Splenic infarction in a pregnant woman with systemic lupus erythematosus

Here we describe a 20-year-old pregnant woman with systemic lupus erythematosus who had high anticardiolipin antibodies and presented with splenic infarction.     

Thrombotic disease in systemic lupus erythematosus is associated with a maintained systemic platelet activation

Patients with systemic lupus erythematosus (SLE) have an increased risk of thrombosis. Platelet-induced extracellular phosphorylation of plasma proteins suggests that this is due to persistent activation of the platelets. We examined 30 SLE patients (15 with thrombotic disease), 18 non-SLE patients with deep vein thrombosis (DVT) and 50 healthy controls by analysing beta-thromboglobulin, activated factor XI-antithrombin complexes and fibrinogen-bound phosphate. All parameters were elevated in SLE patients, particularly those with thrombosis, but normal in DVT cases and healthy controls. We conclude that thrombotic disease in SLE patients is associated with a persistent systemic platelet activation that may lower the threshold for induction of thrombosis.     

Anti‐nucleosome antibodies as a disease activity marker in patients with systemic lupus erythematosus

Aim: To measure the level of anti‐nucleosome antibodies in systemic lupus erythematosus (SLE) patients, to determine the sensitivity and the specificity of these antibodies in the diagnosis of the disease and to evaluate the relationship between the levels of anti‐nucleosome antibodies, anti‐dsDNA (double‐stranded DNA) and SLE disease activity. Methods: A cross‐sectional study was conducted. All patients attended either a medical specialist clinic or were admitted to the medical wards of Hospital Universiti Sains Malaysia with the diagnosis of SLE (n = 90), other connective tissue diseases (n = 45) or were normal controls (n = 90) within the period from July 2004 until September 2005. They were tested for anti‐nucleosome antibodies by enzyme‐linked immunosorbent assay and anti‐DNA antibodies by immunofluorescence. SLE disease activity was evaluated by SLE disease activity index (SLEDAI) score. Results: Out of 90 SLE patients, anti‐nucleosome antibodies were positive in 47 (52.2%) patients, whereas these antibodies were positive in three (6.7%) patients with other connective tissue diseases. Anti‐dsDNA antibodies were positive in 33 (36.7%) SLE patients, whereas these antibodies were positive in four (8.9%) patients with other connective tissue diseases. Anti‐nucleosome antibodies were positive in 40 (97.6%) patients with active SLE, whereas these antibodies were positive in seven (14.3%) patients with inactive SLE. Anti‐nucleosome antibodies had a stronger correlation than anti‐dsDNA antibodies with SLEDAI score. There was a significant association between anti‐nucleosome antibodies and disease activity. Conclusion: Anti‐nucleosome antibodies test is highly sensitive and specific for the diagnosis of SLE, especially when the anti‐dsDNA antibodies are absent. They are additional disease activity markers in the assessment of SLE disease activity.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Abstract

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

Early morning neutropenia in a patient with systemic lupus erythematosus

Abstract

An autopsy case of an 11-year-old boy with polyarteritis nodosa is described in which the onset of the disease was associated with the presence of hepatitis B (HB) antigens (Ag) in the cytoplasm and nuclei of hepatocytes as detected by immunohistological methods. Deposits of HBsAg, HBeAg, IgG, IgM, C3, and C1q were demonstrated in systemic vascular lesions. It is considered that the arteritis was due to deposition in the arteries of immune complexes formed by HBAg and HB antibodies.     

