Profile of Autoantibodies and Clinical Symptoms in Guinean Patients with Connective Tissue Diseases

Connective tissue diseases (CTDs) are Autoimmune diseases (AIDs) characterized by the appearance of autoantibodies, which are diagnostic markers. Investigations of these autoantibodies play a major role in the management of several autoimmune diseases. The objective of this study was to describe the profile of anti-ENA antibodies according to the clinical symptoms of mixed CTDs in Conakry teaching Hospital. We performed a cross-sectional study during six months. A total of 20 patients was recruited and we measured antibodies using the ELISA technique. The mean age of our patients was 36.5 years, with a predominance of females. Cutaneous and rheumatological signs were the main clinical manifestations. SLP was the most frequent CTDs;the threshold of ENA antibodies positivity was higher in scleroderma with and SLP. Anti-ENA identification reveals the frequency of anti-SSA (83.33%), anti-U1RNP (66.66%) and anti-histone (50%) antibodies. Antinuclear antibodies (ANA) react with various components of the cell nucleus. Their detection is of major interest in the diagnosis of CTDs. Our results highlight the importance of determining the specificity of these antibodies to guide differential diagnosis.

Connective tissue diseases in primary biliary cirrhosis:A population-based cohort study

AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.

Prevalence of Anti-endothelial Cell Antibodies in Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Objective To 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. Methods AECAs 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. Results The 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. Conclusion AECAs 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.

The Differences of Interstitial Lung Diseases in High-Resolution Computerized Tomography and Pulmonary Function Test among Different Connective Tissue Diseases, and the Correlated Factors

Objective. To study the difference of interstitial lung diseases (ILDs) in high-resolution computerized tomography and pulmonary function test among different connective tissue diseases (CTDs). Methods. 209 patients with different CTDs were recruited and underwent lung HRCT and PFT. Eerythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum ferritin (SF), anti-SSA, and so on were tested. Based on HRCT, a patient was classified into ILD group (CTD+ILD) or non-ILD group (CTD-ILD). HRCT, PFT, and laboratory markers were compared according to CTDs and CTD-associated ILDs. Results. The incidences of ILD were 79.6%, 82.0%, 89.7%, and 97.1% respectively for Rheumatoid arthritis (RA), primary Sjogren’s symptom (pSS), dermatomyositis/polymyositis (DM/PM), and systemic sclerosis (SSc) groups. RA and pSS patients exhibited more nodules, patching, ground-glass opacity, and cord shadow foci in HRCT, DM/PM and SSc patients exhibited more reticular opacity and honeycombing foci. RA and pSS patients exhibited more obstructive ventilatory disorder, small airway dysfunction and emphysema in PFT, and DM/PM and SSc patients exhibited more restrictive ventilatory disorder, mixed ventilatory disorder. ESR, CRP and SF were significantly higher in total CTD+ILD group than in total CTD-ILD group (P = 0.047, 0.006, 0.004, respectively), and higher in different CTD+ ILD groups than in comparable CTD-ILD groups (P = 0.049, 0.048, and 0.023, pSS+ILD, SSc+ILD and RA+ILD compared to pSS-ILD, SSc-ILD and RA-ILD, respectively for ESR, CRP, SF). The positive rate of anti-SSA was significantly higher in DM/PM+ILD group than in DM/PM-ILD group (P = 0.025). Conclusions. The manifestations and incidences of ILDs differ among different CTDs in HRCT and PFT, and inflammation and anti-SSA are positively correlated with ILDs in different CTDs, which provide important evidences for judging disease condition and prognosis.

