OBJECTIVES: To investigate the clinical and immunological features of children with primary immune thrombocytopenia (pITP) or connective tissue disease (CTD) with thrombocytopenia as the initial manifestation at initial diagnosis, and to provide a basis for early differentiation. METHODS: A retrospective study was performed on 236 children with pITP (pITP group) or CTD with thrombocytopenia as the initial manifestation (CTD-TP group) who were admitted from January 2019 to August 2024. Clinical and immunological indicators were compared between the two groups to identify potential influencing factors for early differentiation and their discriminative validity. RESULTS: Compared with the pITP group, the CTD-TP group had a significantly older age of onset and significantly lower leukocyte count, eosinophil count, lymphocyte count, and complement C4 level (P<0.05), as well as significantly higher levels of C-reactive protein, IgE, and IgM (P<0.05). The logistic regression analysis showed that age, IgE, IgM, total B cells, and complement C4 were predictive factors for early differentiation between pITP and CTD-TP (P<0.05). The receiver operating characteristic curve analysis showed that a combination of these five factors had a good discriminative validity, with an area under the curve of 0.944. The correlation analysis showed a negative correlation between IgG and platelet count in the pITP group (r_s=-0.363, P<0.05) and a positive correlation between NK cells and platelet count in the CTD-TP group (r_s=0.713, P<0.05). CONCLUSIONS There is heterogeneity in the clinical and immunological indicators between children with pITP and CTD-TP at initial diagnosis, and these research findings can help with the early differentiation between the two diseases.
Purpura, Thrombocytopenic, Idiopathic/immunology* ; Diagnosis, Differential ; Connective Tissue Diseases/immunology* ; Retrospective Studies ; Early Diagnosis ; Age of Onset ; Leukocyte Count ; Complement C4/immunology* ; C-Reactive Protein/immunology* ; Immunoglobulin E/immunology* ; Immunoglobulin M/immunology* ; Humans ; Male ; Female ; Infant ; Child, Preschool ; Child ; Adolescent ; Biomarkers/blood*

OBJECTIVES: Patients with connective tissue diseases (CTD) have a high incidence of cardiac involvement, which often presents insidiously and can progress rapidly, making it one of the leading causes of death. Multiparametric cardiovascular magnetic resonance (CMR) provides a comprehensive quantitative evaluation of myocardial injury and is emerging as a valuable tool for detecting cardiac involvement in CTD. This study aims to investigate the correlations between CMR features and serological biomarkers in CTD patients, assess their potential clinical value, and further explore the impact of pre-CMR immunotherapy intensity on CMR-specific parameters, thereby evaluating the role of CMR in the early diagnosis of CTD-related cardiac involvement. METHODS: A retrospective analysis was conducted on 72 consecutive CTD patients who underwent CMR at Xiangya Hospital of Central South University between September 2019 and March 2024. Clinical data, serological markers, and CMR parameters were collected. Differences in CMR parameters were compared between CTD patients with positive and negative serological markers. Correlations between serological biomarkers and CMR parameters were analyzed, with subgroup analyses performed for different CTD subtypes. Logistic regression (univariate and multivariate) was applied to explore the effects of pre-CMR immunotherapy intensity on CMR parameters, and receiver operating characteristic (ROC) curve analysis was used to determine cutoff values. RESULTS: In differential analyses, CTD patients with elevated interleukin (IL)-1β and IL-6 levels exhibited significantly higher myocardial T₂ values compared with those with normal levels (P=0.014, P=0.012). Elevated IL-10 was associated with a higher prevalence of microvascular lesions on CMR (P=0.038). Correlation analysis revealed a significant positive association between high-sensitivity cardiac troponin T (hs-cTnT) and T₂ values (r=0.371, P=0.009). ROC analysis indicated that when the hs-cTnT threshold was 0.01 ng/mL, the sensitivity and specificity for predicting elevated left ventricular T₂ values were 85.71% and 61.11%, respectively [area under the curve (AUC)=0.767, P=0.001]. hs-cTnT and creatine kinase (CK) were also positively correlated with native T₁ values (r=0.371, P=0.009; r=0.364, P=0.032). Erythrocyte sedimentation rate (ESR) showed a positive correlation with the percentage of the late gadolinium enhancement (LGE) (r=0.236, P=0.047). Conversely, hs-cTnT correlated negatively with global radial strain (GRS) (r=-0.297, P=0.034), while CK correlated negatively with both GRS and global circumferential strain (GCS) (r=-0.292, P=0.022; r=-0.282, P=0.027). Among patients with elevated hs-cTnT, the cumulative glucocorticoid dose prior to CMR was negatively associated with elevated T₂ values (OR=0.997, P=0.018), and this correlation remained significant after adjusting for duration of steroid use (OR=0.997, P=0.044). ROC analysis showed that when the cumulative glucocorticoid dose did not exceed 613 mg/mL (prednisone equivalent), the sensitivity and specificity for predicting elevated T₂ values were 90.48% and 77.78%, respectively (AUC=0.862, P<0.001). CONCLUSIONS Several inflammatory biomarkers demonstrate correlations with specific CMR parameters, with hs-cTnT showing the strongest associations across multiple indices. Elevated hs-cTnT suggests a high likelihood of cardiac involvement in CTD patients. Furthermore, pre-CMR immunotherapy intensity significantly influences the specificity of T₂ mapping, indicating its importance in interpreting CMR results. These findings provide critical insights for clinicians in the early recognition, timely intervention, and disease evaluation. Future research should further explore the role of CMR in the assessment of CTD-related cardiac assessment of CTD-related cardiac involvement. Future studies should further explore the role of CMR in evaluating CTD cardiac manifestations and its integration with other clinical data to optimize patient management.
Humans ; Retrospective Studies ; Male ; Female ; Connective Tissue Diseases/blood* ; Middle Aged ; Adult ; Biomarkers/blood* ; Magnetic Resonance Imaging/methods* ; ROC Curve ; Interleukin-6/blood* ; Troponin T/blood*