Antiendothelial cell antibodies in systemic lupus erythematosus

Objective: Anti‐endothelial cell antibodies (AECAs) are a heterogeneous group of antibodies against a variety of antigenic determinants on endothelial cells (EC). AECAs are known to play an immunopathogenic role in triggering EC activation, leading to vascular damage. The purpose of this study was to assess: (i) the incidence of AECAs in systemic lupus erythematosus (SLE) patients with nephritis (LN) and to compare this with SLE patients without clinical evidence of nephritis; and (ii) to understand the association of AECAs with disease severity based on renal histopathological findings. Method: Fifty‐three clinically and histopathologically proven cases of LN were studied along with 20 patients without evidence of nephritis. AECAs were detected by immunofluorescence using cultured human umbilical vein endothelial cells (HUVECs). The titres and immunoglobulin subclass of AECAs were also identified. Other autoantibodies were also detected. Results: In the LN group, 21 (39.6%) were AECA positive and 19 (35.8%) were antineutrophil cytoplasmic antibody (ANCA) positive. Autoantibodies to double‐stranded DNA (anti‐dsDNA) were present in 49 (92.4%) cases. In patients without nephritis, seven (35%) tested positive for AECA, five for ANCA and all were antinuclear antibody (ANA) positive. Anti‐dsDNA was detected in 16 patients (80%), higher incidence of AECAs was noted in diffuse proliferative glomerulonephritis (41.2%) as compared to focal proliferative glomerulonephritis (37.5%) and membranoproliferative glomerulonephritis (33.3%). IgG‐AECA subclass was noted in 85.7% patients, IgM‐AECA and IgG + M AECA subclasses of AECA were detected in 7.1% cases. AECAs were also found to be associated with other autoantibodies such as ANA, anti‐dsDNA and ANCA. Conclusion: No significant differences in AECA positivity was found between SLE with and without nephritis.     

Drug-induced mononucleosis-like hepatic injury in a patient with systemic lupus erythematosus

A case of systemic lupus erythematosus (SLE) with mononucleosis-like hepatic injury was described. An emergent cesarean section was performed in a 25 yr-old house wife at 34 weeks gestation, followed by administration of several antibiotics. After the surgery she complained of high fever, hepatomegaly and dull right hypochondralgia, and mild liver dysfunction was also found. The liver biopsy showed prominent mononuclear cell infiltration in the sinusoids with minimum hepatocellular necrosis and mild triaditis, resembling hepatic lesion in infectious mononucleosis (mononucleosis-like injury). There were no clinical and serological features suggestive of infectious mononucleosis. This hepatic lesion was thought to be a manifestation of allergic reaction to drugs to which the lymphocyte stimulation test was found to be positive. Immunological abnormalities inherent in SLE might be related to occurrence of such allergic drug reaction.     

Increased platelet activation and abnormal membrane glycoprotein content and redistribution in myeloproliferative disorders

Chronic myeloproliferative disorders (MPDs) are characterized by a high incidence of thrombohaemorrhagic complications, possibly caused by platelet dysfunction. In an attempt to define platelet functional abnormalities, we assessed the expression of activation-dependent membrane proteins in unstimulated and agonist [ADP and thrombin receptor-activating peptide (TRAP)]-stimulated platelets using quantitative whole blood flow cytometry in samples from 50 MPD patients and 30 controls. The receptor densities of activation markers and glycoproteins (GPs) were quantified using standardized fluorescent beads. Compared with controls, the mean percentage of P-selectin-positive (15.3% vs. 7.2%; P < 0.001) and thrombospondin (TSP)-positive (6.6% vs. 3.7%; P = 0.003) platelets was increased in unstimulated platelets from patients. Patients having experienced a thrombotic event had a higher mean percentage of TSP-positive non-stimulated platelets than patients without a history of thrombosis (9.0% vs. 4.6%; P = 0.02) and a higher GPIV molecules of equivalent fluorochrome (MEF) value (33113 vs. 24471 MEF; P = 0.02). Mean MEF values of monoclonal antibodies (mAbs) against GPIb (34055 vs. 38945 MEF; P < 0.001) and GPIIb/IIIa (1416 vs. 1648 MEF; P < 0. 001) were significantly reduced among patients, whereas surface expression of GPIV was increased in patients (28273 vs. 16258 MEF; P < 0.001). In TRAP (10 micromol/l) stimulated whole blood, the MEF of P-selectin (9611 vs. 13293 MEF; P = 0.004) and CD63 (2385 vs. 5177 MEF; P < 0.001) and the ratio of PAC-1/GPIIb/IIIa MEF (0.98 vs. 2. 00; P < 0.001) was reduced in patients, indicating either a reduced granule GP content or an intrinsic cellular defect in receptor-mediated granule secretion and activation of the GPIIb/IIIa complex. Expressed as the relative change of MEF compared with unstimulated platelets, TRAP induced decrease of GPIb (7.8% vs. 45%; P < 0.001) and increase of GPIIb/IIIa (49.1% vs. 95.7%; P < 0.001) and GPIV expression (17.8% vs. 55.2%; P < 0.001) was attenuated in patients.     

