Drug-induced immune thrombocytopenia is an adverse reaction marked by accelerated destruction of blood platelets. In cancer therapy, thrombocytopenia has many other causes including bone marrow suppression induced by chemotherapeutic agents, infection, and progression of cancer; drug-induced thrombocytopenia can easily be misdiagnosed or overlooked. Here, we present a case of an ovarian cancer patient with a history of mixed connective tissue disease who underwent surgery followed by treatment with paclitaxel, cisplatin, and bevacizumab. The patient developed acute isolated thrombocytopenia after the sixth cycle. Serum antiplatelet antibody testing revealed antibodies against glycoprotein IIb. After we analyzed the whole therapeutic process of this patient, drug-induced immune thrombocytopenia was assumed, and bevacizumab was conjectured as the most probable drug. Thrombocytopenia was ultimately successfully managed using recombinant human thrombopoietin, prednisone, and recombinant human interleukin-11. We provide a summary of existing literature on immune thrombocytopenia induced by bevacizumab and discuss related mechanisms and triggers for drug-induced immune thrombocytopenia. The present case underscores the potential of bevacizumab to induce immune-mediated thrombocytopenia, emphasizing the need for heightened vigilance towards autoimmune diseases or an autoimmune-activated state as plausible triggers for rare drug-induced immune thrombocytopenia in cancer therapy.

UNASSIGNED: To investigate the clinical features, severity and prognosis of interstitial lung disease (ILD) in patients with mixed connective tissue disease (MCTD).
UNASSIGNED: We performed a retrospective study on clinical data of MCTD patients admitted to China-Japan Friendship Hospital between October 2012 and October 2022. Data including long-term follow-up were retrieved from medical records. We compared MCTD patients with and without ILD in terms of clinical features, laboratory and imaging findings, severity and treatment response.
UNASSIGNED: A total of 59 patients were included, with a mean age of 46 years, among which 91.5% (n = 54) were females. Symptoms of pulmonary involvement were present in 44 patients (74.6%, 95% CI: 62.3-84.9%). Based on lung high-resolution computed tomography (HRCT), ILD was diagnosed in 39 (66.1%) patients, among which 31 (79.5%) showed nonspecific interstitial pneumonia (NSIP) as the radiological pattern, 21 (53.9%) showed a reticulation pattern, while 24 (61.5%) showed ground glass opacity (GGO). Eight (13.6%) patients had pulmonary arterial hypertension (PAH), and 7 (11.9%) had pleural effusions. Based on pulmonary function tests (PFTs), 27 patients were divided into the mild 13 (48.1%) and moderate 14 (51.9%) groups. Multivariate analysis showed that gastroesophageal reflux (GER; OR=5.28, p=0.010) and cough (OR=4.61, p=0.043) were the predictive factors for ILD. With a median follow-up of 50 months, the mortality rate was 2.38%.
UNASSIGNED: ILD is common in MCTD patients, with NSIP as the common imaging pattern. Patients with GER and cough are relevant factors in the development of ILD. The majority of MCTD patients with ILD are mild to moderate in severity.

