Atherosclerosis ; etiology ; therapy ; Humans ; Lupus Erythematosus, Systemic ; pathology ; therapy

Adolescent ; Heart Valve Diseases ; etiology ; Heart Valves ; pathology ; Humans ; Lupus Erythematosus, Systemic ; complications ; pathology ; Male

Lupus is a systemic autoimmune disease of an unknown origin, and systemic lupus erythematosus (SLE) can be triggered by numerous stimuli. Bee venom therapy is an alternative therapy that is believed to be effective for various kinds of arthritis. We present here a case of a 49-year-old female who experienced a new onset lupus after undergoing bee venom therapy, and this looked like a case of angioedema. The patient was successfully treated with high dose steroids and antimalarial drugs. We discuss the possibility of bee venom contributing to the development of SLE, and we suggest that such treatment should be avoided in patients with lupus.
Bee Venoms/*adverse effects ; Female ; Humans ; Lupus Erythematosus, Systemic/*etiology/pathology ; Middle Aged

Adult ; Biopsy ; Fatty Liver ; etiology ; pathology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications

A 16-year-old female patient with systemic lupus erythematosus (SLE) presented with abdominal pain and oliguria, after one month of hospital treatment with methylprednisolone and cyclophosphamide. Computed tomography of the abdomen revealed the calcification of bilateral superior segmental ureteral wall. Computed tomography urography revealed stenosis of bilateral ureters. This calcification may be associated with lupus-induced inflammatory reaction of ureteral endomembrane. By receiving the ureteral catheters, her abdominal pain and oliguria had been completely resolved. After discharge, she underwent the surgery of cutaneous ureterostomy in the local hospital. During two years follow-up, her condition is stable without any symptom of the urinary system.
Adolescent ; Calcinosis ; diagnosis ; etiology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Ureter ; pathology

OBJECTIVE: To improve the understanding of lupus cystitis. METHODS: Clinical manifestations, laboratory Results , and image information of 2 cases of lupus cystitis were analysed retrospectively, and another 6 cases in the literature were reviewed. RESULTS: Two patients were female. The urinary symptoms followed the gastrointestinal symptoms. Ureterectasia and hydronephrosis were detected in both patients, and intestinal pseudo-obstruction was detected in one patient. In the 6 cases from the literature, ureterectasia and hydronephrosis were detected in all patients, and intestinal pseudo-obstruction was detected in 4. CONCLUSION The possibility of lupus cystitis should be considered when lupus patients complain of urinary or bowel symptoms. Glucocorticoid and immunodepressant are effective for lupus cystitis.
Adolescent ; Adult ; Cystitis ; complications ; diagnosis ; Female ; Humans ; Hydronephrosis ; etiology ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Ureter ; pathology

We report on a 13-year-old female with systemic lupus erythematosus (SLE) who exhibited symptoms of severe migraine and familial moyamoya disease. Cerebral magnetic resonance angiography (MRA) showed stenosis and occlusion of the bilateral internal carotid arteries associated with the development of collateral circulation (moyamoya vessels). In a child, as in this case, headaches with cerebral infarction associated with moyamoya disease are unusual. Few cases of SLE associated with familial moyamoya disease have been reported, with no previous reports of such cases from Korea. There were no evidences of antiphospholipid syndrome, and activity of SLE or other risk factors for cerebral occlusion were also absent.
Adolescent ; Carotid Artery, Internal/pathology ; Female ; Human ; Lupus Erythematosus, Systemic/*complications ; Magnetic Resonance Angiography ; Migraine/etiology ; Moyamoya Disease/*diagnosis/*genetics