The Effects of Sex on Patient Reported Outcomes in Inflammatory Arthritis and Connective Tissue Diseases

Background: It was thought that women report higher pain than men. We studied if there was a sex difference for several patient reported outcomes (PROs) in rheumatic diseases. Materials and Methods: Health Assessment Questionnaire disability index (HAQ-DI) as well as 100 mm Visual Analogue Scale (VAS) for pain, fatigue, sleep disturbance, and patient global assessment were compared cross-sectionally between the sexes for ankylosingspondylitis (AS), psoriatic arthritis (PsA), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Data were collected using standardized forms administered during routine care. Results: The sample included 136 patients (97 males) with AS, 200 (83 males) with PsA, 232 (40 males) with RA, 199 (12 males) with SLE, and 113 (17 males) with SSc. There were no significant differences in AS. There were sex differences in PsA for HAQ (0.85 females, 0.57 males;p s, 36.8 males;p s, 31.6 males;p s, 36.0 males;p s, 33.1 males;p whereas, in SSc, men had a higher global assessment (52.9 males, 38.1 females;p Conclusions: A significant sex difference was observed in PsA with females reporting worse symptoms. In SSc, global assessments were worse in males possibly due to proportionately more diffuse cutaneous SSc. Sex differences for PROs are not consistent between rheumatic inflammatory diseases in prevalent patients.

Interstitial Lung Diseases Associated with Connective Tissue Pathologies: Radiologic Features

Introduction: The high resolution computed tomography (HRCT) is an important part in the diagnostic approach of interstitial lung disease (ILD) associated with connective tissue diseases (CTD) by providing detailed information on the elementary lesion and the radiological pattern of ILD. Aim: to point out the role of HRCT in the diagnosis of ILD associated with CTD (ILD-CTD). Methods: A Retrospective descriptive study was conducted between 2008 and 2017. Data of 24 patients presenting ILD-CTD were collected. A review of HRCT was performed by a radiologist without knowledge of the CTD. Results: Predominant elementary lesion of ILD associated with dermatomyositis (9 cases) was ground glass opacity (n = 9) followed by consolidation (n = 6). Non Specific Interstitial Pneumonia (NSIP) was the most reported pattern (5 cases). Ground glass opacity was also the predominant elementary lesion for the 2 cases of scleroderma and in Sj&#246gren’s syndrome (4 cases/5). NSIP was the predominant radiological presentation in these two CTD. Lymphoid interstitial pneumonia revealed Sj&#246gren’s syndrome in one case. In rheumatoid arthritis (6 cases), the elementary HRCT lesions were irregular interlobular septal thickening (n = 4) and honeycombing (n = 4) consistent with Usual Interstitial Pneumonia (UIP) in 2 cases. Similarly UIP has been described for the 2 patients with lupus and mixed connective tissue disease. Conclusion: HRCT plays an important role in the management of ILD-CTD. Description of the HRCT elementary lesions and the radiological pattern of ILD can be helpful for CTD’s diagnosis.

Undifferentiated connective tissue diseases-related hepatic injury

Hepatic injury is rarely associated with undifferentiated connective tissue diseases (UCTD). We report, here, a case of a middle-aged woman with UCTD-related hepatic injury, including its case history, clinical manifestations, laboratory findings, treatment and its short-term effect. The patient was admitted to the hospital with symptoms of fatigue, anorexia, low-grade fever and skin rashes. She had a past history of left knee joint replacement. Laboratory tests showed elevated levels of serum transaminase, IgG and globulin, accelerated erythrocyte sedimentation rate, eosinophilia and a high titer of antinuclear antibodies (1:320). Imaging studies showed interstitial pneumonitis and hydropericardium. Liver biopsy showed the features which were consistent with those of connective tissue diseases-related polyangitis. After treatment with a low-dose of oral prednisone, both symptoms and laboratory findings were significantly improved. UCTD-related hepatic injury should be considered in the differential diagnosis of connective tissue diseases with abnormal liver function tests. Low- dose prednisone may effectively improve both symptoms and laboratory tests.