Objective: To analyze the clinical characteristics of children with multisystem inflammatory syndrome (MIS-C) associated with SARS-CoV-2 in China, and to improve the understanding of MIS-C among pediatricians. Methods: Case series study.Collect the clinical characteristics, auxiliary examinations, treatment decisions, and prognosis of 64 patients with MIS-C from 9 hospitals in China from December 2022 to June 2023. Results: Among the 64 MIS-C patients, 36 were boys and 28 were girls, with an onset age being 2.8 (0.3, 14.0) years. All patients suffered from fever, elevated inflammatory indicators, and multiple system involvement. Forty-three patients (67%) were involved in more than 3 systems simultaneously, including skin mucosa 60 cases (94%), blood system 52 cases (89%), circulatory system 54 cases (84%), digestive system 48 cases (75%), and nervous system 24 cases (37%). Common mucocutaneous lesions included rash 54 cases (84%) and conjunctival congestion and (or) lip flushing 45 cases (70%). Hematological abnormalities consisted of coagulation dysfunction 48 cases (75%), thrombocytopenia 9 cases (14%), and lymphopenia 8 cases (13%). Cardiovascular lesions mainly affected cardiac function, of which 11 patients (17%) were accompanied by hypotension or shock, and 7 patients (12%) had coronary artery dilatation.Thirty-six patients (56%) had gastrointestinal symptoms, 23 patients (36%) had neurological symptoms. Forty-five patients (70%) received the initial treatment of intravenous immunoglobulin in combination with glucocorticoids, 5 patients (8%) received the methylprednisolone pulse therapy and 2 patients (3%) treated with biological agents, 7 patients with coronary artery dilation all returned to normal within 6 months. Conclusions: MIS-C patients are mainly characterized by fever, high inflammatory response, and multiple organ damage. The preferred initial treatment is intravenous immunoglobulin combined with glucocorticoids. All patients have a good prognosis.
Male ; Child ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Blood Coagulation ; COVID-19 ; China/epidemiology* ; Connective Tissue Diseases ; Coronary Aneurysm ; Fever ; Systemic Inflammatory Response Syndrome/therapy*

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.
Anesthetics, Local ; Blood Vessels ; Cicatrix ; Collagen ; Connective Tissue Diseases ; Ehlers-Danlos Syndrome ; Foot ; Hematoma ; Hemorrhage ; Humans ; Intestines ; Joint Instability ; Postoperative Care ; Skeleton ; Skin ; Wound Healing ; Wounds and Injuries

