Objective:To study the clinical and immunological features of two case of rare antisynthetase syndrome (ASS), so as to improve the level of diagnosis and treatment.Methods:Two cases with rare antisynthetase syndrome admitted to the First Affiliated Hospital of Zhengzhou University from July 2020 to August 2022 were collected.Results:The two rare ASS were anti-Zo antibody and anti-Ha antibody positive patients, both of which had interstitial lung disease (ILD) as the main clinical manifestation and positive anti-Ro52 antibody. Two rare antisynthetase autoantibodies manifested cytoplasmic ANA indirect immunofluorescence (IIF) staining pattern, but it is different from the cytoplasmic dense speckled pattern of several common ASS antibodies. After treatment with glucocorticoids and immunosuppressants, case 1 died of respiratory failure due to a long course of disease and late diagnosis, the lung lesions of case 2 improved significantly.Conclusion:When encountering the cytoplasmic ANA fluorescent pattern in ILD patients, especially with anti-Ro52 antibody, it is necessary to screen more myositis specific antibodies to rule out the possibility of rare ASS.

Polymyalgia rheumatica (PMR) is a syndrome characterized by pain and morning stiffness in the neck and shoulder and pelvic girdles, as well as raised acute-phase reactants, with or without systemic symptoms, such as fever. Giant cell arteritis (GCA) is a systemic vasculitis of unclear etiology that involves systemic arteries, principally affecting medium- and large-sized arteries with skipped, segmental alterations and granulomatous vasculitis seen on histopathology. In China, epidemiological data describing GCA are still limited; thus, the prevalence might be underestimated. The involvement of vessels in GCA can cause irreversible visual impairment or loss and stroke, which are serious complications. PMR is three times more prevalent than GCA, and other specific diseases should be excluded before the diagnosis is established. PMR symptoms can be present in 40%-60% of patients with GCA. Conversely, GCA can develop in 15% of patients with PMR. Chinese Rheumatology Association, based on the clinical diagnosis and treatment guidelines in 2005, utilizing the experience and guidelines of diagnosis and treatment at home and abroad, formulated this specification to standardize the diagnosis and treatment of GCA and PMR and improve the patient′s prognosis.

Objective:To study the clinical features of infective endocarditis (IE) with positive anti-neutrophil cytoplasmic antibodies (ANCA) in order to improve the level of diagnosis and treatment.Methods:Eighteen IE cases with positive ANCA admitted to the First Affiliated Hospital of Zhengzhou University from June 2016 to July 2021 were collected. The demographic information, clinical symptom, laboratory tests, imaging examinations, treatment and clinical outcomes were analyzed retrospectively. Statistical program for social sciences (SPSS) 20.0 statistical software was used for analysis. Enumeration data were expressed as the number of cases and percentage (%), and measurement data were expressed as Mean± SD. Results:Twelve cases were male and 6 cases were female, with an average age of (50±16) years. Sixteen patients had positive PR3-ANCA, in which 2 cases had positive myeloperoxidase (MPO)-ANCA. The major clinical manifestations included fever (88.9%, 16/18), anemia (72.2%, 13/18), splenomegaly (44.4%, 8/18), cardiac murmur (33.3%, 6/18), arthralgia (22.2%, 4/18), liver damage (22.2%, 4/18), thromboembolic events (16.7%, 3/18), Osler's node (11.1%, 2/18) and renal dysfunction (11.1%, 2/18). Higher C-reactive protein (CRP), erythrocyte sedimentation (ESR) and procalcitionin (PCT) were detected in 83.3% (15/18) patients. The positive rate of blood culture was 50.0%(9/18) and streptococcus was the most common pathogen (77.8%, 7/9). Echocardiograms of all patients showed abnormal vegetation, most commonly involving the mitral valve (66.7%, 12/18) and aortic valve (33.3%, 6/18). Two patients were misdiagnosed as ANCA associated vasculitis (AAV), but the other one was diagnosed as AAV with IE as the first manifestation. Except for one case who died of multiple organ failure, all cases reached clinical recovery after surgery and antibiotic therapy.Conclusion:IE patients with positive ANCA may present with the clinical manifestations similar to AAV. We should highly alert to avoid misdiagnosis and treatment.