BACKGROUND: Fractional exhaled nitric oxide (FeNO) has been extensively studied in various causes of pulmonary hypertension (PH), but its utility as a noninvasive marker remains highly debated. The objective of our study was to assess FeNO levels in patients with idiopathic pulmonary arterial hypertension (IPAH) and mixed connective tissue disease complicating pulmonary hypertension (MCTD-PH), and to correlate them with respiratory functional data, disease severity, and cardiopulmonary function.
METHODS: We collected data from 54 patients diagnosed with IPAH and 78 patients diagnosed with MCTD-PH at the Shanghai Pulmonary Hospital Affiliated to Tongji University. Our data collection included measurements of brain natriuretic peptide (pro-BNP), cardiopulmonary exercise test (CPET), pulmonary function test (PFT), impulse oscillometry (IOS), and FeNO levels. Additionally, we assessed World Health Organization functional class (WHO-FC) of each patient.
RESULTS: (1) The fractional exhaled concentration of nitric oxide was notably higher in patients with IPAH compared to those with MCTD-PH. Furthermore, within the IPAH group, FeNO levels were found to be lower in cases of severe IPAH compared to mild IPAH (P = 0.024); (2) In severe pulmonary hypertension as per the WHO-FC classification, FeNO levels in IPAH exhibited negative correlations with FEV1/FVC (Forced Expiratory Velocity at one second /Forced Vital Capacity), MEF50% (Maximum Expiratory Flow at 50%), MEF25%, and MMEF75/25% (Maximum Mid-expiratory Flow between 75% and 25%), while in severe MCTD-PH, FeNO levels were negatively correlated with R20% (Resistance at 20 Hz); (3) ROC (Receiving operator characteristic curve) analysis indicated that the optimal cutoff value of FeNO for diagnosing severe IPAH was 23ppb; (4) While FeNO levels tend to be negatively correlated with peakPETO2(peak end-tidal partial pressure for oxygen) in severe IPAH, in mild IPAH they had a positive correlation to peakO2/Heart rate (HR). An interesting find was observed in cases of severe MCTD-PH, where FeNO levels were negatively correlated with HR and respiratory exchange ratio (RER), while positively correlated with O2/HR throughout the cardiopulmonary exercise test.
CONCLUSIONS: FeNO levels serve as a non-invasive measure of IPAH severity. Although FeNO levels may not assess the severity of MCTD-PH, their significant makes them a valuable tool when assessing severe MCTD-PH.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a severe complication of mixed connective tissue disease (MCTD) and contributes to increased morbidity and mortality. Still, the demographic characteristics and risk factors of PAH in MCTD remain poorly understood. This study explored risk factors for PAH development in MCTD.
METHODS: Data from patients with MCTD and PAH hospitalized from May 2009 to December 2022 in a single center were collected and compared with patients with MCTD without PAH. The variables were analyzed by logistic regression to identify the factors associated with PAH in patients with MCTD. The receiver-operating characteristic (ROC) curve was used to assess the diagnostic value of the identified factors.
RESULTS: Finally, 119 patients with MCTD were included; 46 had PAH. The mean age at PAH onset and diagnosis was 38.9 ± 13.4 and 39.9 ± 13.7 years, respectively. The median pulmonary arterial systolic pressure (PASP) was 67.0 mmHg. The median brain natriuretic peptide (BNP) level was 180.0 pg/ml at PAH diagnosis. Red cell distribution width (RDW) (OR: 2.128; 95% confidence interval: 1.497-3.026; P < 0.001) was associated with PAH in patients with MCTD. There was a positive correlation between RDW and PASP (r = 0.716, P < 0.001). At a cutoff of 15.2%, RDW had the best sensitivity (80.4%) and specificity (82.2%) for PAH.
CONCLUSIONS: RDW may serve as a sensitive index to predict PAH in patients with MCTD.

暂无摘要。

Anti-MDA5 antibody dermatomyositis (DM) is a special type of myositis, which can potentially cause rapidly progressive interstitial lung disease (RP-ILD). Mixed connective tissue disease (MCTD) is a complex disease with different characteristics of autoimmune connective tissue disease, associated with ILD. Both are rare diseases, and few patients with both diseases have been reported. A 71-year-old woman complained of palpitations, with a 2 months history of rash around her hands, extensor surface of right elbow, and the nape of her neck. Subsequently, the patient had acute exacerbation of dyspnea and tachypnea. Anti-Ro52, U1 RNP and MDA5 antibodies were positive; the presenting evidence was suggestive of anti-MDA5+ DM-RP-ILD complicated with MCTD. Our patient deteriorated rapidly and had a fatal outcome, despite \"triple therapy\" for RP-ILD. This case illustrates that patients with coexisting anti-MDA5+ DM and MCTD have the former\'s typical clinical manifestations, and may develop ILD quickly rather than slowly as in MCTD, especially with the coexistence of anti-Ro52 antibodies.

OBJECTIVE: We investigated plasma sirtuin-1 (SIRT1) levels in systemic lupus erythematosus (SLE) patients, and discussed potential of plasma SIRT1 as a biomarker for SLE.
METHODS: A total of 359 subjects, including 299 patients (89 SLE, 50 rheumatoid arthritis, 30 osteoarthritis, 30 gout, 38 Sjögren\'s syndrome, 20 ankylosing spondylitis, 30 mixed connective tissue disease, 12 systemic sclerosis) and 60 healthy controls were recruited. SIRT1 in plasma of SLE patients was detected by enzyme-linked immunosorbent assay. Relationship between SIRT1 levels, clinical, laboratory characteristics in SLE patients was discussed. Plasma SIRT1 to discriminate SLE from different rheumatic patients and healthy controls was assessed by receiver operating characteristic (ROC) curve analysis.
RESULTS: SIRT1 levels were elevated in SLE patients compared with healthy controls (6.28 [5.89-6.68] vs 2.42 [2.10-2.74] ng/mL, P < .001). SIRT1 concentration in plasma was significantly associated with disease activity (rs  = .317, P < .001). Area under the ROC curve (AUC) analysis showed that compared to healthy controls, SIRT1 had a good ability for diagnosis of SLE (AUC = 0.986, P < .001). Compared with rheumatoid arthritis, osteoarthritis, Sjögren\'s syndrome, ankylosing spondylitis, mixed connective tissue disease and systemic sclerosis patients, the AUC of plasma SIRT1 in SLE patients was 0.982, 0.881, 0.810, 0.860, 0.781, 0.889, 0.736, respectively. When evaluating the discriminative power of SIRT1, the sensitivity and specificity for distinguishing SLE from non-SLE patients were 95.51%, 61.43%, respectively, at the optimal cut-off value of 4.323 ng/mL.
CONCLUSIONS: Circulating SIRT1 was elevated in SLE, and might be a promising SLE diagnostic marker.