OBJECTIVE: To describe the clinical, immunological characteristics and organ involvement of patients with systemic lupus erythematosus (SLE) in Tibet plateau, China. METHODS: We retrospectively investigated 70 patients admitted in the Tibet Autonomous Region People's Hospital between May 2014 and April 2016. In the study, 120 hospitalized patients with SLE from the Department of Rheumatology and Immunology of the Peking University People's Hospital were randomly selected as the control (plain) group. The major organ involvement, clinical and immunological characteristics were compared between the two groups. RESULTS: The female to male ratio of Tibet plateau group was 10.7, while the corresponding ratio of plain group was 11.0. The mean age at disease diagnosis was (32.21±11.40) and (35.38±13.25) years, respectively. the most common initial manifestations of SLE were arthritis (78.6%), alopecia (55.7%) and malar rash (48.6%) in Tibet plateau group, the prevalence of arthritis and alopecia was significantly higher than in plain group (P<0.05). The incidence of neuropsychiatric and kidney involvement was significantly lower in Tibet plateau group compared with plain group (P<0.05). As for the serological manifestations, the positivity of anti-double-stranded DNA (dsDNA) (57.1%), anti-Smith (Sm) antibody (55.7%), anti-Sjögren syndrome A (SSA) antibody (72.3%), anti-Sjögren syndrome B (SSB) antibody (41.4%) and anti-u1-ribosenuclear protein (u1RNP) antibody (45.7%) was significantly higher in Tibet plateau group (P<0.05). While the incidence of low serum complement C3 (61.4%), C4 (38.6%) less frequent in Tibet plateau group. Mean SLE disease activity index (SLEDAI) score was similar in the Tibet plateau group (12.18±5.58) and plain group (12.69±7.28). Moreover, there were 13 (18.6%) SLE patients suffering from tuberculosis and 7 (10%) SLE patients infected with hepatitis B virus in Tibet plateau group. The number of recent-onset SLE patients with lower 25-dihydroxy-vitamin D3 (25-OH-VD3) in Tibet plateau group was fewer than that in the plain group (76.7% vs. 90.0%, P=0.046). Serum 25-OH-VD3 levels in Tibet plateau plateau group were (31.14±18.74) nmol/L, those in plain group were (26.91±14.27) nmol/L, and the difference was not significant. CONCLUSION The age, gender and SLEDAI scores in Tibet plateau group was similar to those in plain group. But there are significant differences in clinical manifestations, distributions of antibodies and immunological changes between Tibet plateau group and plain group. The patients with lower serum 25-OH-VD3 levels were more in plain group than in Tibet plateau group, while there was no significant difference in the 25-OH-VD3 level between the two groups.
Adult ; Antibodies/analysis* ; Arthritis/etiology* ; China ; Female ; Humans ; Lupus Erythematosus, Systemic/pathology* ; Male ; Middle Aged ; Retrospective Studies ; Tibet ; Young Adult

Antibodies, Antineutrophil Cytoplasmic ; blood ; Biopsy ; Child ; Diagnosis, Differential ; Female ; Humans ; Kidney Glomerulus ; pathology ; Lupus Erythematosus, Systemic ; complications ; immunology ; Lupus Nephritis ; diagnosis ; etiology ; immunology ; pathology ; Necrosis

OBJECTIVETo study the clinical manifestations of rheumatic disorders with macrophage activation syndrome (MAS) in children.

METHODSThe authors characterized MAS by carrying out a retrospective study on patients who were identified during the past 12 years in Tianjin Children's Hospital.

RESULTSSix cases (4 females, 2 males) were studied. Four had typical systemic onset juvenile idiopathic arthritis (SOJIA), two had systemic lupus erythematosus (SLE) with lupus nephritis. Clinical manifestations at diagnosis, which occurred in the lower activity state of these primary diseases, included high spiking fever (in 5 cases) or high fever (in 1), hepatosplenomegaly (in 6), lymphadenopathy (in 6), profound decrease of all 3 blood cell lines (in 6), significant injury of liver (in 6), diseminated intravascular coagulation (DIC)-like picture (in 2), and central nervous system dysfunction (in 3). Hypofibrinogenemia, elevated liver enzymes and hypertriglyceridemia were found consistently. The phagocytic histiocytes with plasmacytosis were found in 3 bone marrow smears (not done in others). MAS was presumed to have been precipitated by viral infections in 3 patients, two had evidences for herpes simplex virus infection and one for hepatitis A virus infection. The treatment regimen was tailored to each patient, as the clinical course was variable.

CONCLUSIONSMAS may not only be most frequently seen in children with SOJIA, but also in those with other rheumatic diseases, and may be a syndrome that is more common than previously thought. Infection may be main trigger factor for MAS. The immunoapheresis combined with immunochemotherapy may be optimal for severe injury of the liver in patients with MAS.

Adolescent ; Arthritis, Juvenile ; complications ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; pathology ; Macrophage Activation Syndrome ; etiology ; pathology ; Male ; Retrospective Studies