69 patients with systemic lupus erythema (SLE) at the Central Institute of Dermatology and Venerology were studied on. The control group included 57 healthy blood donors. The investigation on the existance of 2bp compression phenomenon on exon 29 using PCR technique showed that: C4A deficiency caused by 2bp compression on exon 29 was not detected on these patients of SLE and in control subjects also. There was no relation between the deficiency of the gene encoding C4A caused by 2bp compression on exon 29 and the development of SLE.
Lupus Erythematosus, Systemic/deficiency ; Control Groups ; Tissue Donors

PURPOSE: Antiphospholipid syndrome is a disorder of recurrent vascular thrombosis, recurrent abortion, thrombocytopenia, neurologic disorders associated with the elevation of antiphospholipid antibodies. The aim of our study was to characterize the patient profile and frequency of antiphospholipid syndrome in patients with deep vein thrombosis of the lower legs. METHOD: From January 1998 to December 1999, 25 patients with the lower leg swelling were classified according to their risk factors. Deep vein thrombosis was confirmed by radiologic diagnosis such as duplex ultrasonography or venography. The items for the identification of hypercoagulability were antithrombin III, protein-C, protein-S, lupus anticoagulant, anticardiolipin antibody (IgG). For the differential diagnosis of systemic lupus erythematosus, we tested antinuclear antibody and anti-dsDNA for the patients with positive results of antiphospholipid antibodies. Antiphospholipid syndrome was diagnosed according to its criteria. RESULT: Of the 25 patients with the lower leg swelling, 17 patents (68%) were revealed to have deep vein thrombosis. In that 17 patients, 8 patients showed hypercoagulabilities including 4 patients (24%) with positive test for lupus anticoagulant, 1 patient (6%) with combined multiple abnormalities of protein C and protein S deficiencies and lupus anticoagulant positivity, 2 patients (12%) with antithrombin III deficiencies, 1 patient (6%) with protein C deficiency, and there was no patient with IgG type anticardiolipin antibody positivity. According to the American Rheumatism Association criteria (ARA), there was no patient with systemic lupus erythematosus, but we could find out 1 patient (6%) who met the dagnostic criteria of antiphospholipid syndrome. CONCLLUSION: In our study, 6% (1of 17) of patient with the lower leg deep vein thrombosis revealed antiphospholipid syndrome. We described the clinical profile and diagnostic process of antiphospholipid syndrome in this study.
Abnormalities, Multiple ; Abortion, Habitual ; Antibodies, Anticardiolipin ; Antibodies, Antinuclear ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Antithrombin III ; Antithrombin III Deficiency ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G ; Leg ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Nervous System Diseases ; Phlebography ; Pregnancy ; Protein C ; Protein C Deficiency ; Protein S Deficiency ; Rheumatic Diseases ; Risk Factors ; Thrombocytopenia ; Thrombophilia ; Thrombosis ; Ultrasonography ; Venous Thrombosis*

OBJECTIVETo observe the intervention of Maiwei Dihuang Oral Liquid (MDOL) on hormonotherapy in treating active systemic lupus erythematosus (SLE).

METHODSSixty SLE patients in active stage were randomly and equally allocated into two groups, and administered with prednisone, which was medicated in initial dose of 0.5-1 mg/kg, and with the dose being reduced conditionally 6-8 weeks. To the 30 patients in the treated group 10 ml MDOL twice daily was given additionally. The therapeutic course was 3 months.

RESULTSThe therapeutic effect in the treated group was better than that in the control group (P < 0.05). Systemic lupus erythematosus disease activity index (SLEDAI) was significantly improved in both groups (P < 0.01), but was superior in the treated group (P < 0.05). The dose of prednisone used was significantly reduced (P < 0.01), and the scores of Yin-deficiency fire-flourishing syndrome were obviously decreased (P < 0.01) in the treated group while in the control group, these indexes were unchanged (P > 0.05), the difference between the two groups was significant (P < 0.01). The occurrence of adverse reaction was significantly lower in the treated group than that in the control group (P < 0.05).