Connective Tissue Diseases on the Elderly Population in Dakar Hospital Setting

Introduction: Immunosenescence contributes to the development of auto-antibodies. However, while the prevalence of some autoantibodies increases with age, the incidence of connective tissue diseases decreases with age. This study aims to determine the clinical and paraclinical characteristics of connective tissue diseases in the elderly population. Materials and Methods: We conducted a retrospective and descriptive study, from March 2010 to March 2021, in the Internal Medicine Department of Aristide Le Dantec Hospital, including all the medical records of patients followed up for connective tissue disease and whose symptomatology began at an age greater than or equal to 65 years. Epidemiological, clinical, paraclinical, therapeutic and evolutionary data were collected from a pre-established survey form and then processed using SPSS software. Results: Overall, 22 cases were collected out of 275 seen on consultation. They involved 16 women and 6 men, aged between 65 and 85 years with a median of 70 years. The mean duration of the clinical picture was 15.7 months with extremes of 1 and 96 months. The clinical symptoms were joint pain in 21 patients associated with dry eye and mouth syndrome in seven cases and one patient underwent isolated dry eye and mouth syndrome. Joint involvement was deforming in 13 patients. Poor general condition was noted in 20 patients. Biology showed anemia in 14 patients, hyperleukocytosis (2 patients) and thrombocytosis (5 patients). The sedimentation rate (SR) was accelerated in 9 patients and the C-reactive protein (CRP) was positive in 12 patients. Immunology showed a positive Latex test (3 patients/6), Waaler-Rose reaction (8 patients/13), anti-cyclic citrullinated peptides (anti-CCP) antibodies (11 patients/11), anti-nuclear antibodies (2 patients/4). Anti-extractable nuclear antigens (anti-ENA) antibody testing in 4 patients showed positive anti-U1RNP (1 case), anti-SSA and anti-SSB (1 case). Plain X-Rays of the hands, wrists and feet showed destructive lesions in 16 patients. The diagnosis of rheumatoid arthritis (RA) was set in 21 patients, one of which was associated with Sjogren’s syndrome (SS) and one with primary Sjogren’s syndrome. Treatment was based on prednisone (21 cases), methotrexate (17 patients), and hydroxychloroquine (9 patients). The evolution was remarkable in 10 patients. Conclusion: Autoimmune diseases in the elderly are uncommon compared to the young adult population. In our study, we only found cases of rheumatoid arthritis and Sjogren’s syndrome. As the population ages, connective tissue diseases may be increasingly diagnosed.

COVID-19 Induced Mixed Connective Tissue Disease (MCTD)—Case Report

Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder that damages multiple organs simultaneously and is associated with chronic inflammation, in which the signs of systemic sclerosis, systemic lupus erythematosus, and polymyositis can co-occur. Its distinctive feature and the basis for the diagnosis of MCTD is the presence of an antibody against the uridine-rich RNP of the cell nucleus (Anti-U1-RNP). It has been observed that intercurrent infections can trigger autoimmune diseases, however, the fact that viral infections—such as SARS-CoV-2—induce them is currently poorly understood. The present study raises the etiological role of the SARS-CoV-2 virus in the development of the disease. Authors describe the case of a 38-year-old patient in good general condition, who was diagnosed with mixed connective tissue disease three months after COVID-19 infection.

Hydroxychloroquine-induced renal phospholipidosis resembling Fabry disease in undifferentiated connective tissue disease: A case report

BACKGROUND Fabry disease is a kind of lysosomal storage disease resulting from deficient activity of the lysosomal hydrolase alpha-galactosidase A(GLA). A mutation in the GLA gene leads to a loss of activity of alpha-galactosidase A. Some drugs,such as hydroxychloroquine, can cause pathological changes similar to those usually seen in Fabry disease.CASE SUMMARY We report the case of a 41-year-old female patient who was diagnosed with undifferentiated connective tissue disease in 2008. Hydroxychloroquine treatment started 2 years ago, and proteinuria and hematuria increased. Renal biopsy demonstrated renal phospholipidosis. Zebra bodies and myelin figures were found by renal electron microscopy and were initially thought to be indicators of Fabry disease. A genetic analysis of the patient and her family members did not reveal mutations in the GLA gene, supporting a diagnosis of hydroxychloroquine-induced renal phospholipidosis.CONCLUSION This report reveals one of the adverse effects of hydroxychloroquine. We should pay more attention to hydroxychloroquine-induced renal phospholipidosis.

