Efficacy and safety of pharmacological treatments for autoimmune disease-associated interstitial lung disease: A systematic review and network meta-analysis.

BACKGROUND: Immunosuppressants, biologic agents, antifibrotic drugs, and other drugs can be used to treat autoimmune disease-associated interstitial lung disease (ILD), but the preferred treatment is uncertain. We aimed to evaluate the efficacy and safety of multiple drugs in the treatment of autoimmune disease-associated ILD. METHODS: PubMed, Embase, Web of Science, Cochrane Central Register of Controlled Trials and ClinicalTrials.gov were searched for relevant randomized controlled trials (RCTs) from inception to July 2023. Primary outcomes were percentage of predicted forced vital capacity (FVC% predicted) and discontinuations for adverse events (AEs). We estimated summary mean differences (MDs) and odds ratios (ORs) using network meta-analysis with fixed effects. RESULTS: The analysis is based on 15 RCTs involving 1832 patients. In terms of FVC% predicted, mycophenolate mofetil (MMF) (MD 1.27, 95 % credible interval [CrI] 0.08 to 2.43), cyclophosphamide (1.89, 0.10 to 3.68), rituximab (9.29, 2.79 to 15.80), tocilizumab (6.30, 3.27 to 9.34), nintedanib (1.71, 0.54 to 2.88), pirfenidone (2.03, 0.65 to 3.40) and nintedanib+MMF (2.43, 0.95 to 3.89) were more effective than placebo. Analysis based on a small sample size showed that riociguat also had good therapeutic potential when compared with placebo. By contrast, bosentan and pomalidomide showed no significant difference compared with placebo. Regarding discontinuations for AEs, nintedanib (OR 2.09, 95 %CrI 1.20 to 3.73) and pirfenidone (3.46, 1.31 to 10.56) were associated with higher dropout rates than placebo, and the combination therapy of nintedanib+MMF did not increase the risk of AEs compared with nintedanib monotherapy. CONCLUSIONS: MMF, cyclophosphamide, rituximab, tocilizumab, nintedanib and pirfenidone are effective in the treatment of autoimmune disease-associated ILD. The efficacy of riociguat and the superiority of combination therapy need to be demonstrated in more RCTs. The tolerance of nintedanib and pirfenidone is a concern, but most of their AEs are mild and controllable.

Value of SLE-DAS in assessing disease activity in patients with systemic lupus erythematosus: a single-centre retrospective study.

OBJECTIVES: This study aimed to evaluate the clinical value of the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) for assessing disease activity in patients with SLE. METHODS: Clinical data were collected from patients with SLE who were admitted at the Second Affiliated Hospital of Soochow University from January 2009 to December 2022. The glucocorticoid dose grading was used as the gold standard for disease activity assessment in SLE. The SLE-DAS value was calculated, and the SLE disease activity status was graded based on the SLE-DAS value. Another scoring criterion, the SLE Disease Activity Index 2000 (SLEDAI 2000), served as a control. Spearman correlation analysis was used to calculate the correlation between the scoring criteria and other variables. RESULTS: The analysis included 396 patients with SLE. A strong correlation was found between SLE-DAS and SLEDAI 2000 (ρ=0.709, 95% CI 0.648 to 0.766, p<0.001), with median SLE-DAS and SLEDAI 2000 scores of 15.32 (7.90 to 24.45) and 13 (8 to 19), respectively. Compared with the SLEDAI 2000 value, the SLE-DAS value correlated better with glucocorticoid dose grading (ρ=0.434 vs 0.518), gammaglobulin use (ρ=0.170 vs 0.318) and immunosuppressant use (ρ=0.122 vs 0.221). A moderate correlation based on disease activity grading was found between SLE-DAS and glucocorticoid dose grading (ρ=0.441), whereas a mild correlation was observed between SLEDAI 2000 and glucocorticoid dose grading (ρ=0.325). Additionally, SLE-DAS revealed a positive correlation with severe thrombocytopenia, cardiac involvement and pulmonary involvement but not SLEDAI 2000. CONCLUSION: Compared with SLEDAI 2000, SLE-DAS may provide a more accurate disease activity assessment in patients with SLE, especially those with severe thrombocytopenia and cardiopulmonary involvement.

Classifying systemic lupus erythematosus using laboratory items alone: a preliminary study.