CONCLUSIONMDOL can obviously improve the effect of hormonotherapy in SLE, it has advantages in reducing the dosage used and antagonizing the adverse reactions of glucocorticoid.

Adolescent ; Adult ; Drug Administration Schedule ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Lupus Erythematosus, Systemic ; drug therapy ; Male ; Middle Aged ; Phytotherapy ; Prednisone ; administration & dosage ; adverse effects ; Yin Deficiency ; chemically induced ; drug therapy

INTRODUCTIONWe report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE).

CLINICAL PICTUREA diagnosis of angioedema was delayed in a patient presenting with limb and facial swelling until she developed acute upper airway compromise. After excluding allergic and hereditary angioedema, acquired angioedema (AAE) was suspected, possibly precipitated by respiratory tract infection. Associated clinical and laboratory features led to a diagnosis of SLE.

TREATMENTManagement proved challenging and included high dose steroids and immunosuppressants.

OUTCOMEThe patient responded to treatment and remains in remission without recurrence of the angioedema.

CONCLUSIONAAE occurs due to the acquired deficiency of inhibitor of C1 component of complement (C1 INH). Lymphoproliferative disorders and anti-C1 INH antibodies are well-described associations. However, one should also consider the possibility of SLE.

Angioedema ; blood ; etiology ; physiopathology ; therapy ; Antiphospholipid Syndrome ; diagnosis ; etiology ; Brain ; pathology ; Complement C1 Inactivator Proteins ; analysis ; deficiency ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; etiology ; Magnetic Resonance Imaging ; Middle Aged ; Respiration, Artificial ; Respiratory Insufficiency ; etiology ; therapy

OBJECTIVETo investigate the application of surface-enhanced laser desorption ionization time-of-flight mass spectrometry (SELDI) protein chip in diagnosis and Chinese medicie syndrome type researching of systemic lupus erythematosus (SLE).

METHODSEighteen female SLE patients of mild/moderate degree with yin-deficiency caused internal heat syndrome (YDHS) were enrolled in the treatment group, and 15 women healthy volunteers was set up as the control group. Using SELDI method, the pre-, mid- and post-treatment peripheral blood mononuclear cell (PBMC) protein fingerprint of them was created respectively, which was then managed to screen out the markers by using ZUCI-PDAS package for establishing a diagnosis model.

RESULTSStudy was completed in 15 cases of the treatment group with 2 cases dropped out and 1 case lost. Before treatment, 44 protein peaks in the treatment group were found significantly different to those in the control group (P<0.01), and the sensitivity and specificity of the created models, 10542 Da m/z and 2554 Da m/z, reached 100%. After a 12-week treatment, 30 peaks were found significantly different between the two groups (P<0.01), and the sensitivity of 3365, 7104, 3882 and 6796 Da m/z created peak models was 100%, its specificity being 93.33%. Comparing the 35 samples (pre-, mid- and post-treatment) got from the treatment group with the 15 samples from the control group, significant difference was found in 55 peaks (P<0.01), the sensitivity and specificity of the 7103, 3882, 7143 Da m/z created peak models was 100% and 91.43% respectively.

CONCLUSIONSignificant differences of PBMC protein expression patterns were found between SLE patients of YDHS and healthy persons at times of before, during and after treatment, suggesting that SELDI may be used as a new method to create the diagnosis model, and its application in effecter protein screening, activity scoring and Chinese medicine syndrome type researching are expectable and waiting for further study in depth.

Adolescent ; Adult ; Case-Control Studies ; Female ; Humans ; Leukocytes, Mononuclear ; metabolism ; Lupus Erythematosus, Systemic ; diagnosis ; Middle Aged ; Protein Array Analysis ; Sensitivity and Specificity ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; methods ; Yin Deficiency ; Young Adult