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis , which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.     

A case of aplastic anemia in a patient with systemic lupus erythematosus

Abstract

A case of aplastic anemia with a 16-year history of systemic lupus erythematosus (SLE) is described. The diagnosis of aplastic anemia was established by bone marrow biopsy. Aplastic anemia is an extremely rare complication of SLE. The pathogenesis of aplastic anemia associated with SLE remains to be clarified.     

Platelets as target cells in rheumatoid arthritis and systemic lupus erythematosus: A platelet specific immunoglobulin inducing the release reaction

Summary Sera from patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) were assessed for in vitro platelet activation as measured by serotonin release; 24% (30) of 124 tested RA sera and 51% (35) of 69 SLE sera induced a significant 3H serotonin release. Investigation of 17 synovial fluid samples from RA patients revealed significant release in 82%. Concomitant testing for lymphocytotoxic antibodies and immune complexes did not show any correlation to platelet activation. Upon gel filtration the release-inducing activity of positive sera was localized in the region of 160 000 Daltons. Further characterization by ion exchange chromatography, immune electrophoresis, chromatographic and SDS PAGE molecular weight determinations, as well as analytical ultracentrifugation all confirmed the IgG nature of the release-inducing protein. Negative blocking experiments performed by preincubation of platelets with Fc-IgG fragments prior to challenge with a release-inducing serum excluded the participation of Fc receptors in the reaction. It was concluded that the release was caused by a platelet reactive IgG antibody. This antibody may also cause release of platelet mediators in vivo and may thus contribute to the pathogenesis of the generalized vasculopathy in both diseases.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

Abstract

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.     

Possible induction of systemic lupus erythematosus by interferon-alpha treatment in a patient with a malignant carcinoid tumour.

Interferon-alpha (IFN-alpha) is currently used in the treatment of various malignant tumours. Development of different autoimmune disorders has been reported in some patients during IFN-alpha therapy. Systemic lupus erythematosus (SLE) after treatment with IFN-alpha has not been described, although a majority of SLE patients have demonstrable serum levels of IFN-alpha, which correlate with disease activity and have been suggested to be of pathogenetic significance. In this paper we describe a patient with a malignant carcinoid tumour who developed a SLE-like syndrome during treatment with leucocyte IFN-alpha. The patient developed myalgia and low grade arthritis in multiple joints together with a high titre of antinuclear antibodies (ANA) and anti-dsDNA antibodies. After the treatment was stopped, the symptoms subsided although a moderate ANA titre persisted. However, the tumour continued to regress despite cessation of IFN-alpha therapy. During a short course with recombinant IFN-alpha the syndrome relapsed, supporting the concept that the SLE syndrome was precipitated by IFN-alpha. A connection between IFN-alpha treatment, the induced autoimmune disorder and regression of the carcinoid tumour is suggested.     

A case of aplastic anemia in a patient with systemic lupus erythematosus

 A case of aplastic anemia with a 16-year history of systemic lupus erythematosus (SLE) is described. The diagnosis of aplastic anemia was established by bone marrow biopsy. Aplastic anemia is an extremely rare complication of SLE. The pathogenesis of aplastic anemia associated with SLE remains to be clarified. Received: April 11, 2002 / Accepted: August 28, 2002 Acknowledgments The authors would like to thank Prof. Oshimi and Dr. Isobe of the Department of Hematology their helpful comments and discussions.     