OBJECTIVES: To explore the performance of laboratory items alone in systemic lupus erythematosus (SLE) classification. METHODS: Our cohort consisted of 352 and 385 (control) patients with and without SLE. This study evaluated the performance of the American College of Rheumatology (ACR)-1997, Systemic Lupus International Collaborating Clinics (SLICC)-2012, European League Against Rheumatism (EULAR)/ACR-2019, and Systemic Lupus Erythematosus Risk Probability Index (SLERPI) using laboratory items alone, including blood and urine test results. RESULTS: The median ratio of laboratory items/total items was 66.7%, 75.0%, 60.4%, and 77.4% in ACR-1997, SLICC-2012, EULAR/ACR-2019, and SLERPI, respectively. After including laboratory items alone, the sensitivity of ACR-1997, SLICC-2012, EULAR/ACR-2019, and SLERPI was 31.3% (95% confidence interval [CI]: 26.4%-36.4%), 79.8% (95% CI: 75.3%-83.9%), 75.9% (95% CI: 71.0%-80.2%), and 85.2% (95% CI: 81.1%-88.8%), respectively. We referenced the SLERPI and removed the additional restrictions, i.e., SLICC-2012 criteria only needs to fulfill at least four items (mSLICC-2012) and EULAR/ACR-2019 criteria needs to have ≥ 10 points (mEULAR/ACR-2019) to qualify for SLE classification. The mSLICC-2012 and mEULAR/ACR-2019 criteria, including laboratory items alone, newly identified 13 and 25 patients, respectively. Based on laboratory items alone, the combination of mSLICC-2012, mEULAR/ACR-2019, and SLERPI identified 348 patients with an improved sensitivity of 90.6% (95% CI: 87.1%-93.5%). Patients, who were classified according to the mEULAR/ACR-2019 criteria, all met the other criteria. CONCLUSION: Incorporating laboratory items alone was clinically feasible to help identify SLE. SLERPI and SLICC-2012, using laboratory items alone, were more worthwhile to promote in the clinic compared with EULAR/ACR-2019. Key Points • Laboratory items play a crucial role in the SLE classification criteria, and incorporating laboratory items alone was clinically feasible to help in the identification of SLE. • The SLERPI and SLICC-2012, using laboratory items alone, were more worthwhile to promote in the clinic compared with EULAR/ACR-2019, and the combination of the two could further improve the sensitivity. • The relative simplicity of evaluating laboratory indices may help nonrheumatologists and inexperienced rheumatologists to identify SLE more quickly, thereby reducing the risk of delayed diagnosis in patients.

Tumour-associated antigens in systemic lupus erythematosus: association with clinical manifestations and serological indicators.

OBJECTIVES: To explore the relationship of tumour-associated antigens (TAAs) with the clinical manifestations and serological markers of SLE. METHODS: This was a retrospective study. Clinical data of SLE patients were extracted from the electronic medical records, including serum levels of TAAs such as alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), cancer antigen (CA) 19-9, CA125, CA15-3 and cytokeratin 19-fragments (CYFRA21-1). TAA positivity was defined as serum level exceeding the upper limit of the corresponding reference range. RESULTS: A total of 149 SLE patients (SLE group) and 149 age- and sex-matched healthy subjects (control group) were enrolled. Compared with healthy controls, the SLE group had higher positivity rates for CA19-9 and CYFRA21-1, and elevated serum levels of CA125, CA15-3 and CYFRA21-1. SLE patients with TAA positivity were older, had a higher prevalence of serous effusion, pericardial effusion, albuminuria and thrombocytopenia, and lower positivity rate for anti-dsDNA than patients without TAA positivity. The levels of serum creatinine (SCR), blood urea nitrogen, glutamic oxalate transaminase and 24-h urinary protein were also higher in SLE patients with TAA positivity, but platelet count and serum albumin levels were lower. On logistic regression, thrombocytopenia and SCR levels were identified as independent risk factors for TAA positivity. CA125 positivity rate and serum levels of CA125 were associated with SLE disease activity. CONCLUSION: The positivity rates and serum levels of some TAAs were elevated in SLE, and thrombocytopenia and SCR levels were independent risk factors for TAA positivity.

Association of serum tumor markers with serous effusion in systemic lupus erythematosus.

