have the disadvantages of pharmacological immunosuppression, further controlled studies of Adacolumn cytapheresis are warranted in SLE.     

Autonomic dysfunction in systemic lupus erythematosus

The objectives were to study the frequency and pattern of autonomic dysfunction in systemic lupus erythematosus (SLE). Fifty-one patients of SLE and 30 age- and sex-matched healthy controls were studied prospectively using a standard battery of noninvasive tests. Autonomic symptoms were seen in 37% patients. On laboratory testing incipient dysfunction was seen in 9 (18%) cases and 1 (3%) control, while atypical involvement was seen in 11 (21%) cases and 6 (20%) controls. Autonomic dysfunction did not correlate with disease duration, lupus activity, disease damage, any particular organ involvement or the presence/absence of peripheral neuropathy. Autonomic neuropathy is not uncommon in lupus and may exist independent of peripheral neuropathy. There are no specific clinical predictors. The clinical significance of autonomic dysfunction detected by laboratory testing warrants longitudinal studies.