The pathological changes of systemic lupus erythematosus (SLE) could involve multiple organs and systems, showing a complicated and multivariate clinical features. Since the authors held that the previous clinical disease-syndrome integrative thought can not embody the regularity in the diagnosis and treatment of SLE, in order to establish a novel operable thought of syndrome differentiation which could reflect the clinical practice of SLE, they suggested, in accordance with their clinical experience, that the basis for integration of the disease and its syndromes should not be SLE itself but the clinical manifestations of the organs and systems, such as fever, nervous system injury, lupus kidney, thrombocytopenic purpura of SLE, etc. They claimed that the new clinical thought of diagnosis and treatment will contribute to conduct systematic and standard research on SLE, and the regulation of TCM therapy based on syndrome differentiation would be more closer to the clinical practice of SLE.
Diagnosis, Differential ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Lupus Erythematosus, Systemic ; diagnosis ; drug therapy ; Medicine, Chinese Traditional ; Phytotherapy ; Research Design

Systemic lupus erythematosus(SLE) is a prototypic autoimmune disease affecting various organ systems. Hypothermia is a rare manifestation of SLE. We experienced a case of SLE combined with hypothermia. A 36-year-old woman, who had been diagnosed as SLE 3 days before admission, admitted complaining of mental confusion. After admission, her body temperature, initially 36.1 degree C, became 32.6 degree C. Her core body temperature was less than 35.0 degree C. Despite of warming with heating lamp and blankets, her core temperature did not reach 35.0 degree C during 18 hours. Ten days later, her temperature exceeded 36.0 degree C.
Adrenal Cortex Hormones/administration & dosage ; Adult ; Atrial Fibrillation/diagnosis ; Atrial Fibrillation/complications* ; Body Temperature/physiology ; Case Report ; Cyclophosphamide/administration & dosage ; Drug Therapy, Combination ; Electrocardiography ; Female ; Follow-Up Studies ; Human ; Hypothermia/therapy ; Hypothermia/diagnosis ; Hypothermia/complications* ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/complications*

Antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis, spontaneous fetal losses, thrombocytopenia and persistently elevated levels of antiphospholipid antibodies. We experienced a case of Budd-Chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema, ascites and esophageal varix. The clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus (SLE) and she was found to have anticardiolipin antibody, thrombocytopenia and prolonged partial thromboplastin time. Initially, she was treated with intravenous heparin and uroki nase and she was followed up with warfarin, baby aspirin and steroids.
Adult ; Angiography ; Animal ; Antibodies, Antiphospholipid/blood* ; Case Report ; Drug Therapy, Combination ; Female ; Hepatic Vein Thrombosis/complications ; Hepatic Vein Thrombosis/diagnosis* ; Hepatic Vein Thrombosis/drug therapy ; Human ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis* ; Lupus Erythematosus, Systemic/drug therapy ; Tomography, X-Ray Computed

BACKGROUNDGlaucoma secondary to systemic lupus erythematosus (SLE) is an uncommon but serious complication that threatens vision and therefore cannot be neglected. A few cases of secondary glaucoma resulting from lupus-induced or iatrogenic ocular impairments have been reported in association with SLE. However, a systematic analysis of the diagnosis and treatment of glaucoma secondary to SLE has not been reported in the literature. The aim of this study is to further investigate the relationship between glaucoma and SLE.

METHODSIn this study, we reviewed nine eyes of five patients diagnosed with secondary glaucoma associated with SLE, including one case of neovascular glaucoma and four cases of steroid-induced glaucoma.

RESULTSNeovascular glaucoma was successfully treated by Ahmed glaucoma valve implantation surgery with adjunctive ranibizumab intravitreal injection, followed by panretinal photocoagulation (PRP). The steroid-induced glaucoma in eight eyes of four cases were controlled by trabeculectomy along with antiproliferative agents.

CONCLUSIONRegular follow-up ocular examinations should be conducted to ensure early diagnosis and treatment of secondary glaucoma in SLE patients to improve the prognosis of vision.

Adult ; Antibodies, Monoclonal, Humanized ; therapeutic use ; Female ; Glaucoma ; diagnosis ; drug therapy ; Humans ; Lupus Erythematosus, Systemic ; diagnosis ; drug therapy ; Ranibizumab ; Retrospective Studies ; Young Adult

OBJECTIVETo acquire more knowledge about neonatal lupus erythematosus (NLE).