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of unknown cause. SLE can involve various major organs including heart, lung, kidney, nervous system and bone marrow. Abnormality in immune system producing various autoantibodies is one of the marked features of this disease. Although most diagnostic items primarily depends on clinical symptoms, four of them are the results of laboratory tests; hematologic abnormalities, antinuclear antibody (ANA), immunologic abnormalities and urinalysis. Most patients with SLE have anemia during the disease course. Anemia with chronic disease is the most common in SLE, followed by immune mediated hemolytic anemia and iron deficiency anemia. Fifteen percent of SLE patients have leukopenia and 20% of them have lymphopenia. Leukopenia in SLE is mostly associated with immune mechanism, drugs, bone marrow dysfunction and hyperspenism. Thrombocytopenia in SLE is induced by immune-mediated destruction of platelet, aggregation of platelet in hemolytic anemia, decreased production of platelet by immunosuppressant and concurrent antiphospholipid syndrome. ANA is the most typical blood test in SLE and can be useful screening test. ANA can be also detected in healthy people and patients with other rheumatic and non-rheumatic diseases. Anti-ds DNA Ab and anti-Sm Ab are specific autoantibodies for SLE and are associated clinical manifestations. Anti-ds DNA Ab is well correlated with disease activity of SLE. Lupus nephritis can be classified into six patterns by light microscopy, immunofluorescence and electron microscopy. Class III and IV represent focal and diffuse glomerulonephritis and relatively poor prognosis. Thus patients having these classes of glomerulonephritis need intensive immunosuppressive treatment. The risk of development of lupus nephritis increases in male and younger patients. In SLE patients with end stage renal disease, SLE activity is usually low.
Anemia ; Anemia, Hemolytic ; Anemia, Iron-Deficiency ; Antibodies, Antinuclear ; Antiphospholipid Syndrome ; Autoantibodies ; Autoimmune Diseases ; Blood Platelets ; Bone Marrow ; Chronic Disease ; DNA ; Glomerulonephritis ; Heart ; Hematologic Tests ; Humans ; Immune System ; Kidney ; Kidney Failure, Chronic ; Leukopenia ; Light ; Lung ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Lymphopenia ; Male ; Mass Screening ; Microscopy, Electron ; Microscopy, Fluorescence ; Nervous System ; Prognosis ; Thrombocytopenia ; Urinalysis

OBJECTIVETo explore the mRNA expressions of IL-10, IL-18 and Fas in peripheral blood mononuclear cells (PBMCs) in systemic lupus erythematosus (SLE) patients of various Chinese medicine syndrome types in order to seek the objective indices for syndrome differentiation of SLE.

METHODSSLE patients were sorted into various syndrome types by Chinese medicine syndrome differentiation. In them, patients with the 4 often encountered types were selected, i.e. the qi-xue excessive heat type (Group A, 12 patients), the Pi-Shen yang-deficiency type (Group B, 19 patients), the qi-yin deficiency with blood stasis type (Group C, 23 patients), and the qi stagnation and blood stasis with gan stagnation type (Group D, 28 patients). The mRNA expressions of IL-10, IL-18 and Fas in PBMCs were detected by SYBR Green I real time PCR.

RESULTSThe mRNA expressions of IL-10, IL-18 and Fas in PBMC of all SLE patients (0.40 +/- 0.53, 1.41 +/- 1.93 and 0.33 +/- 0.70) were significantly higher than those in the normal control group (0.07 +/- 0.11, 0.24 +/- 0.25, 0.08 +/- 0.21, P<0.01, P<0.05), their levels in different syndrome types decreased in order of type A-->B-->C-->D. The levels of the three indices in patients of type A and B were obviously higher than those in patients of type C and D (P<0.01), while no significant difference was found between type A and B (P>0.05), and between type C and D (P>0.05).

CONCLUSIONThe mRNA expressions of IL-10, IL-18 and Fas in PBMCs could be taken as the objective indices for reference in syndrome differentiation of SLE.

Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Interleukin-10 ; blood ; Interleukin-18 ; blood ; Leukocytes, Mononuclear ; metabolism ; Lupus Erythematosus, Systemic ; blood ; diagnosis ; therapy ; Male ; Medicine, Chinese Traditional ; methods ; Middle Aged ; RNA, Messenger ; genetics ; Yang Deficiency ; diagnosis ; Yin Deficiency ; diagnosis ; Young Adult ; fas Receptor ; blood