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

Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE), needing prompt diagnosis and proper management. However, SLE rarely presents as lupus enteritis at the time of initial diagnosis. Thus, delayed diagnosis and misdiagnosis are common. We report a case of a 25-year-old woman with lupus panenteritis. The patient had multiple hospitalizations for abdominal pain, nausea, and diarrhea, initially without any other symptoms suggestive of SLE, but was later observed to have malar rash and oral ulcers. Laboratory investigations were compatible with SLE, including positive antinuclear antibody (1:320) with speckled pattern. CT revealed diffuse hypodense submucosal thickening of the stomach, the entire small bowel, colon, appendix, and rectum. Treatment with high-dose corticosteroids followed by maintenance therapy with mycophenolate mofetil, hydroxychloroquine, and azathioprine resulted in clinical improvement. Diagnosis of lupus enteritis requires a high index of suspicion given the low incidence and nonspecific clinical findings.
Abdominal Pain/complications ; Adrenal Cortex Hormones/therapeutic use ; Adult ; Brain/diagnostic imaging ; Diagnosis, Differential ; Diarrhea/complications ; Endoscopy, Gastrointestinal ; Enteritis/pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy ; Magnetic Resonance Imaging ; Nausea/complications ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.
Adult ; Aged ; Biomarkers/blood ; Cholesterol/blood ; Drug Therapy, Combination ; Edema/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypoalbuminemia/diagnosis/drug therapy/*etiology ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Protein-Losing Enteropathies/diagnosis/drug therapy/*etiology ; Remission Induction ; Risk Factors ; Serum Albumin/metabolism ; Tertiary Care Centers ; Time Factors ; Treatment Outcome

No abstract available.
Antirheumatic Agents/therapeutic use ; Arthritis, Rheumatoid/blood/*complications/diagnosis/drug therapy/physiopathology ; Biological Markers/blood ; Drug Therapy, Combination ; Facial Dermatoses/complications/diagnosis ; Female ; Hand Joints/physiopathology/radiography ; Humans ; Immunosuppressive Agents/therapeutic use ; Inflammation Mediators/blood ; Knee Joint/physiopathology/radiography ; Lupus Erythematosus, Systemic/blood/*complications/diagnosis/drug therapy ; Middle Aged ; Syndrome ; Treatment Outcome

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

A 53-year-old Asian woman was treated with hydroxychloroquine and chloroquine for lupus erythematosus. Within a few years, she noticed circle-shaped shadows in her central vision. Upon examination, the patient's visual acuity was 20 / 25 in both eyes. Humphrey visual field (HVF) testing revealed a central visual defect, and fundoscopy showed a ring-shaped area of parafoveal retinal pigment epithelium depigmentation. Fundus autofluorescence imaging showed a hypofluorescent lesion consistent with bull's eye retinopathy. Adaptive optics scanning laser ophthalmoscope (AO-SLO) revealed patch cone mosaic lesions, in which cones were missing or lost. In addition, the remaining cones consisted of asymmetrical shapes and sizes that varied in brightness. Unlike previous studies employing deformable mirrors for wavefront aberration correction, our AO-SLO approach utilized dual liquid crystal on silicon spatial light modulators. Thus, by using AO-SLO, we were able to create a photographic montage consisting of high quality images. Disrupted cone AO-SLO images were matched with visual field test results and functional deficits were associated with a precise location on the montage, which allowed correlation of histological findings with functional changes determined by HVF. We also investigated whether adaptive optics imaging was more sensitive to anatomical changes compared with spectral-domain optical coherence tomography.
Chloroquine/*adverse effects/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Image Enhancement/*methods ; Lupus Erythematosus, Systemic/drug therapy ; Macula Lutea/drug effects/*pathology ; Middle Aged ; Ophthalmoscopy/*methods ; Retinal Diseases/chemically induced/*diagnosis

A 53-year-old Asian woman was treated with hydroxychloroquine and chloroquine for lupus erythematosus. Within a few years, she noticed circle-shaped shadows in her central vision. Upon examination, the patient's visual acuity was 20 / 25 in both eyes. Humphrey visual field (HVF) testing revealed a central visual defect, and fundoscopy showed a ring-shaped area of parafoveal retinal pigment epithelium depigmentation. Fundus autofluorescence imaging showed a hypofluorescent lesion consistent with bull's eye retinopathy. Adaptive optics scanning laser ophthalmoscope (AO-SLO) revealed patch cone mosaic lesions, in which cones were missing or lost. In addition, the remaining cones consisted of asymmetrical shapes and sizes that varied in brightness. Unlike previous studies employing deformable mirrors for wavefront aberration correction, our AO-SLO approach utilized dual liquid crystal on silicon spatial light modulators. Thus, by using AO-SLO, we were able to create a photographic montage consisting of high quality images. Disrupted cone AO-SLO images were matched with visual field test results and functional deficits were associated with a precise location on the montage, which allowed correlation of histological findings with functional changes determined by HVF. We also investigated whether adaptive optics imaging was more sensitive to anatomical changes compared with spectral-domain optical coherence tomography.
Chloroquine/*adverse effects/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Image Enhancement/*methods ; Lupus Erythematosus, Systemic/drug therapy ; Macula Lutea/drug effects/*pathology ; Middle Aged ; Ophthalmoscopy/*methods ; Retinal Diseases/chemically induced/*diagnosis

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/microliter. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/microliter after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.
Azathioprine/therapeutic use ; Bone Marrow/pathology ; Cyclophosphamide/*therapeutic use ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/*therapeutic use ; Infusions, Intravenous ; Lupus Erythematosus, Systemic/complications/*diagnosis ; Middle Aged ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Platelet Count ; Thrombocytopenia/*diagnosis/*drug therapy/etiology ; Young Adult

Bullous systemic lupus erythematosus (SLE) is a kind of LE-non-specific bullous skin disease that is rarely induced by a medication. We describe the first case of bullous SLE to develop after administration of methimazole. A 31-yr-old woman presented with generalized erythematous patches, multiple bullae, arthralgia, fever, conjunctivitis, and hemolytic anemia. Biopsy of her bulla showed linear deposition of lgG, lgA, C3, fibrinogen, and C1q at dermo-epidermal junction. She was diagnosed as bullous SLE and treated with prednisolone, dapsone, hydroxychloroquine, and methotrexate. Our experience suggests that SLE should be considered as a differential diagnosis when bullous skin lesions develop in patients being treated for hyperthyroidism.
Adult ; Anti-Inflammatory Agents/therapeutic use ; Antirheumatic Agents/therapeutic use ; Antithyroid Agents/*adverse effects/therapeutic use ; Blister/chemically induced/pathology ; Drug Therapy, Combination ; Female ; Graves Disease/diagnosis/drug therapy ; Humans ; Hydroxychloroquine/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/chemically induced/*diagnosis/drug therapy ; Lupus Nephritis/diagnosis/drug therapy ; Methimazole/*adverse effects/therapeutic use ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Prednisolone/therapeutic use ; Skin/pathology

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