Atherosclerosis and its complications are often reported in patients with connective tissue diseases (CTDs) showing chronic inflammation. Traditional cardiovascular risk factors do not account for accelerated atherosclerosis in patients with CTDs. Inflammation, although non-traditional, is considered one of the risk factors for endothelial dysfunction, atrial stiffness, and atherosclerosis. Therefore, it is essential to evaluate other risk factors for cardiovascular disease (CVD) in patients with CTDs. The interest in pulse wave analysis (PWA) is growing because of its predictive value for CVD. The arterial pressure waveform is a composite of an incidental wave produced by a ventricular contraction and a reflected wave. The wave reflection can be quantified using the augmentation index (AIx); it is defined as the difference between the inflection and peak systolic pressure, and expressed as a percentage of the pulse pressure. The PWA is represented by AIx. Risk score systems, such as the Framingham scoring system, were correlated with AIx. Many studies have analyzed the ability of the AIx to predict the CAD severity in the general population. In patients with CTDs, the AIx was found to increase compared to a healthy control group. The AIx was related to the activity of CTDs. The treatment for inflammation appeared to improve the AIx in some CTDs. Although more studies will be needed to obtain conclusive evidence, AIx is expected to be a prognostic factor or a risk factor for CVD in patients with CTDs.
Arterial Pressure ; Atherosclerosis ; Blood Pressure ; Cardiovascular Diseases ; Connective Tissue Diseases* ; Connective Tissue* ; Humans ; Inflammation ; Pulse Wave Analysis ; Risk Factors

Livedo racemosa (LR) is characterized by a striking violaceous netlike patterning of the skin similar to the livedo reticularis, from which it differs by its localization (more generalized), and shape (irregular, broken circular segments). LR is probably caused by patchy impairment of cutaneous arteriolar circulation, resulting in venous dilatation and stasis of blood. LR is always associated with a pathological condition, including hematologic/hypercoagulable disease, vasculitis, connective tissue diseases, neoplasm, lymphoma, infection, cerebrovascular disease, adverse response to a drug, and etc. Hence, clinical, pathological and laboratory examinations are important for excluding these underlying diseases. To date, there have been few reports of LR secondary to posttransplant lymphoproliferative disease (PTLD) in dermatologic literatures. Herein, we report a case of LR associated with Epstein-Barr virus-induced PTLD of cervical lymph node in a 19-year-old female, who had generalized reticular erythematous to violaceous patch on the entire body after allogenic peripheral blood stem cell transplantation.
Connective Tissue Diseases ; Dilatation ; Female ; Herpesvirus 4, Human ; Humans ; Livedo Reticularis ; Lymph Nodes ; Lymphoma ; Lymphoproliferative Disorders ; Peripheral Blood Stem Cell Transplantation ; Skin ; Strikes, Employee ; Vasculitis

Although autoantibodies are routinely screened in patients with idiopathic interstitial pneumonia, there are no reliable data on their clinical usefulness. The aim of this study was to investigate the prognostic value of autoantibodies for predicting the development of new connective tissue disease in these patients and also mortality. We conducted retrospective analysis of the baseline, and follow-up data for 688 patients with idiopathic interstitial pneumonia (526 with idiopathic pulmonary fibrosis, 85 with nonspecific interstitial pneumonia, and 77 with cryptogenic organizing pneumonia) at one single tertiary referral center. The median follow-up period was 33.6 months. Antinuclear antibody was positive in 34.5% of all subjects, rheumatoid factor in 13.2%, and other specific autoantibodies were positive between 0.7%-6.8% of the cases. No significant difference in patient survival was found between the autoantibody-positive and -negative groups. However, the presence of autoantibodies, especially antinuclear antibody with a titer higher than 1:320, was a significant predictor for the future development of new connective tissue diseases (relative risk, 6.4), although the incidence was low (3.8% of all subjects during follow-up). In conclusion, autoantibodies are significant predictors for new connective tissue disease development, although they have no prognostic value.
Aged ; Antibodies, Antinuclear/blood ; Autoantibodies/*blood ; Cohort Studies ; Connective Tissue Diseases/pathology ; Female ; Follow-Up Studies ; Humans ; Idiopathic Interstitial Pneumonias/*blood/diagnosis ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Rheumatoid Factor/blood ; Risk Factors ; Tertiary Care Centers ; Tomography, X-Ray Computed