Mixed Connective Tissue Disease Complicated by Retinal Microvasculopathy and Its Relationship with Fragile-X Syndrome

Purpose: To report an unusual case of retinal microvasculopathy secondary to mixed connective tissue disease (MCTD) on a background history of fragile-X syndrome (FXS). Methods: Case report and literature review. Results: A cotton wool spot was discovered in a 29-year-old female who presented with an ischaemic digit secondary to Raynaud’s phenomenon. She also has a background history of MCTD and FXS. Fundus examination and automated perimetry findings were normal. Magnet resonance imaging and computed tomography aortogram did not demonstrate any evidence of vasculitis in the head and neck. She was tested positively for U1-ribonuclear peptide. Interestingly, the re-distribution of Fragile-X related gene 1 has been suggested to trigger autoimmune responses in experiments. This finding makes the case peculiar as it suggests an alternate explanation for this patient’s clinical findings. Conclusion: Retinal vasculopathy is a rare complication of MCTD. The background history of FXS potentially highlights an alternate autoimmune pathogenetic mechanism.

Risk of connective—tissue disease in men with testicular or penile prostheses：a preliminary study

AIM: To help clarifying the possibility of connective-tissue diseases in men with penile or testicular prostheses. METHODS: Eight patients underwent inflatable penile prostheses and 15, testicular prostheses consented to the study. Their medical records were reviewed and a follow-up interview and physical and serological examinations were performed. RESULTS: In patients with penile prostheses, there was no abnormal antinuclear antibody (ANA) or IgM elevation. The serum levels of the rheumatoid factor (RF), C4, IgA and IgG were abnormal in one patient, and the levels of erythrocyte sedimentation rate (ESR) and C3, abnormal in two. Four had elevated IgE. In patients with testicular prostheses, there was no abnormal RF, ANA or IgM. The serum levels of ESR and IgA were abnormal in two, and three had abnormal C4, ten abnormal C3, and eleven decreased IgG. All had increased IgE. Men with penile prostheses had higher serum levels of IgG and IgM than those with testicular prostheses (P=0.001, P=0.016, respectively). The rates of abnormal values of IgE and IgG were higher in men with testicular prostheses than in men with penile prostheses (P=0.008, P=0.009, respectively). Physical examination was normal in all patients and nobody had documented symptoms pertinent to connective-tissue diseases. CONCLUSION: Our findings suggest that the risk of connective-tissue diseases is not higher in patients wearing prostheses as the ANA is negative and there is no apparent manifestation suggestive of connective-tissue diseases.

Immunosuppressive Treatment of Connective Tissue Disease and Occurrence of Tuberculosis