Autoantibodies against platelet-derived growth factor receptor alpha in patients with systemic lupus erythematosus

Abstract

Autoantibody against platelet-derived growth factor receptors (PDGFRs) has been reported in scleroderma (SSc). However, it remains unknown whether anti-PDGFRs antibody exists in collagen vascular diseases other than SSc. To answer the question, we developed an ELISA system and examined sera from patients with SLE (n = 75), SSc (n = 31), RA (n = 25) and control individuals. We also reviewed medical records to clarify clinical features of patients with anti-PDGFRα antibody. To examine the functions of anti-PDGFRα antibody in patients, fibroblasts were cultured and stimulated in the presence of purified IgG from patients, and their cell numbers were counted. Anti-PDGFRα antibody was detected in 29% of patients with SLE and in 21% of patients with SSc. Anti-PDGFRα antibody was found in 36% with active SLE, but in 10% in an inactive phase. Immunosuppressive therapy decreased the titer of the antibody. Patients with anti-PDGFRα antibody frequently developed a rash and hematological abnormalities, particular hemolytic anemia. Moreover, anti-PDGFRα antibody in SLE failed to demonstrate agonistic or antagonistic activities on PDGFR signaling. These findings indicate that nonfunctional anti-PDGFRα autoantibody exists in patients with SLE as well as those with scleroderma, and that the antibody could be a marker of disease activity and may be a marker of a subgroup of SLE.     

Thyroid dysfunction and anti-thyroid antibodies in Egyptian patients with systemic lupus erythematosus: Correlation with clinical musculoskeletal manifestations

Aim of the workTo study the prevalence of thyroid dysfunction and anti-thyroid antibodies (ATA) in Egyptian patients with systemic lupus erythematosus (SLE), and their association with musculoskeletal manifestations of the disease.Patients and methodsCross sectional study included 100 SLE patients and 100 matched controls. Clinical manifestations at any time during disease course were reported. Detailed musculoskeletal examination was done using Ritchie articular index (RAI), 44-Swollen joint count and fibromyalgic tender points. Phalen’s test was used to diagnose carpal tunnel syndrome. Free-thyroid hormones (FT3 and FT4), thyroid stimulating hormone (TSH), anti-thyroglobulin (anti-TG) and anti-thyroid peroxidase (anti-TPO) antibodies were measured.ResultsThe prevalence of thyroid dysfunction was significantly higher in patients than controls (18% vs. 4%, p = 0.003) and all were females. Prevalence of subclinical hypothyroidism (SCHT) and clinical hypothyroidism (CHT) is 10% (p = 0.002) and 4% (p = 0.121) versus non among controls while, that of subclinical hyperthyroidism (4%) was not significantly different. Prevalence of anti-TPO and anti-TG is higher in patients than controls (35% vs 11%, p < 0.001 and 22% vs. 6%, p = 0.001). All patients with SCHT had anti-TPO and half of them had anti-TG while all patients with CHT had both antibodies. Hypothyroidism was associated significantly with aging (p = 0.01), longer disease duration (p < 0.001), high BMI, high RAI scores, arthritis, positive Phalen’s test and fibromyalgia (p < 0.001 for all) in comparison to euthyroid patients.ConclusionHypothyroidism was more prevalent in SLE patients and its detection is recommended to reduce the risk of musculoskeletal related morbidity.     

Immunoadsorption in systemic lupus erythematosus

Plasmapheresis and immunoadsorption (IA) have been introduced into the therapeutic strategy of patients with systemic lupus erythematosus (SLE) and severe organ involvement. Despite numerous reports about the clinical efficacy of extracorporeal treatments controlled studies have failed to show significant advantages compared with standard immunosuppressive treatment. We describe the historical development of plasmapheresis‐based IA‐techniques with special comments on substitution fluids and especially immunoglobuline (Ig) specific adsorption columns. In addition, an overview is given of our clinical experience with different columns and a new strategy of aggressive Ig lowering combined with standard cyclophosphamide pulse therapy in SLE patients with severe organ involvement.