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

To analyze the clinical characteristics of patients with antisynthetase syndrome (ASS) and positive anti-Ro52 antibody. The clinical data of 203 ASS patients admitted to the First Affiliated Hospital of Zhengzhou University from 2017 to 2020 were analyzed retrospectively. Demographics, clinical manifestations, laboratory results, treatment and outcome were collected including data of 18 patients with rapidly progressive interstitial lung disease (RP-ILD). In total, the majority were women (148,72.9%). The average onset age was (51.9±13.3) years. There were 163 (80.3%) patients with positive anti-Ro52 antibody. The positivity in women (77.3% vs. 55.0%, P=0.004) was higher, and the median time from disease onset to diagnosis [4.5 (2.0, 24.0) months vs. 2.0 (1.0, 12.0) months, P=0.024] was longer in patients with positive anti-Ro52 antibody than those negative. Compared with negative patients, patients with positive anti-Ro52 antibody had a higher incidence of interstitial lung disease (ILD) (96.9% vs. 65.0%, P<0.001), arthritis (33.7% vs. 17.5%, P=0.046), and arthralgia (39.3% vs. 20.0%, P=0.022). Higher rate of positve antinuclear antibody (ANA) (85.3% vs. 55.0%, P<0.001), lower rate of positive anti-Jo-1 antibody (32.5% vs. 50.0%, P=0.039), lower albumin level [(34.6±5.2) g/L vs. (37.3±4.7) g/L, P=0.004] and lower lymphocyte counts [(1.4±0.8) ×10 9/L vs. (1.8±0.8) ×10 9/L, P=0.014] were more common in patients with positive anti-Ro52 antibody. The presence of anti-Ro52 antibody is associated with a particular phenotype of ASS, leading to common ILD, involvement of joints, high ANA positivity, low albumin and low lymphocyte counts.

To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG 4 was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmH 2O (1 mmH 2O=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG 4 tests for AAV or IgG 4-related disease.

Objective:To study clinical features of rheumatoid arthritis (RA) patients with peripheral neuropathy (PN).Methods:The clinical data of 46 RA patients with PN in the First Affiliated Hospital of Zhengzhou University from August 2012 to August 2019 were retrospectively analyzed, including clinical manifestations, laboratory and imaging results, previous treatment, treatment and clinical outcome. The other 92 RA patients without PN at the same period were selected as controls.Results:In RA patients with PN, the male to female ratio was 1∶2.1 with an average age (59.1±11.8) years. The course of RA and PN was 102.0 (19.0-156.0) months and 4.2 (0.7-5.5) months respectively. Numbness (84.8%, 39/46) and muscle weakness (21.7%, 10/46) were the most common symptoms. According to results of electromyography, polyneuropathy (60.0%, 27/46) was the predominant manifestation, followed by mononeuritis multiplex (31.1%, 14/46). Compared to RA patients, rheumatoid factor (RF) ( P<0.001) and the percentage of cutaneous vasculitis ( P=0.042) were higher in RA patients with PN. Logistic regression analysis revealed significant correlation between RF>178.4 IU/ml ( OR＝5.626, 95% CI 2.509-12.618, P<0.001) and development of PN. Paresthesia in 27 patients (58.7%, 27/46) were relieved after treatment of high dose glucocorticoid and immunoglobulins (IVIG). Twelve patients were followed up regularly and the mean duration of follow-up was 17.0(4.8-52.8)months. Paresthesia in 10 (10/12) patients were relieved compared to that at discharge, 1 (1/12) patient achieved complete remission. Conclusion:Numbness and muscle weakness are the common symptoms in RA patients with PN and polyneuropathy is the main type. RF>178.4 IU/ml is correlated with the development of PN in RA patients. Intensive treatment such as high dose glucocorticoid and IVIG are effective.