Introduction: The occurrence of tuberculosis (TB) during the follow-up of Connective tissue diseases (CTD) is a particular situation often posing real diagnostic problems. This is an association described in the literature. Patients and methods: We conducted a retrospective and descriptive study at the internal medicine department of Aristide Le Dantec Hospital. The purpose of this study was to determine the treatment that patients followed for CTD and suffering from TB took before the occurrence of this one. Results: During a study period of 11 years and 6 months, 21 cases of TB were diagnosed in 602 cases of CTD (0.03%). The predominance was female with a sex ratio (H/F) of 0.6. The median age was 42 years old. The majority of cases originated from the Dakar region (13 patients or 61.9%) and 85.7% had previous follow-up exclusively in modern medicine and 21 cases (95%) received the CTD’s treatment. This consisted of prednisone (90.5%) combined with methotrexate (52.4%), azathioprine (23.8%) or cyclophosphamide (19.4%). The respective medians doses of these drugs were 12.5 mg per day for prednisone, 13.5 mg per week for methotrexate and 100 mg per day for azathioprine. The median duration of patient follow-up was 36 months. The cumulative dose of prednisone during this period was 23.6 g and that of methotrexate 2.25 g. CTD were dominated by rheumatoid arthritis (RA) (57.1%), and systemic lupus (19%). Isolated cases of systemic scleroderma, primary Sj&#244;gren, SHARP syndrom, mixed connective tissue disease, and multiple autoimmune syndrom were noted. TB was localized in 95% of cases, readily bilateral and poorly disseminated. The respective medians diagnostic delays for systemic disease and TB were 21 months and 5 months. Tuberculin intradermal reaction was performed in 16 cases and was positive in 9 cases, sputum bacilli was performed in 19 cases and was positive in 15 cases. Conclusion: The association of TB and CTD was characterized by its rarity, its poorly disseminated character and its frequency on RA field.

Trimethoprim-Sulfamethoxazole-Induced Hepatitis in Mixed Connective Tissue Disease

Trimethoprim-Sulfamethoxazole (TMP-SMZ) is associated with severe hepatic toxicity or liver failure. We present a case of severe hepatic toxicity for whom TMP-SMZ was prescribed as part of treatment for mixed connective tissue disease (MCTD). TMP-SMZ was used to prevent complications from steroid therapy, but fever and hepatic toxicity developed with repeated TMP-SMZ medication. While the drug lymphocyte stimulation test (DLST) for TMP-SMZ showed negative, the genotype for N-acetyltransferase 2 (NAT2) showed type *6/*7, which is the slow acetylating type for NAT2 activity. This finding for NAT2 genotype and the patient’s clinical history lead us to speculate that her fever and hepatic toxicity were caused by TMP-SMZ.

Clinical significance of connective tissue growth factor in hepatitis B virus-induced hepatic fibrosis

AIM:To determine the utility of connective tissue growth factor(CCN2/CTGF) for assessing hepatic fibrosis in hepatitis B virus(HBV)-induced chronic liver diseases(CLD-B).METHODS:Enzyme-linked immunosorbent assay was used to measure CCN2 in sera from 107 patients with chronic hepatitis B(CHB) and 39 patients with HBVinduced active liver cirrhosis and 30 healthy individuals.Liver samples from 31 patients with CHB,8 patients with HBV-induced liver cirrhosis and 8 HBV carriers with normal liver histology were examined for transforming growth factor β-1(TGF-β1) or CCN2 mRNA levels by in situ hybridization,and computer image analysis was performed to measure integrated optimal density(IOD) of CCN2 mRNA-positive cells in liver tissues.Histological inflammation grading and fibrosis staging were evaluated by H and E staining and Van Gieson's method.RESULTS:Serum CCN2 concentrations were,respectively,4.0-or 4.9-fold higher in patients with CHB or active liver cirrhosis as compared to healthy individuals(P < 0.01).There was good consistency between the levels of CCN2 in sera and CCN2 mRNA expression in liver tissues(r = 0.87,P < 0.01).The levels of CCN2 in sera were increased with the enhancement of histological fibrosis staging in patients with CLD-B(r = 0.85,P < 0.01).Serum CCN2 was a reliable marker for the assessment of liver fibrosis,with areas under the receiver operating characteristic(ROC) curves(AUC) of 0.94 or 0.85 for,respectively,distinguishing normal liver controls from patients with F1 stage liver fibrosis or discriminating between mild and significant fibrosis.CONCLUSION:Detection of serum CCN2 in patients with CLD-B may have clinical significance for assessment of severity of hepatic fibrosis.