The objective of this study was to investigate the relationship between serum tumor markers and serous effusion in systemic lupus erythematosus (SLE) patients, thereby contributing preliminary data on the utility of these tumor markers in diagnosing serous effusion. In this retrospective analysis, clinical data of SLE patients were extracted from electronic medical records. This included the levels of serum tumor markers, including pro-gastrin-releasing peptide, neuron-specific enolase (NSE), cytokeratin-19 fragments (CYFRA 21-1), various carbohydrate antigens (CA 153, CA 125, CA 19-9), along with carcinoembryonic antigen, and alpha-fetoprotein. Positivity of tumor markers was established based on serum levels surpassing the upper threshold of the respective reference ranges. This study included 149 eligible patients with SLE, of whom 38 (25.50%) had serous effusion, and the prevalence of pleural, pericardial, and peritoneal effusions was 11.41%, 14.77%, and 6.71%, respectively. The analysis revealed that patients with serous effusion had higher scores on the SLE Disease Activity Index 2000 (SLEDAI 2000) than those without serous effusion. Notably, this disparity remained significant when the serositis score was excluded from the SLEDAI 2000 calculation. The positivity rate and serum levels of CA 125 were higher in patients with serous effusion and pleural effusion. Patients with pericardial effusion demonstrated an elevated CYFRA 21-1 positivity rate and serum CA 125 and CYFRA 21-1 levels compared to patients without pericardial effusion. CA 125 and NSE were higher both in terms of positivity rate and serum levels for patients with peritoneal effusion. Through receiver operating characteristic curve analysis, a moderate relationship was discerned between the conjoined levels of CYFRA 21-1 and CA 125 and the occurrence of pericardial effusion. Additionally, CA 125, NSE, and their combination revealed the moderate diagnostic ability of peritoneal effusion. In summary, this study observed elevated serum levels of various tumor markers in SLE patients exhibiting serous effusion, which is likely attributable to lupus-induced inflammation. These findings suggest that serum tumor markers can be valuable in diagnosing pericardial and peritoneal effusions.

A Rare Case of Primary Sjogren's Syndrome Coexisting With Gilbert Syndrome.

Gilbert syndrome (GS) is an autosomal recessive inherited bilirubin metabolism disorder characterized by chronic unconjugated hyperbilirubinemia in the absence of hemolysis and liver disease. Primary Sjogren's syndrome (pSS), mainly occurring in women, is a common connective tissue disease (CTD) wherein bilirubin levels are generally reduced. We report a rare case of pSS coexisting with GS. A 35-year-old female patient presented to our hospital with pSS and chronic unconjugated hyperbilirubinemia, for which low-dose methylprednisolone was ineffective. The patient's liver function test results were normal, serological tests for hepatitis virus were negative, and abdominal ultrasound did not indicate abnormal liver morphology. Bone mineral density determination showed that the Z scores of the left femoral neck and lumbar spine were -1.9 and -2.6, respectively, with T scores of -2.1 and -2.8, respectively. Full-exon sequencing revealed a homozygous TA insertion in the TATA box (A(TA)7TAA) and a heterozygous base substitution from C to A at nucleotide position 686 in exon 1 (c.686C>A) in the uridine glucuronosyltransferase 1A1 (UGT1A1) gene. Therefore, the patient was diagnosed with pSS, GS, and osteoporosis. The dose of methylprednisolone was then reduced and gradually stopped, and treatment for osteoporosis was strengthened. To our knowledge, this is the first report of pSS with GS. It is important to clarify the cause of hyperbilirubinemia in patients with CTD, including pSS, which affects the formulation of correct treatment plans.

Systemic Lupus Erythematosus Risk Probability Index: ready for routine use? Results from a Chinese cohort.

