Cardiovascular System ; physiopathology ; Child ; Humans ; Lupus Erythematosus, Systemic ; physiopathology

Interleukin-15 (IL-15) has multiple biological properties, including the induction of other cytokine production and the inhibition of T cell apoptosis. Recently, IL-15 was reported to have a major role in synovial inflammation of rheumatoid arthritis, and that it provokes and amplifies the inflammatory process through the activation of TNF-alpha production. In systemic lupus erythematosus (SLE), the dysregulation of apoptosis and various cytokine production were observed and have been implicated in the pathogenesis of SLE. Thus, we tried to determine serum IL-15 levels in SLE patients and to assess the relationship among IL-15 levels, TNF-alpha levels and disease activity of SLE. Twenty SLE patients and 10 controls were studied. Paired serum samples were collected from all SLE patients at the time of presentation with active disease and at 4 weeks after institution of treatment. IL-15 levels were determined by ELISA and compared with the disease activity indices in SLE. The disease activity of SLE was measured using the SLE Disease Activity Index (SLEDAI) and laboratory parameters such as circulating immune complex (CIC), C3, C4, anti-DNA antibody, IgG, IgM, and IgA. The IL-15 levels in SLE patients were significantly higher than those of controls (5.38 +/- 4.89 vs. 1.04 +/- 1.26 pg/ml). However, elevated IL-15 levels did not correlate with the SLEDAI, nor did they correlate with other laboratory activity indices. The changes in serum IL-15 levels did not correlate with the changes in serum TNF-alpha in the disease course of SLE patients, whereas TNF-alpha reflected the changes in disease activity of SLE. Serum levels of IL-15 are elevated in SLE patients, but IL-15 did not correlate with the disease activity of SLE. TNF-alpha production in SLE patients was unlikely to be related with IL-15.
Adolescence ; Adult ; Female ; Glucocorticoids, Synthetic/therapeutic use ; Human ; Interleukin-15/blood* ; Lupus Erythematosus, Systemic/physiopathology ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/blood* ; Male ; Prednisolone/therapeutic use ; Tumor Necrosis Factor/analysis

OBJECTIVETo investigate the diagnostic value of the tissue strain imaging in myocardial dysfunction in children with systemic lupus erythematosus (SLE).

METHODS24 patients with SLE and 32 healthy controls underwent conventional and tissue Doppler echocardiography. Peak strain and strain rate value during systolic and diastolic phases as well as E/A, LVFS, LVEF were measured in both SLE and the control group subjects.

RESULTSThe E/A, LVFS and LVEF did not significantly differ between SLE children and controls (P > 0.05). The systolic peak strains and strain rates of SLE children were lower than those of the controls but there was no significant difference (P > 0.05). The diastolic peak strains and strain rates of SLE children were significantly lower than those of the controls (P < 0.01). The diastolic peak strains and strain rates of anticardiolipin antibodies (aCL) positive children were significantly lower than the aCL negative ones (P < 0.05).

CONCLUSIONStrain and strain rate combined with aCL could sensitively detect myocardial dysfunction of children with SLE.

Adolescent ; Case-Control Studies ; Child ; Echocardiography, Doppler ; methods ; Female ; Humans ; Lupus Erythematosus, Systemic ; diagnostic imaging ; physiopathology ; Ventricular Function, Left

Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; pathology ; physiopathology ; Pelvic Organ Prolapse ; pathology ; physiopathology ; Uterine Diseases ; pathology ; physiopathology ; Uterus ; pathology ; physiopathology ; Young Adult

OBJECTIVE: To analyze the echocardiographic abnormalities and the prevalence of anticardiolipin antibodies (aCL) in systemic lupus erythematosus (SLE) patients and to evaluate the relationship between aCL and cardiac valvular abnormalities in SLE patients. METHODS: Ninety SLE patients were performed M-mode, 2-dimensional and Doppler echocardiography and aCL IgG and IgM were measured by an enzyme-linked immunosorbent assay (ELISA). According to the abnormalities in the echocardiography, the patients were assigned into valvular abnormality group and non-valvular abnormality group. Chi-square method was used to compare the difference of aCL prevalence between the two groups. RESULTS: The prevalence of echocardiographic abnormalities was 53.33%, and valvular abnormality (38.89%) and pericardial effusion (34.44%) presented most frequently. The aCL prevalence was 32.56% in the 43 SLE patients. The prevalence of aCL in the valvular abnormality group was significantly higher than that in non-valvular abnormality group (52.94% vs 19.23%, P<0.05). CONCLUSION The incidence of echocardiographic abnormalities is high in SLE patients, most often in valves and pericardium. The aCL is probably related to valvular damage in SLE patients.
Adolescent ; Adult ; Antibodies, Anticardiolipin ; blood ; Echocardiography, Doppler ; Female ; Heart Valve Diseases ; etiology ; immunology ; physiopathology ; Humans ; Lupus Erythematosus, Systemic ; complications ; immunology ; physiopathology ; Male

