OBJECTIVES: To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA. METHODS: A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results. RESULTS: Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05). CONCLUSIONS There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.
Child ; Humans ; Lupus Nephritis/complications* ; Kidney/pathology* ; Retrospective Studies ; Thrombotic Microangiopathies/therapy* ; Prognosis ; Hypertension/complications* ; Cholesterol ; Lupus Erythematosus, Systemic

OBJECTIVETo investigate diagnostic value of MRI, X ray and CT for bone infarction in children with systemic lupus erythematosus.

METHODSEleven systemic lupus erythematosus children with bone infarction were retrospectively analyzed from January 2015 to January 2017 , and tested by MRI, X-ray and CT. Among them, including 1 male and 10 females aged from 6 to 16 years old with an average of 13 years old. All patients were detected by MRI, 9 patients were detected by X-ray and 3 patients were detected by CT, imaging findings were analyzed.

RESULTSThe location of bone infarction involved 60 sits, 30 sites located on metaphyseal-diaphyseal region, 8 located on patella, 21 located on epiphysis, and 1 located on talus. Focus of 11 patients were detected by MRI, the main manifestation showed geographic change, long T1 and T2 signal could seen around focus, and showed double ring sign and three ring sign; 5 of 9 patients by X-ray examination detected focus;2 of 3 patients by CT examination detected focus. No abnormity seen at early stage by X-ray and CT examination, and low density focus around harden edge at chronic stage.

CONCLUSIONSMRI could display bone fracture at early stage, X-ray and CT could only display lesion at chronic stage, MRI is the most effective method in diagnosing bone infarction.

Adolescent ; Bone and Bones ; diagnostic imaging ; pathology ; Child ; Female ; Humans ; Infarction ; diagnostic imaging ; Lupus Erythematosus, Systemic ; complications ; Magnetic Resonance Imaging ; Male ; Radiography ; Tomography, X-Ray Computed

Systemic lupus erythematosus-related acute pancreatitis (SLEAP) has a poor prognosis with a high mortality. We described the clinical features of SLEAP, and discussed the feasibility of plasma exchange (PE) combined with glucocorticosteroids (GC) in short-term prognosis and possible mechanism in reducing serum inflammatory cytokine IL-6 and removing serum lipids. A retrospective study was performed by an independent rheumatologist. Medical records of SLEAP from March 2010 to December 2014 were retrieved from Tongji Hospital information system, and patients were divided into two groups according to whether PE therapy was adopted. Sixteen patients treated with PE in combination with GC were classified as group A, and the other 10 patients who were treated with merely GC were classified as group B. Patients' clinical remission rate and average daily GC dosage after two-week therapy were compared between the two groups. Patients' serum inflammatory cytokines and lipid concentration were compared between baseline and after two-week treatment in both groups. Pearson correlation test was performed to determine association between serum cytokines and Ranson score. SLEDAI score in group A patients at baseline (14.8±3.1) showed no statistical difference from that in group B (14.1±3.3). At baseline serum IL-6 levels had no significant difference between group A [13.14 (11.12, 16.57) mg/L] and group B [14.63 (11.37, 16.37) mg/L]; after two-week therapy IL-6 decreased significantly in group A [9.16 (7.93, 10.75)mg/L] while it did not show decreasing trend in group B [13.62 (9.29,17.63) mg/L]. Serum lipid concentration after two-week therapy in group A [(TC=5.02±0.53, TG=1.46±0.44) mmol/L] decreased significantly compared to baseline [(TC=6.11±0.50, TG=2.14±1.03) mmol/L], while similar tendency was not observed in group B. The remission rate after two-week therapy was higher in group A (70.0%) than in group B (25.0%). Acute pancreatitis (AP) was one of the clinical manifestations of active SLE. PE combined with GC could reduce serum IL-6 level, and remove serum lipid to improve short-term prognosis. Therefore, it might be a safe and effective way in treating SLEAP and was worth continuing to explore its feasibility.
China ; Female ; Glucocorticoids ; administration & dosage ; Humans ; Interleukin-6 ; blood ; Lipids ; blood ; Lupus Erythematosus, Systemic ; complications ; genetics ; pathology ; therapy ; Male ; Middle Aged ; Pancreatitis ; blood ; etiology ; pathology ; therapy ; Plasma Exchange ; methods ; Prognosis

The severity of systemic lupus erythematosus (SLE) patients with pulmonary bacterial infection varies widely. We investigated the significance of procalcitonin (PCT) and C-reactive protein (CRP) in evaluating the severity of pulmonary infection in SLE patients. This retrospective study contained a total of 117 patients (107 women and 10 men) with SLE from January 2010 to June 2011. Serum levels of PCT and CRP were measured by enzyme-linked immunosorbent assay. The severity of pulmonary bacterial infection (PBI) was evaluated using the pneumonia severity index (PSI). SLE patients with PBI, particularly those with bacterial isolates, had significantly higher levels of serum PCT and CRP than those without PBI. Serum PCT and CRP were not associated with SLE disease activity, but positively with the values of PSI in active SLE patients with PBI. Serum levels of PCT and CRP may be additional biomarkers in evaluating the severity of PBI in lupus patients.
Aged ; Bacterial Infections ; blood ; microbiology ; pathology ; C-Reactive Protein ; metabolism ; Calcitonin ; blood ; Female ; Humans ; Lung ; microbiology ; pathology ; Lupus Erythematosus, Systemic ; blood ; complications ; pathology ; Male ; Severity of Illness Index