CTD-ILD合并肺部感染患者肺部微生物菌群特点及对肺纤维化的影响

目的探讨结缔组织病相关间质性肺疾病(CTD-ILD)合并肺部感染患者肺部微生物菌群特点及对肺纤维化的影响。方法选取2021年2月至2022年5月在医院治疗的45例CTD-ILD合并肺部感染患者作为观察组,同时选取45例CTD-ILD未合并肺部感染患者作为对照组,比较两组肺功能、高分辨CT(HRCT)肺纤维化评分,同时分析观察组病原菌分布情况。结果观察组用力肺活量占预计值百分比(FVC%pred)、第1秒用力呼气容积占预计值百分比(FEV 1%pred)、一氧化碳弥散量占预计值百分比(DLCO%pred)、用力肺活量(FVC)、最大呼气流量(MEF)和残气容积(RV)低于对照组(P<0.05);观察组HRCT影像学斑片状、支气管扩张比率高于对照组(P<0.05),CT纤维化评分高于对照组(P<0.05)。观察组病原菌分布:细菌分布中以肺炎克雷伯菌、铜绿假单胞菌为主,分别占24.44%和15.56%,病毒检出巨细胞病毒、人类疱疹病毒,分别占11.11%和6.67%,真菌检出白念珠菌,占11.11%。观察组年龄≥60岁患者CT纤维化评分高于年龄<60岁患者(P<0.05)。结论CTD-ILD合并肺部感染患者以细菌感染为主;相比较未合并肺部感染患者,合并肺部感染患者肺纤维化较重。

MS19通过抑制IRF5表达调控巨噬细胞极化减轻CTD-ILD肺部炎症的作用研究

目的 探讨MS19通过靶向干扰素调节因子5(IRF5)对结缔组织疾病相关肺间质病变(CTD-ILD)小鼠模型肺部炎症的治疗作用及其相关机制。方法 动物实验:构建CTD-ILD小鼠模型,予以MS19干预,研究MS19对CTD-ILD小鼠肺部炎症的影响。细胞实验:对RAW264.7细胞进行OE-IRF5转染,然后予以MS19干预,研究MS19对IRF5调控的巨噬细胞M1型极化及炎症反应的影响。结果 动物实验:CTD-ILD小鼠出现明显的肺部炎症,小鼠支气管肺泡灌洗液(BALF)中IRF5的表达增高、巨噬细胞M1型极化增加及促炎因子(TNF-α、IL-6和IL-1β)的表达升高;而MS19干预后,CTD-ILD小鼠的肺部炎症减轻,BALF中IRF5表达降低、巨噬细胞M1型极化减少及促炎因子表达下降。细胞实验:脂多糖诱导巨噬细胞M1型极化、促炎因子表达增加;转染OE-IRF5后,巨噬细胞M1型极化增加、促炎因子表达增加;MS19干预后,巨噬细胞M1型极化减少、促炎因子表达减少。结论 MS19通过靶向抑制IRF5调控巨噬细胞极化及炎症反应,从而改善CTD-ILD的肺部炎症,为防治CTD-ILD提供潜在靶点和候选药物。

Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases

Background:Pulmonary arterial hypertension(PAH)associated with connective tissue diseases(CTD)(CTD-PAH)remains a difficult challenge in clinical practice.We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD-PAH.Methods:The data of 53 patients with severe CTD-PAH hospitalized at the Department of Rheumatology and Immunology,The Affiliated Drum Tower Hospital of Nanjing University Medical School,were retrospectively reviewed.Patients were followed up for an average of 2 years to track their outcomes.The efficacy of treatment and the survival rate of patients with severe CTD-PAH were determined.Results:Among the causes of severe CTD-PAH,systemic lupus erythematosus(SLE)was the most common(39.6%),and the age at onset in patients with SLE-PAH was younger than that of patients with other CTD.Bosentan was more effective than sildenafil in reducing pulmonary artery pressure,improving cardiac function,and increasing survival time.Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD-PAH compared with monotherapy.Conclusions:Patients with severe CTD-PAH should be treated early with targeted vasodilators.In this study,bosentan was superior to sildenafil.Combined treatment might be an option for severe CTD-PAH.