Pure red cell aplasia (PRCA) is a rare cause of anemia associated with systemic lupus erythematosus (SLE), and fewer than 20 cases have been reported. The development of PRCA may be mediated by an autoimmune mechanism which is supported by the presence of antibodies that impair various stages and mechanisms of erythropoiesis, by the association with immunological disorders or lymphoma, and by a favorable response to immunosuppressive drugs, antilymphocyte globulin, thymectomy, and splenectomy. However, these therapies have not been successful in all patients with PRCA. We report our experience with a 31-year-old female patient with SLE who developed PRCA that did not respond to immunosuppressive therapies. However, complete normalization of erythropoiesis was achieved after the removal of the autoantibodies by plasmapheresis, and the patient has now maintained a normal hemoglobin level for more than eight months. We suggest that plasmapheresis might be tried in the treatment of PRCA cases before other more aggressive therapies are commenced.
Adult ; Bone Marrow/pathology ; Case Report ; Erythropoiesis ; Female ; Human ; Lupus Erythematosus, Systemic/*complications ; *Plasmapheresis ; Red-Cell Aplasia, Pure/*complications/pathology/physiopathology/*therapy

Objective To investigate the clinical features of patients with Castleman's disease (CD) and systemic lupus erythematosus (SLE). Methods According to the diagnostic information between 1994 to 2014 extracted from the database of the Medical Record Department of Peking Union Medical College Hospital (PUMCH),patients with CD and SLE were included. A thorough literature review utilizing the key words of "Castleman's disease","systemic lupus erythematosus","SLE",and "lupus" was performed in PubMed during the same period. Cases with detailed clinical information were included while cases without detailed information were excluded from the analysis of this study. Results Nine patients worldwide were available for analysis [2 cases from PUMCH,accounted for 0.03%(2/6502) of all patients diagnosed as SLE and 1.0% (2/100) of patients diagnosed as CD during the same period] with a male-to-female ratio of2:7. The median age at diagnosis of CD was 39.0 years (range:21- 60 years). All patients were diagnosed as multicentric CD with generalized peripheral lymphadenopathy. Pathologic examination showed a balanced distribution:plasma cell variant:hyaline-vascular variant:mixed variant=3:3:3. Fever was the most common symptom (88.9%,8/9). Blood system was the most commonly involved system (88.9%,8/9) and kidneys were the most commonly involved organ (88.9%,8/9). Autoimmune thrombocytopenia (AITP) was observed in 55.6% (5/9) of patients,which was significantly higher than the general SLE patients (15.0%) (P<0.01). None of the 9 patients had evidence of central nervous system involvement. Conclusions CD complicated by SLE is a rare clinical condition. Compared to the general SLE population,this subgroup of patients may have higher rate of AITP and lower rate of central nervous system involvement.
Adult ; Castleman Disease ; complications ; physiopathology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Lymphatic Diseases ; complications ; Male ; Middle Aged ; Purpura, Thrombocytopenic, Idiopathic ; complications ; Young Adult

OBJECTIVE: To investigate the changes of bone mineral density (BMD) and serum bone turnover factor in newly diagnosed systemic lupus erythematous (SLE) patients. METHODS: Eighty newly diagnosed SLE patients and 80 age and gender matched healthy controls were enrolled. None of the SLE patients had ever received glucocorticoid, immunosuppressive agents or vitamin D. BMD was measured at radius,lumbar spine and hip by dual X ray absorptiometry (DXA). Bone turnover markers including serum levels of tartrate-resistant acid phosphatase 5b (TRAP5b),bone alkaline phosphatase (BAP) and 25-hydroxy vitamin D3 (25-OH-VD3) were measured by enzyme-linked immunosorbent assay (ELISA). Logistic regression was employed to analyze the risk factors associated with decreased BMD. RESULTS: Mean age of the SLE patients was (32.8±12.4) years, and 85% were female, none of whom were post-menopausal. BMD was significantly reduced in all the measured sites, compared with the healthy controls. Sixteen (20%) of the patients were osteopenic in at least one site measured locations. The serum levels of 25-OH-VD3 were markedly reduced in the newly diagnosed SLE patients than those of the normal controls [(46.1+12.3) nmol/L vs. (25.4+11.2) nmol/L, P<0.001)]. The serum levels of 25-OH-VD3 in the SLE patients with nephritis were much lower than those without nephritis (P=0.04). A significant negative correlation was demonstrated between the serum concentration of 25-OH-VD3 and the disease activity scores as measured by SLE disease activity index (SLEDAI) (r=-0.3,P=0.001). The serum TRAP5b concentration was positively correlated with SLEDAI (r=0.435,P=0.003). Age (P=0.058) and SLEDAI (P=0.085) were probably associated with decreased BMD in Logistic regression analysis. CONCLUSION The study showed reduced BMD in untreated SLE patients. The role of chronic inflammation was of probable importance in bone metabolism.
Absorptiometry, Photon ; Adult ; Bone Density ; Bone Diseases, Metabolic ; Bone Remodeling ; Female ; Humans ; Lupus Erythematosus, Systemic/physiopathology* ; Male ; Middle Aged ; Young Adult

The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.
Adrenal Cortex Hormones/therapeutic use ; Adult ; Humans ; Hypoprothrombinemias/*diagnosis/drug therapy/immunology/physiopathology ; Lupus Coagulation Inhibitor/*immunology ; Lupus Erythematosus, Systemic/*diagnosis/immunology/physiopathology ; Male ; Partial Thromboplastin Time ; Preoperative Care ; Prothrombin Time

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