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

Systemic lupus erythematosus (SLE) predominantly affects women in their reproductive years and has a significant impact on childbearing. We investigated the influence of personal decision on family size among Korean women with SLE and factors that affect the decisions. A case-control study comparing childbearing history and decisions of 112 SLE patients and 135 controls was performed. Women with SLE participating in the Network for Lupus Clinical Research in South Korea and matching controls between ages of 18-45, who are/were married or living with a partner were included. Data regarding socio-demographics, reproductive history, and childbearing decisions were collected through a survey using a standardized questionnaire and medical record review. More women with SLE reported at least one pregnancy (85.7% vs. 71.9%, P = 0.009) or at least one live birth (85.7% vs. 71.9%, P = 0.003) compared with controls. Mean number of pregnancies was significantly higher (2.4 ± 1.6 vs. 1.4 ± 1.3, P < 0.001), and mean number of live births was significantly lower in women with SLE (1.2 ± 0.8 vs. 1.6 ± 0.8, P < 0.001). Significantly more women with SLE made the decision not to have children compared with controls (54.5% vs. 40.7%, P = 0.031), and health-related concerns were the major cause of the decision. Other socio-demographic factors did not influence the decision to limit childbearing in SLE women. The disease-related concerns had significant impact on family size and childbearing decisions among Korean women with SLE.
Adolescent ; Adult ; Asian Continental Ancestry Group ; Case-Control Studies ; Demography ; Family Characteristics ; Female ; Humans ; Live Birth ; Lupus Erythematosus, Systemic/*pathology/psychology ; Odds Ratio ; Pregnancy ; Pregnancy Complications ; Reproductive Behavior/*psychology ; Republic of Korea ; Surveys and Questionnaires ; Tertiary Care Centers ; Young Adult

Thyroglossal duct carcinoma is a malignant tumor which occurs in the thyroglossal duct cyst. The incidence of thyroglossal duct carcinoma has been reported as approximately 1%. Up to now, just about 250 cases of thyroglossal duct carcinoma have been reported in the literature,most of which are single case reports and small case series. In most cases, the diagnosis of the thyroglossal duct carcinoma is not made until the histologic examination after surgery operation. The preoperative examination such as CT or fine needle aspiration cytology can help the preoperative diagnosis. But the surgical treatment for the thyroglossal duct carcinoma is still controversial. Now we report a case of a thyroglossal duct carcinoma combined with systemic lupus erythematosus. The patient herself found an anterior neck mass in the median submental region one year ago. The preoperative CT examination suggested thyroglossal duct cyst with pouch canceration(papillary carcinoma). Then she underwent a Sistrunk procedure and level I neck dissection, and the histopathological diagnosis was thyroglossal duct carcinoma. The patient was treated with levothyroxine therapy at suppressive dose after the surgery. Now the patient is at regular follow-up with no relapse occur.
Biopsy, Fine-Needle ; Carcinoma, Papillary ; complications ; diagnosis ; pathology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; pathology ; Neck Dissection ; Skin ; Thyroglossal Cyst ; complications ; diagnosis ; pathology ; Thyroid Neoplasms ; complications ; diagnosis ; pathology

OBJECTIVETo identify the risk factors which will indicate the Pneumocystis carinii (Pc) infection in children with systemic lupus erythematosus (SLE) and investigate the clinical features and to elevate the level to find out the high-risk patients and make early diagnosis and treatment.

METHODThe characteristics, clinical features, laboratory examinations, treatment and prognosis of Pneumocystis carinii pneumonia (PCP) in children with SLE under 18 years of age treated in our hospital between January 2000 and January 2013 were prospectively reviewed. A comparison was made with the 26 cases of SLE children without PCP who were matched for gender, age and course, and a literature review was made.

RESULTS(1) Five cases were enrolled, 3 were male and 2 female. Their age range was 13-17 (14.0 ± 1.6) years. All the children had kidney involvement. The courses were from 3 months to 4.5 years. All patients were receiving daily glucocorticoid therapy and immunosuppressive drugs before the diagnosis of PCP.Four patients were in the inactive phase of SLE (SLEDAI 2-4 points), and the fifth case was in active phase (SLEDAI 8, low complement 2 points, anti-dsDNA antibody positive 2 points, urine-protein 4 points). (2) Besides the clinical manifestations of SLE, most patients had progressive dyspnea, fever and dry cough at onset of PCP. Two children accepted mechanical ventilation because of respiratory failure. The mean duration of the symptoms to diagnosis was 10-30 (17.6 ± 7.8) days. Lactose dehydrogenase (LDH) was elevated more or less, median was (700 ± 263) U/L. Lymphocyte count were (0.3-1.4)×10(9)/L (median 0.5×10(9)/L), and three children had CD4 T lymphocyte count <0.3×10(9)/L. Arterial blood gas analyses showed severe hypoxemia. Chest radiographs showed in all cases diffuse interstitial infiltration. Pc was positive in the sputum. All patients were treated with trimethoprim-sulfamethoxazole and corticosteroids.