To investigate the clinical manifestations and imaging characteristics of patients with different types of infectious sacroiliitis. Clinical data of 40 patients diagnosed with infectious sacroiliitis were retrospectively analyzed. Among the 40 patients, 16 patients were diagnosed as non-brucellar and non-tuberculous infectious sacroiliitis (ISI), 13 with tuberculous infectious sacroiliitis (TSI), and 11 with brucellar sacroiliitis (BSI). In the ISI and TSI group, female patients accounted for 11/16, 12/13, while the proportion of unilateral involvement was 15/16 and 12/13, respectively. Compared with ISI and TSI group, BSI patients were mainly male (8/11) and presented more bilateral involvement (6/11) ( P<0.05). Bone erosion was more common in ISI and TSI groups than in BSI group (6/15, 7/11 and 2/10), as well as abscess formation (3/15, 4/11 and 1/10, respectively). Symptoms in all patients relieved 1-2 weeks after administration of antibiotics or anti-tuberculosis treatment, but the resolution of the magnetic resonance imaging findings delayed about 6 (3-9) months. ISI and TSI patients with infectious sacroiliitis should be differentiated from spondyloarthritis, with a characteristic of more female patients, unilateral sacroiliitis, bone erosion, soft tissue involvement and abscess formation. However, BSI patients are mainly male, more bilateral involvement and less bone destruction and abscess formation. Antibiotic therapy demonstrates significant therapeutic effects, but resolution of the magnetic resonance imaging findings responses late.

Objective:By analyzing the clinical characteristics of anti-Ku antibody associated disease, this paper aims to improve the diagnosis and treatment level of it.Methods:The clinical symptoms, laboratory tests and prognosis of 40 anti-Ku-antibody positive patients from the First Affiliated Hospital of Zhengzhou University from September 2017 to September 2019 were retrospectively collected, and then hierarchical clustering analyzed.Results:The average age of 40 anti-Ku positive patients was 48±18 years, and the male to female ratio was 1∶4. The average follow-up was (11±7) months, of which 2 cases were accompanied by malignant tumors and 3 cases died. Interstitial lung disease was most common and was found in 24 cases (60%). The most common disease was inflammatory myopathy (11 cases, 28%), followed by systemic lupus erythematosus (SLE) (9 cases, 22%). According to hierarchical cluster analysis, the anti-Ku-antibodies positive patients were divided into 3 groups. Among them, group A had the highest incidence of pulmonary interstitial fibrosis (84%, P<0.01), and the lowest incidence of renal involvement (0, P<0.01), cytopenia (0, P<0.01), serositis (0, P<0.01). Although the incidence of anti-Jo-1 antibody positivity in group A was the highest (16%, P=0.44) but without statistically significant difference. The characters of group A were in line with inflammatory myopathy. Group C had the highest incidence of renal involvement (57%), lupus rash (71%), cytopenia (57%), low complement (71%) and lupus-related antibodies positivity ( P value were all<0.05), which was in line with SLE. These two groups had their own significant biological characteristics, and were rarely overlapped. Conclusion:Anti-Ku antibody appears in a wide spectrum of autoimmune diseases, among which inflammatory myopathy is the most common, followed by SLE. Patients with anti-Ku antibody rarely have SLE and myositis overlapped, and the overall prognosis is good, but it is necessary to be alert to complications, such as tumors.

To investigate how Chinese rheumatologists treated patients with rheumatoid arthritis (RA). We performed a survey on the choices of first-line and second-line anti-RA therapies, prescription of methotrexate and glucocorticoids, assessment of disease activity and frequencies of follow-up at the Asia Pacific League of Associations for Rheumatology meeting 2016 in Shanghai. The majority (85.1%) of rheumatologists preferred methotrexate as first-line treatment. As alternative agents, 71.0% rheumatologists chose leflunomide or sulfasalazine. If methotrexate was not tolerable, only 8.6% rheumatologists would switch to parenteral administration. After failure of responding to methotrexate, 62.0% rheumatologists recommended to change or combine other conventional synthetic disease modifying anti-rheumatic drugs (DMARDs). Etanercept was the most popular biological option in 65.2% rheumatologists. Almost all (97.3%) rheumatologists prescribed methotrexate at an initial dose of 7.5 to 15 mg/week and 73.8% rheumatologists at a maximum of 10 to 15 mg/week. There were 49.3% rheumatologists prescribing oral glucocorticoids at first-line therapy. Surprisingly, 42.6% rheumatologists never or rarely assessed disease activity in daily work. For patients having achieved remission, 74.2% rheumatologists would follow up them every 1 to 3 months. This study suggests that most Chinese rheumatologists treat RA patients consistent with international guidelines, while the maximum dose of methotrexate, glucocorticoid as first-line treatment, assessment of disease activity and follow-up frequency are locally modified.