OBJECTIVES: To evaluate the performance of Systemic Lupus Erythematosus Risk Probability Index (SLERPI) in patients with SLE using a Chinese cohort. METHODS: The Chinese cohort included 352 patients with and 385 without SLE (control group). The clinical data of patients, including demographic data, clinical findings and serological profiles, were collected. Patients with an SLERPI score >7 were classified as SLE. The performance of the American College of Rheumatology (ACR)-1997, Systemic Lupus International Collaborating Clinics (SLICC)-2012 and European League Against Rheumatism (EULAR)/ACR-2019 criteria were used as references. RESULTS: Of these four classification criteria, SLERPI has the highest sensitivity (98.3% (95% CI 96.3% to 99.4%)), but lowest specificity (89.4% (95% CI 85.8% to 92.2%)). In the control group, patients eligible for the classification criteria for SLE were mainly those with primary Sjogren's syndrome (pSS) and undifferentiated connective tissue disease (UCTD), which adversely affected the specificity of the classification criteria. Moreover, significantly more patients with pSS and UCTD met SLERPI than those who met other classification criteria. After excluding patients with pSS and UCTD from the control group, the specificity and accuracy of SLERPI improved to 94.3% (95% CI 91.0% to 96.6%) and 96.5% (95% CI 95.0% to 97.9%), respectively, and both outperformed the EULAR/ACR-2019 criteria. The time to SLERPI classification was the same as their clinical time to diagnosis in 261 patients, earlier than the clinical diagnosis in 23 patients and later than the clinical diagnosis in 9 patients. A total of 280 patients had the same time to SLERPI classification as EULAR/ACR-2019, 8 patients had earlier than EULAR/ACR-2019 and 1 patient had later than EULAR/ACR-2019. CONCLUSION: SLERPI performed well in patients with SLE, particularly for the earlier diagnosis of SLE.

Clinical Characteristics of Dermatomyositis with Interstitial Lung Disease: A Retrospective Case-Control Study.

INTRODUCTION: Interstitial lung disease (ILD) is a common complication of dermatomyositis (DM) and one of the main risk factors for poor prognosis in DM patients. The aim of this study was to reveal the clinical characteristics of DM patients with ILD. METHODS: Clinical data from the Second Affiliated Hospital of Soochow University were used to conduct a retrospective case-control study. Univariate and multivariate logistic regression analysis were performed to identify risk factors for ILD in DM. RESULTS: A total of 78 DM patients were included in this study, including 38 DM patients with ILD and 40 DM patients without ILD. Compared with patients without ILD, patients with ILD were older (59.6 vs. 51.2 years, P = 0.004), and had higher rates of clinically amyopathic DM (CADM) (45 vs. 20%, P = 0.019), Gottron's papules (76 vs. 53%, P = 0.028), mechanic's hands (13 vs. 0%, P = 0.018), myocardial involvement (29 vs. 8%, P = 0.014), and higher positive rates of anti-SSA/Ro52 (74 vs. 20%, P < 0.001) and anti-melanoma differentiation-associated gene-5 (MDA5) (24 vs. 8%, P = 0.048) antibodies, while albumin (ALB) (34.5 vs. 38.0 g/l, P = 0.006), prognostic nutritional index (PNI) (40.3 vs. 44.7, P = 0.013), the rates of muscle weakness (45 vs. 73%, P = 0.013) and heliotrope rash (50 vs. 80%, P = 0.005) were lower. In addition, the five patients who died were all DM patients with ILD (13 vs. 0%, P = 0.018). Multivariate logistic regression showed that old age (odds ratio [OR] = 1.119, 95% confidence interval [CI] = 1.028-1.217, P = 0.009), Gottron's papules (OR = 8.302, 95% CI = 1.275-54.064, P = 0.027) and anti-SSA/Ro52 (OR = 24.320, 95% CI = 4.102-144.204, P < 0.001) were independent risk factors for ILD in DM. CONCLUSIONS: DM patients with ILD usually present with older age, higher rates of CADM, Gottron's papules, mechanic's hands, myocardial involvement, higher positive rates of anti-MDA5 and anti-SSA/Ro52 antibodies, lower ALB, PNI, and lower rates of muscle weakness and heliotrope rash. Old age, Gottron's papules, and anti-SSA/Ro52 were independent risk factors for ILD in DM.

Diagnostic accuracy of the European League against rheumatism/American College of Rheumatology-2019 versus the Systemic Lupus International Collaborating Clinics-2012 versus the ACR-1997 classification criteria in adult systemic lupus erythematosus: A systematic review and meta-analysis.