METHODSeven cases with neonatal lupus erythematosus who were seen in this hospital from 1990 to 2009 are reported in this paper and 87 cases reported previously from 1980 to now in China were reviewed. The clinical manifestations, serum autoantibodies, treatment and results of long-term follow-up are analyzed and summarized.

RESULTTotally 94 cases were summarized. Male/female ratio was 48/46; 73 cases had skin rash; 23 had heart abnormality, among whom 13 had cardiac conductive problems including 8 cases of atrioventricular blockage (AVB) (3 degree I, 3 degree II and 2 degree III) and 5 cases of right bundle branch block cases (RBBB). Nine cases had anatomical abnormality including 5 cases of atrial septal defect (ASD), 2 cases of ventricular septal defect (VSD) and 2 cases of atrial enlargements. Forty-four cases had hematological problems including 28 with thrombocytopenia, 11 with leukocytopenia and 34 with anemia. Thirty cases had hepatic abnormality, including 24 liver dysfunction, 22 hepatomegaly, 6 splenomegaly and 3 cholestasis. Three cases had nephropathy; 3 had elevated creatine kinase; 2 had nervous disorder. Among the 94 cases, 86 (91.5%) were positive for anti-SSA, 51 (54.3%) anti-SSB, 16 anti-ds-DNA, 14 anti-U1-RNP, 13 anti-Sm (+), 6 anti-RNP and 4 anti-rRNP(+). Among the corresponding mothers, 39 cases (44.8%) were asymptomatic before pregnancy, 35 had SLE, 5 had SCLE, 3 had Sjogren syndrome, 2 had chilblain, photosensitivity and arthralgia, respectively, 1 had rheumatoid arthritis and 1 had psoriasis. During pregnancy, 8 mothers developed SLE. Totally 48 mothers (51.1%) suffered from LE. Together with 15 mothers who had transient skin rash during the pregnancy, there were 23 mothers (59%) who had new clinical manifestation among the 39 asymptomatic mothers. Twenty NLE cases accepted glucocorticoid treatment, 4 of them were treated with intravenous immunoglobulin. Sixty-eight cases were followed up for up to 12 years, 58 cases were healthy, 5 cases improved and 3 died. Two cases still had grade III AVB without pacemaker.

CONCLUSIONNLE is a rare acquired autoimmune disease. Although nearly half of the mothers were asymptomatic before pregnancy, more than half of them developed LE or other symptoms. The clinical presentations in Chinese cases include a transient rash, cardiac lesion while grade III AVB was rare, hematological changes and liver impairments which were common but not serious. Anti-SSA and/or anti-SSB were the most related autoantibody. Most patients with NLE have relatively good prognosis.

Adult ; Autoantibodies ; blood ; Female ; Follow-Up Studies ; Humans ; Infant, Newborn ; Lupus Erythematosus, Cutaneous ; drug therapy ; pathology ; Lupus Erythematosus, Systemic ; congenital ; diagnosis ; drug therapy ; pathology ; Male ; Mothers ; Pregnancy ; Prognosis ; Retrospective Studies

Both systemic lupus erythematosus and Sjogren's syndrome are autoimmune diseases. Almost all organs can be involved but the gall bladder is an unusual site. We report a 39-year-old woman with systemic lupus erythematosus and Sjogren's syndrome presenting with acute acalculous cholecystitis. It is a very rare complication and, in the literature review, surgical interventions are mostly applied for treatment. In our case, high dose corticosteroid was tried and clinical manifestations and radiologic findings were improved.
Acute Disease ; Adult ; Case Report ; Cholecystitis/*complications/diagnosis/drug therapy ; Female ; Human ; Lupus Erythematosus, Systemic/*complications/diagnosis ; Methylprednisolone/therapeutic use ; Sjogren's Syndrome/*complications/diagnosis ; Tomography, X-Ray Computed

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

Retinal vascular lesions are the most common ophthalmologic manifestation of systemic lupus erythematosus (SLE), occurring in 3% to 29% of cases, generally late in the disease. More rare is the severe vaso-occlusive disease, often termed "retinal vasculitis", which includes central retinal artery occlusion, multifocal arteriolar occlusions, extensive capillary nonperfusion and central venous occlusion. Patients with SLE and raised serum concentrations of anticardiolipin antibodies (ACA) have a higher risk of developing occlusive ocular vascular disease. We report a case in which retinal involvement was an earlier manifestation of SLE in a patient without ACA.
Adolescent ; Adrenal Cortex Hormones/administration & dosage ; Angiography ; Case Report ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Lupus Erythematosus, Systemic/*diagnosis/drug therapy ; Ophthalmoscopy ; Retinal Vein Occlusion/*diagnosis/drug therapy ; Treatment Outcome ; Visual Acuity