Necrobiosis lipoidica (NL) is a dermatologic disorder characterized by sharply demarcated yellow-brown plaques or indurated plaques with violaceous, irregular borders usually located on the pretibial region. Lesions may flatten and develop a central yellow or orange area as it becomes atrophic, often accompanied by the appearance of telangiectasias, giving it the characteristic "glazed¬porcelain" sheen. Typical histologic findings are seen at the edge of the lesions and include: variable amount of dermal fibrosis, particularly in its lower two-thirds of the dermis; and a superficial and deep perivascular inflammatory reaction. The dermal changes extend to the underlying subcutaneous septa. Although these histologic findings were first described in diabetic patients, not all patients with NL have concurrent diabetes. Treatment of NL involves the use of potent topical steroids and the prevention of the development of ulcers. This is a case of a 29-year-old female who presented with few erythematous plaques on the left anterolateral portion of the left lower extremity, histologically confirmed as NL with normal fasting blood sugar levels.

Human ; Female ; Adult ; Atrophy ; Blood Glucose ; Citrus Sinensis ; Connective Tissue Diseases ; Dermis ; Diabetes Mellitus ; Necrobiosis Lipoidica ; Skin Diseases ; Telangiectasis ; Ulcer

Eosinophic fasciitis (EF) is an uncommon connective tissue disease characterized by scleroderma-like cutaneous changes, peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR). Typical histopathologic findings include chronic inflammatory infiltration affecting the deep fascia with lymphocytes, histiocytes, and occasionally eosinophils. We report two cases of EF, the first of which is a 36-year-old man with a tender brownish induration on both forearms, for 2 months. Histopathologic examination showed fibrotic fascia with a mixed inflammatory cell infiltration. The second case is a 52-year-old woman with a symmetrical painful swelling and skin induration on both forearms, for 4 months. A deep biopsy demonstrated chronic inflammatory cell infiltration and hyaline degeneration in the fascia. Increased signal intensity in the fascia and tendon sheath was shown on magnetic resonance imaging. In laboratory examination, mild eosinophilia was found in both cases. Both patients had a history of physical activity (weight training and excessive housework, respectively) and showed marked improvement with high doses of oral prednisolone for several months.
Adult ; Biopsy ; Blood Sedimentation ; Connective Tissue Diseases ; Eosinophilia ; Eosinophils ; Fascia ; Fasciitis ; Female ; Forearm ; Histiocytes ; Housekeeping ; Humans ; Hyalin ; Hypergammaglobulinemia ; Lymphocytes ; Magnetic Resonance Imaging ; Middle Aged ; Motor Activity ; Prednisolone ; Skin ; Tendons

OBJECTIVETo investigate the prevalence of anti-endothelial cell antibodies (AECAs) in the sera of connective tissue diseases (CTD) patients with pulmonary arterial hypertension (PAH) and its correlation with clinical manifestations.

METHODSAECAs in sera of 39 CTD patients with PAH, 22 CTD patients without PAH, and 10 healthy donors as controls were detected with Western blotting. The prevalence of different AECAs in different groups was compared and its correlation with clinical manifestations was also investigated.

RESULTSThe prevalence of AECAs was 82.1% in CTD patients with PAH, 72.7% in CTD patients without PAH, and 20.0% in healthy donors. Anti-22 kD AECA was only detected in CTD patients with PAH (15.4%). Anti-75 kD AECA was more frequently detected in CTD patients with PAH than in those without PAH (51.3% vs. 22.7%, P < 0.05). In CTD patients with PAH, anti-75 kD AECA was more frequently detected in those with Raynaud's phenomenon or with positive anti-RNP antibody.

CONCLUSIONAECAs could be frequently detected in CTD patients with or without PAH, while anti-22 kD and anti-75 kD AECA might be specific in CTD patients with PAH.

Adult ; Autoantibodies ; blood ; immunology ; Cell Line ; Connective Tissue Diseases ; blood ; immunology ; pathology ; physiopathology ; Endothelial Cells ; cytology ; immunology ; Female ; Humans ; Hypertension, Pulmonary ; blood ; immunology ; pathology ; physiopathology ; Middle Aged