Aim: To evaluate the diagnostic performance of the American College of Rheumatology (ACR)-1997, the Systemic Lupus International Collaborating Clinics (SLICC)-2012, and the European League against Rheumatism (EULAR)/ACR-2019 classification criteria in adult patients with systemic lupus erythematosus (SLE). Methods: PubMed, Embase, Web of Science and Cochrane Library databases were searched for literature comparing the three classification criteria of ACR-1997, SLICC-2012 and EULAR/ACR-2019, which took clinical diagnosis as reference. Meta-analysis was used to evaluate and compare the sensitivity, specificity and diagnostic odds ratio of ACR-1997, SLICC-2012 and EULAR/ACR-2019. To assess the early diagnosis capability of the classification criteria, subgroups of patients with disease duration < 3 years and < 1 year were selected for comparison of sensitivity and specificity based on the inclusion of the original study. The sensitivity and specificity of each item in three sets of classification criteria were evaluated. In addition, the clinical and immunological characteristics of patients who did not meet the three classification criteria were compared. Results: Nine original studies were included in the analysis, including 6404 SLE patients and 3996 controls. Results showed that the diagnostic odds ratios (95% confidence interval) of the SLICC-2012 [136.35 (114.94, 161.75)] and EULAR/ACR-2019 [187.47 (158.00, 222.42)] were higher than those of the ACR-1997 [67.53 (58.75, 77.63)]. Compared with ACR-1997[(0.86 (0.82, 0.89)], SLICC-2012[(0.96 (0.93, 0.97)] and EULAR/ACR-2019[(0.95 (0.92, 0.97)] had higher sensitivity. The specificity of the three classification criteria was similar: ACR-1997, SLICC-2012, and EULAR/ACR-2019 were 0.93 (0.89, 0.95), 0.86 (0.79, 0.91), and 0.91 (0.85, 0.95), respectively. The sensitivity of SLICC-2012 and EULAR/ACR-2019 were higher than that of ACR-1997 in early-course subgroups. Patients who did not meet ACR-1997 had more hypocomplementemia, patients who did not meet SLICC-2012 had more cutaneous lupus and photosensitivity, and patients who did not meet EULAR/ACR-2019 had more cutaneous lupus and leucopenia. Conclusions: SLICC-2012 and EULAR/ACR-2019 have better diagnostic ability than the ACR-1997, and the sensitivity of the former two criteria is also higher than that of the latter; Moreover, the SLICC-2012 and EULAR/ACR-2019 for patients in the early stages of disease performed equally excellent.

Allergic Skin Rash Caused by Iguratimod: A Report of Two Cases.

Iguratimod has been used in the treatment of rheumatoid arthritis and Sjogren's syndrome (SS). Herein, we report two cases of skin allergic reactions caused by iguratimod in our hospital. Case 1 was a woman with SS who developed diffuse pruritus erythema after three weeks of combination therapy of hydroxychloroquine (HCQ) and iguratimod. When the patient was again prescribed iguratimod after the rash subsided, the pruritus erythema reappeared. Case 2 was a 23-year-old girl treated with prednisone, HCQ, and mycophenolate mofetil for systemic lupus erythematosus and SS. In the follow-up treatment, mycophenolate mofetil was replaced by iguratimod. On the 20th day of treatment, a pruritic erythematous maculopapular rash appeared. To the best of our knowledge, this is the first study to report the characteristics of an allergic rash caused by iguratimod. It is better to administer HCQ and iguratimod successively rather than simultaneously to a patient.

Iguratimod alleviates tubulo-interstitial injury in mice with lupus.

INTRODUCTION: Tubulo-interstitial injury is a poor prognostic factor for lupus nephritis (LN). Here, we tested whether iguratimod could inhibit tubulo-interstitial injury in LN. METHODS: MRL/lpr mice, an animal model of lupus, were treated with iguratimod or vehicle solution. Pathological changes of kidney were evaluated blindly by the same pathologist. Renal type I collagen (COL-I), IgG, E-cadherin, fibroblast-specific protein 1 (FSP-1) were detected by immunofluorescence, immunohistochemical staining or quantitative real-time PCR. After treated with transforming growth factor ß1 (TGF-ß1) and iguratimod, E-cadherin, fibronectin, Smad2/3, p38 MAPK, p-Smad2/3, and p-p38 MAPK, ß-catenin and TGF-ß type II receptor (TGFßRII) in HK2 cells were measured by western blotting, quantitative real-time PCR or immunofluorescence. RESULTS: Iguratimod reduced immune deposition along the tubular basement membrane, inhibited the tubulo-interstitial infiltration of inflammatory cells, and alleviated tubular injury in MRL/lpr mice. Moreover, Iguratimod eased the tubulo-interstitial deposition of collagen fibers, which was confirmed by decreased expression of COL-I. Furthermore, iguratimod suppressed the expression of FSP-1 and increased that of E-cadherin in renal tubular epithelial cells. In HK2 cells cultured with TGF-ß1, iguratimod treatment not only reversed cellular morphological changes, but also prevented E-cadherin downregulation and fibronectin upregulation. In addition, iguratimod inhibited phosphorylation of TGFßRII, Smad2/3 and p38 MAPK in HK2 cells treated with TGF-ß1, and also blocked nuclear translocation of ß-catenin. CONCLUSION: Iguratimod eased tubulo-interstitial lesions in LN, especially tubulo-interstitial fibrosis, and might have potential as a drug for inhibiting the progression of tubulo-interstitial fibrosis in LN.