Prevalence of Depression and Anxiety in Patients with Autoimmune Disease: A Comparative Study with a Diabetic Population

Introduction: Systemic diseases are a variety of heterogeneous autoimmune and/or autoinflammatory diseases and syndromes usually affecting multiple systems and resulting from immune system dysregulation. We evaluated risk factors for depression and anxiety in an autoimmune disease cohort compared with diabetic patients. Patients and Methods: We conducted an observational, cross-sectional, case-control survey comparing two groups: individuals with connective tissue disease (CTD) and diabetic controls who were followed within three Dakar University hospitals during the period from April to June 2023. Results: The sample comprised 106 participants, of whom 51 (48%) had CTD and 55 (52%) served as diabetic controls. In the CTD group, the majority had lupus (19) and rheumatoid arthritis (23). The CTD patients had a mean age of 41.0 years (SD 16.9), while the diabetic patients had a mean age of 55.9 years (SD 11.7), with a significant difference observed (p Conclusion: Compared to a chronic disease, devastating in Africa and evolving over the long term, autoimmune diseases are more strongly and more frequently associated with anxiety and depression. This is a factor to be taken into account in the holistic management of these patients.

CT肺容积参数评估CTD-ILD肺功能、病情严重程度的价值分析

目的 分析CT肺容积参数评估结缔组织病相关间质性肺疾病(CTD-ILD)患者肺功能、病情严重程度的价值。方法 选取2020年4月—2022年12月收治的126例CTD-ILD,根据肺间质病变临床严重程度分级分为Ⅰ级组(43例)、Ⅱ级组(45例)、Ⅲ级组(38例),均接受肺功能检查,采用高分辨率CT检查获取CT肺容积参数[正常肺组织容积(NL)、间质纤维化肺容积(ILDV)、全肺总容积(WL)、正常肺组织容积百分比(NL%)、间质纤维化肺容积百分比(ILDV%)],比较各组肺功能指标[肺总量(TLC)、第1秒用力呼气容积(FEV1)、用力肺活量(FVC)、残气量(RV)、第1秒用力呼气容积占预计值百分比(FEV1%)]、GAP等级评分、图像纤维化评分及CT肺容积参数,分析CT肺容积参数与肺功能指标、GAP等级评分、图像纤维化评分的关系,以CT肺容积参数对CTD-ILDⅢ级进行诊断,并分析其与肺功能指标联合检测对CTD-ILDⅢ级的诊断效能。结果 FVC、FEV1、TLC、RV、FEV1%Ⅰ级组>Ⅱ级组>Ⅲ级组,GAP等级评分、国际纤维化评分Ⅰ级组<Ⅱ级组<Ⅲ级组(P<0.05);NL、WL、NL%Ⅰ级组>Ⅱ级组>Ⅲ级组,ILDV、ILDV%Ⅰ级组<Ⅱ级组<Ⅲ级组(P<0.05);NL、WL、NL%与FVC、FEV1、TLC、RV、FEV1%呈正相关,与GAP等级评分、图像纤维化评分呈负相关(P<0.05);ILDV、ILDV%与GAP等级评分、图像纤维化评分呈正相关,与FVC、FEV1、TLC、RV、FEV1%呈负相关(P<0.05);NL、WL、ILDV、NL%、ILDV%诊断CTD-ILDⅢ级的受试者工作特征曲线下面积(AUC)分别为0.765、0.715、0.754、0.789、0.768,NL、WL、ILDV、NL%、ILDV%联合诊断的AUC最大,为0.944。结论 CTD-ILD患者NL、WL、ILDV、NL%、ILDV%与肺功能联系密切,且联合检测对患者病情严重程度有较高的诊断效能。