CONCLUSIONWhen SLE children are treated with corticosteroids and immunosuppressive drugs, low lymphocyte count is the risk factor for Pc infection.It is essential to monitor lymphocyte count.We should pay more attention to fever, dry cough and hypoxemia. Chest radiologic examination may help diagnose the PCP in SLE children.It may be helpful for SLE children whose CD4T lymphocyte was below 0.3×10(9)/L to take trimethoprim-sulfamethoxazole for PCP prophylaxis.

Adolescent ; Anti-Infective Agents ; adverse effects ; therapeutic use ; Case-Control Studies ; Child ; Female ; Glucocorticoids ; adverse effects ; therapeutic use ; Humans ; Immunosuppressive Agents ; adverse effects ; therapeutic use ; Kidney Diseases ; etiology ; Lung ; pathology ; Lupus Erythematosus, Systemic ; complications ; drug therapy ; Lymphocyte Count ; Male ; Opportunistic Infections ; drug therapy ; epidemiology ; Pneumonia, Pneumocystis ; drug therapy ; epidemiology ; Prognosis ; Retrospective Studies ; Risk Factors ; Trimethoprim, Sulfamethoxazole Drug Combination ; therapeutic use

BACKGROUNDLupus hepatitis is yet to be characterized based on its clinical features and is often difficult to differentially diagnose from other liver diseases. We aimed to elucidate clinical, histopathological and immunopathological features of lupus hepatitis and to evaluate primarily the effectiveness of liver immunopathological manifestations on differential diagnosis of lupus hepatitis from other liver diseases.

METHODSA retrospective study was performed to analyze clinical features of lupus hepatitis in 47 patients out of 504 inpatients with systemic lupus erythematosus (SLE) in First Affiliated Hospital of Sun Yat-sen University, China from May 2006 to July 2009, and to evaluate the association between lupus hepatitis and SLE activity. Additionally, liver histopathological changes by hematoxylin and eosin (HE) staining and immunopathological changes by direct immunofluorescence test in 10 lupus hepatitis cases were analyzed and compared to those in 16 patients with other liver diseases in a prospective study.

RESULTSOf 504 SLE patients, 47 patients (9.3%) were diagnosed to have lupus hepatitis. The prevalence of lupus hepatitis in patients with active SLE was higher than that in those with inactive SLE (11.8% vs. 3.2%, P < 0.05). The incidence of hematological abnormalities in patients with lupus hepatitis was higher than that in those without lupus hepatitis (40.4% vs. 21.7%, P < 0.05), such as leucocytes count (2.92×10(9)/L vs. 5.48×10(9)/L), platelets count (151×10(9)/L vs. 190×10(9)/L), serum C3 and C4 (0.34 g/L vs. 0.53 g/L; 0.06 g/L vs. 0.09 g/L) (P < 0.05); 45 of 47 (95.7%) lupus hepatitis patients showed 1 upper limit of normal (ULN) < serum ALT level < 5 ULN. The liver histopathological features in patients with lupus hepatitis were miscellaneous and non-specific, similar to those in other liver diseases, but liver immunopathological features showed positive intense deposits of complement 1q in 7/10 patients with lupus hepatitis and negative complement 1q deposits in all patients with other liver diseases (Fisher's exact test, P = 0.011).

CONCLUSIONSLupus hepatitis was not infrequent in active SLE patients which would be one of the indices indicating SLE activity. Positive intense deposit of complement 1q in liver may be a characteristic immunopathological feature of lupus hepatitis, which provides a new way to differentially diagnose lupus hepatitis from other liver diseases.

Adolescent ; Adult ; Aged ; Child ; Cohort Studies ; Complement C1q ; analysis ; Female ; Hepatitis, Autoimmune ; etiology ; immunology ; pathology ; Humans ; Liver ; pathology ; Lupus Erythematosus, Systemic ; complications ; Male ; Middle Aged ; Retrospective Studies

Adolescent ; Biological Products ; administration & dosage ; therapeutic use ; Child ; China ; Evidence-Based Medicine ; Glucocorticoids ; adverse effects ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lupus Erythematosus, Systemic ; complications ; pathology ; therapy ; Lupus Nephritis ; etiology ; pathology ; therapy ; Osteoporosis ; etiology ; pathology ; therapy ; Practice Guidelines as Topic ; standards ; Severity of Illness Index ; Societies, Medical