Detection of the disease activity with ankylosing spondylitis through intravoxel incoherent motion diffusion-weighted MR imaging of sacroiliac joint.

OBJECTIVE: To explore the value of the quantitative parameter of intravoxel incoherent motion diffusion (IVIM-DWI) at 3.0 T MRI of the sacroiliac joint in differentiating different disease activity statuses of ankylosing spondylitis (AS) and to compare it with traditional diffusion-weighted imaging (DWI) and Spondyloarthritis Research Consortium of Canada (SPARCC) score. METHODS: 56 AS patients (active group, inactive group) and 24 healthy controls were included. Clinical data, quantitative parameters of IVIM-DWI MR images and the SPARCC scores were collected. The Kruskal-Wallis test was used to compare the differences between the groups. Receiver operating characteristic (ROC) curve analysis of histogram data and the SPARCC scores identified the efficacy of the three groups. The Spearman correlation coefficients were used to analyse the correlation between the quantitative IVIIM-DWI parameters and the SPARCC score. RESULTS: The f (10th percentile) and SPARCC score of the active group were significantly higher than those of the inactive group. The f (10th, 25th, 50th percentiles), Dslow (average, entropy, 10th ~ 90 th percentiles), Dfast (kurtosis, skewness), ADC (average, 10th ~ 90 th percentiles) and the SPARCC score of the active group were significantly higher than the control group (p < 0.05). The AUC of the SPARCC score was the highest (0.799) in the identification between the active and inactive groups, and the sensitivity and specificity were 69.23 and 82.35%, respectively, at the cut-off value of 12. The SPARCC score was positively correlated with each percentile and the average value. CONCLUSIONS: Quantitative IVIIM-DWI parameters are helpful for the identification of different AS disease activity levels and are superior to traditional DWI. IVIM-DWI quantitative parameters had a good correlation with the SPARCC score. ADVANCES IN KNOWLEDGE: A new MR technology-quantitative parameters of IVIM-DWI contribute to the identification of AS disease activity. IVIM-DWI quantitative parameters were well correlated with the SPARCC score.

The Clinical Characteristics of Leukopenia in Patients with Systemic Lupus Erythematosus of Han Ethnicity in China: A Cross-Sectional Study.

INTRODUCTION: The characteristics of leukopenia in patients with systemic lupus erythematosus (SLE) in different studies are different, which may be related to region, race, and sample size. Moreover, the extent of leukocyte count decline remains to be studied. This study aimed to analyze the clinical characteristics of leukopenia in patients with SLE of Han ethnicity in China. METHODS: A single-center, retrospective, cross-sectional study was conducted in Chinese Han patients with SLE from June 2013 to August 2020. RESULTS: A total of 125 patients with SLE were included in the study, and 104 age- and sex-matched healthy controls were recruited. The prevalence of leukopenia, neutropenia, and lymphopenia was 40.0, 20.8, and 55.2%, respectively. The median leukocyte count in the leukopenia group was 2.80 × 109/l, the median neutrophil count in the neutropenia group was 1.40 × 109/l, and the median lymphocyte count in the lymphopenia group was 0.60 × 109/l, which was 47.06, 40.58, and 30.00% of the median of the healthy control group, respectively. The lymphocyte count of SLE patients without lymphopenia was also lower than that of healthy controls, and the lymphocyte count was negatively correlated with the SLE disease activity index 2000 score in all patients with SLE. Independent risk factors for neutropenia include decreased platelet count and lymphocyte count, as well as the presentation of cylindruria. For lymphopenia, the independent risk factors were positivity for anti-dsDNA antibody and Coombs' test, decreased platelet count, and cylindruria. CONCLUSIONS: In Han Chinese patients with SLE, leukopenia, neutropenia, and lymphopenia are common clinical manifestations, and the degree of reduction in blood cell count was also remarkable. Lymphopenia is associated with disease severity in patients with SLE. The correlation between Coombs' test results and lymphopenia deserves further study.