BACKGROUND: The onset and clinical presentation of systemic lupus erythematosus (SLE) are sex-related. Few studies have investigated the distinctions in clinical characteristics and treatment preferences in male and female SLE patients in the initial cohort. This study aimed to improve the understanding of Chinese SLE patients by characterizing the different sexes of SLE patients in the inception cohort. METHODS: Based on the initial patient cohort established by the Chinese SLE Treatment and Research Group, a total of 8713 patients (795 men and 7918 women) with newly diagnosed SLE were enrolled between April 2009 and March 2021. Of these, 2900 patients (347 men and 2553 women) were eligible for lupus nephritis (LN). A cross-sectional analysis of the baseline demographic characteristics, clinical manifestations, laboratory parameters, organ damage, initial treatment regimens, and renal pathology classification was performed according to sex. RESULTS: In the SLE group, as compared to female patients, male patients had a later age of onset (male vs. female: 37.0 ± 15.8 years vs. 35.1 ± 13.7 years, P  = 0.006) and a higher SLE International Collaborative Clinic/American College of Rheumatology damage index score (male vs. female: 0.47 ± 1.13 vs. 0.34 ± 0.81, P  = 0.015), LN (male vs. female: 43.6% vs. 32.2%, P < 0.001), fever (male vs. female: 18.0% vs. 14.6%, P  = 0.010), thrombocytopenia (male vs. female: 21.4% vs. 18.5%, P  = 0.050), serositis (male vs. female: 14.7% vs. 11.7%, P  = 0.013), renal damage (male vs. female: 11.1% vs. 7.4%, P < 0.001), and treatment with cyclophosphamide (CYC) (P < 0.001). The frequency of leukopenia (male vs. female: 20.5% vs. 25.4%, P  = 0.002) and arthritis (male vs. female: 22.0% vs. 29.9%, P < 0.001) was less in male patients with SLE. In LN, no differences were observed in disease duration, SLE Disease Activity Index score, renal biopsy pathological typing, or 24-h urine protein quantification among the sexes. In comparisons with female patients with LN, male patients had later onset ages (P  = 0.026), high serum creatinine (P < 0.001), higher end-stage renal failure rates (P  = 0.002), musculoskeletal damage (P  = 0.023), cardiovascular impairment (P  = 0.009), and CYC use (P  = 0.001); while leukopenia (P  = 0.017), arthritis (P  = 0.014), and mycophenolate usage (P  = 0.013) rates were lower. CONCLUSIONS Male SLE patients had more severe organ damage and a higher LN incidence compared with female SLE patients; therefore, they may require more aggressive initial treatment compared to female patients.
Humans ; Female ; Male ; Cross-Sectional Studies ; Sex Characteristics ; East Asian People ; Severity of Illness Index ; Lupus Erythematosus, Systemic/diagnosis* ; Lupus Nephritis/pathology* ; Registries ; Cyclophosphamide/therapeutic use* ; Thrombocytopenia ; Leukopenia/drug therapy* ; Arthritis

A 21-year-old woman presented with bilateral optic neuritis, combined with central retinal vein occlusion. General physical examination and neurologic consultation revealed no other findings. Laboratory investigation yielded an elevated erythrocyte sedimentation rate, positive LE preparation, elevated ANA titer, and elevated blood urea nitrogen and creatinine levels. Diagnosis of systemic lupus erythematosus (SLE) was made. Renal failure developed quickly and she was treated with hemodialysis, transfusion and subsequently systemic corticosteroid. Anti-phospholipid antibody was positive to lupus anti-coagulant and the titer was normalized after 2-month steroid therapy at which time the visual outcome differed between the eyes. The right eye showed improvement in visual acuity and visual field, but the left eye was not improved and retained a central scotoma. SLE needs to be considered in young women with optic neuritis when other causes of optic neuritis have been excluded, and serologic tests including anti-phospholipid antibody should be conducted.
Adult ; Case Report ; Female ; Fluorescein Angiography ; Human ; Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy ; Optic Neuritis/*diagnosis/drug therapy/etiology ; Perimetry ; Prednisolone/*therapeutic use ; Treatment Outcome ; Visual Acuity ; Visual Fields