Objective: The aim of present study is to analyze clinical and laboratory features of mixed connective tissue disease (MCTD)-associated trigeminal neuropathy (TN). Methods: Clinical records of 12 cases of MCTD complicated with TN diagnosed in Peking University People\'s Hospital from January 2008 to October 2019 were analyzed retrospectively. Results: The present study included 12 cases, 1 males and 11 females, average age was(40±13)years. TN was developed before the diagosis of MCTD in 1 case. TN and MCTD were occurred simutaneously in 1 case. Raynaud phenomenon (12 cases), arthritis (10cases), edema of fingers (9cases), myositis (6 cases), and pulmonary involvement (11cases) were main cinical feature of MCTD-associate TN. Antinuclear antibody (ANA) and high titer anti-U1-RNP antibody could be detected in serum of all patients. Elevated erythrocyte sedimentation rate (ESR) and creatine kinase were found in serum of 7cases and 5cases, respectively. Blink reflex tests were positive in 6 cases. Neurological symptoms improve slowly without any progress by using glucocorticoid combined immunosuppressants or intravenous gamma globulin. Conclusions: TN is often associted with actived MCTD. Positive ANA and anti-U1-RNP antibody were common in MCTD-associated TN. Blink reflex test is essential to diagnose MCTD-associated TN. Intensive treatment of MCTD contributes to control the progress of TN.
目的： 了解合并三叉神经病变（TN）的混合性结缔组织病（MCTD）的临床特征、免疫学改变及预后，提高临床医师对MCTD并发TN的认识。 方法： 回顾性分析2008年1月至2019年10月在北京大学人民医院确诊为MCTD合并TN的12例患者临床资料。 结果： 12例患者中男1例，女11例，年龄（40±13）岁。1例以TN首发，10例以MCTD首发，1例两者同时发病。以雷诺现象（12例）、肺部受累（11例）、关节炎（10例）、手指肿胀（9例）及肌炎（6例）为主要表现。所有患者抗核抗体（ANA）及抗U1-RNP抗体均阳性，且抗U1-RNP抗体高滴度，7例血红细胞沉降率均不同程度升高，5例肌酶升高。9例行瞬目反射试验，其中6例阳性。糖皮质激素联合免疫抑制剂或联合静脉注射人免疫球蛋白治疗原发病为主，神经系统症状改善缓慢，但无进展。 结论： MCTD病情活动时出现TN，伴有TN的MCTD中ANA和抗U1-RNP抗体阳性率高，瞬目反射试验对明确诊断MCTD合并TN十分重要，治疗原发病有助于控制疾病的进展。.

Mixed connective tissue disease (MCTD) is a chronic autoimmune disease, which has a broad range of clinical manifestations shared by systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis. MCTD is featured with high serum titers of anti-ribonucleoprotein antibodies and multiple system involvement. Its spinal cord involvement mainly manifests as transverse myelopathy (TM) and longitudinal extensive transverse myelopathy (LETM). Myelopathy in MCTD is extremely rare, and is usually characterized by serious neurological complications, such as paralysis or muscular paresis, sensory impairment, and smooth muscle dysfunction. Progressive clinical manifestations combined with laboratory examinations and magnetic resonance imaging examinations play important roles in the diagnosis of this disease. In order to prevent permanent neurological damage to the spinal cord, plasmapheresis and intravenous immunoglobulin can be performed in patients at the early disease stage. Early high-dose corticosteroids combined with cyclophosphamide, followed by low doses of immunosuppressors, can improve the long-term prognosis of patients. There are only nine global cases reported on MCTD associated with myelopathy at present. The death rate and disability rate of myelopathy in MCTD are extremely high. In this review, the pathomechanisms, clinical manifestations, auxiliary examination, diagnosis, differential diagnosis, treatment, and prognosis of myelopathy in MCTD were systematically elucidated.

BACKGROUND: Phosphaturic mesenchymal tumor mixed connective tissue type (PMT/MCT) is the most common type (up to 90%) of phosphaturic mesenchymal tumor (PMT), a rare clinicopathologic entity. Besides overproduction of fibroblast growth factor 23 (FGF23), there is a big variation of immunohistochemical characteristic across types of PMT, which makes it difficult to obtain an early diagnosis of PMT/MCT. As a benign tumor, PMT/MCT usually happens in subcutaneous tissues and leads to nonhealing of wound. A complete excision of PMT/MCT facilitates wound healing.
CONCLUSIONS: Review of the existing evidence indicates that early diagnosis of PMT/MCT is critically important when treating PMT/MCT wound. Hence standardization of early diagnosis for PMT/MCT is mandated.