Connective Tissue Disease Research Articles

578VP Recurrent rhabdomyolysis as the presenting feature of mixed connective tissue disease

578VP Recurrent rhabdomyolysis as the presenting feature of mixed connective tissue disease

A case report on Sjögren Syndrome: it‘s more than just autoimmune epithelitis

Background. Primary Sjögren’s syndrome (pSS) is an autoimmune epithelitis associated with various pulmonary manifestations, including PAH (pulmonary arterial hypertension), a severe complication often found in collagen tissue disorders. Case presentation. A 26-year-old female having chest pain and exertional dyspnea diagnosed with severe PAH associated with pSS is presented in this case study. Clinical, laboratory, and imaging findings supported the diagnosis, and treatment involved immunosuppressive therapy and standard PAH medications. Discussion and conclusion. The report highlights the complexities of diagnosing and treating PAH associated with pSS, emphasizing the importance of early intervention for improved long-term outcomes. The prognosis for PAH in connective tissue diseases, encompassing pSS, remains challenging, underscoring the need for accurate diagnosis and timely management with immunosuppressants and PAH-specific therapies.

The ultrasound characteristics of the cardiovascular system in systemic lupus erythematosus in children

Background. Systemic lupus erythematosus (SLE) is one of the most typical diffuse connective tissue diseases characterized by the involvement of many organs and systems in the pathological process. Cardiac involvement in SLE is one of the most common manifestations of visceritis in both adults and children. According to various authors, the frequency of heart involvement ranges widely from 40% to 90–95%, with changes being detected in 100% of patients during autopsy. Establishing an accurate diagnosis of cardiovascular system changes in SLE is challenging yet crucial for assessing disease activity and prognosis. This has become possible with the clinical application of modern objective functional methods of examination such as echocardiography and Doppler, which have significantly increased the detection rate of even subclinical forms of heart involvement. Purpose – of the study is to examine the structural and functional state of the heart in children with systemic lupus erythematosus using echocardiography. Materials and Methods. We analyzed echocardiography and Doppler data of 32 children aged from 11 to 18 years old with systemic lupus erythematosus who were examined and treated at the Institute for the Protection of Children’s and Adolescents’ Health of the National Academy of Medical Sciences of Ukraine. There were 7 boys (21.87%) and 25 girls (78.13%) among the patients. The diagnosis was confirmed through comprehensive clinical, laboratory, and instrumental examinations. Ultrasound scanning of the heart and major vessels was performed using «Siemens SL-1» and «Logic-400» General Electric pulse-wave devices with color mapping. Results. The study revealed that the most frequent signs of systemic connective tissue dysplasia diagnosed by echocardiography were the presence of abnormal chords in the left ventricle (in 24 patients – 75%) and mitral valve prolapse (in 12 patients – 37.50%). A typical sign of the disease was lupus carditis manifested by the reduction in the left ventricular diameter systolic contraction (22 patients – 68.75%) and thickening of the left ventricular myocardium and the thickness of the interventricular membrane in 15 patients (46.88%). Left ventricular contractile function was reduced (EF < 60%) in 15 patients (46.88%). In cases with a disease duration of up to two years and grade I activity of the pathological process, an increase in contractile function was observed in 3 patients (9.38%). Grade I/II mitral insufficiency was present in 2 patients (6.25%). Grade I aortic insufficiency was found in 1 patient (3.13%). Characteristic changes in the right ventricle were also observed in patients with systemic lupus erythematosus. The most common findings included enlargement of the right ventricular cavity and the outlet tract of the right ventricle (in 15 patients – 46.88%), hyperkinesia (in 3 patients – 9,38%) and myocardial thickening. Accumulation of fluid in the pericardial cavity during echocardiography was observed in 9 patients (28.13%) as a sign of polyserositis. The amount of liquid was small. Conclusions. The study established the polymorphism of pathological changes in the cardiovascular system of patients with systemic lupus erythematosus, which dictates the necessity of conducting echocardiography and Doppler both at the initial examination and during monitoring of the pathological process and treatment quality. The high frequency of diagnosed abnormal chords may indicate a congenital predisposition of connective tissue in patients with systemic lupus erythematosus to dysplasia. Echocardiography is especially valuable for identifying valve and pericardial lesions.

Evaluation of Progression From Preclinical to Systemic Autoimmune Rheumatic Disease: Novel Use of the European Alliance of Associations for Rheumatology/American College of Rheumatology Systemic Lupus Erythematosus Classification Criteria as an Outcome Measure.

Our objective was to evaluate the development of a systemic autoimmune rheumatic disease (SARD) in undifferentiated and asymptomatic individuals with antinuclear antibodies (ANAs). We comparatively evaluated those who did and did not develop a SARD and fulfillment of classification criteria. We conducted a cohort study of undifferentiated and asymptomatic patients with ANAs who were assessed for the development of a SARD. The primary outcome was a diagnosis of a SARD over a two-year period. We assessed fulfillment of classification criteria. Risk ratios (RRs) were used to evaluate differences among those who did and did not progress to a SARD. We evaluated 207 asymptomatic ANA-positive or undifferentiated patients, of whom 23 (11%) progressed to a SARD, whereas 187 (89%) did not progress. Progressors developed systemic lupus erythematosus (SLE) (n = 11 [48%]), Sjögren disease (n = 5 [22%]), systemic sclerosis (n = 3 [13%]), rheumatoid arthritis (n = 1 [4%]), and from ANA-positive to undifferentiated connective tissue disease (n = 3 [13%]). Fever (RR 0.89, 95% confidence interval [CI] 0.8-0.93) and antiphospholipid antibodies (RR 0.89, 95% CI 0.87-0.93) occurred less frequently, whereas arthritis (RR 1.74, 95% CI 1.20-2.55) occurred more frequently in progressors. Progressors to SLE had arthritis (91%), whereas none developed delirium, psychosis, or nephritis. Among patients with SLE, 100% fulfilled the EULAR/American College of Rheumatology (ACR) SLE criteria (sensitivity 91.7%, specificity 100%), whereas 73% fulfilled the 1997 ACR SLE criteria (sensitivity 81.8%, specificity 98.9%). Most undifferentiated/asymptomatic individuals with ANA do not progress to a SARD over a two-year period. SLE progressors appear to have mild disease in the short term. The EULAR/ACR SLE criteria have improved ability to identify those who develop SLE.

The role of anticoagulation on the long-term survival of patients with pulmonary arterial hypertension: A meta-analysis of 15 cohort studies

IntroductionAnticoagulation was once recommended for patients with pulmonary arterial hypertension (PAH). However, its survival benefit still remained controversial. We performed a meta-analysis to evaluate the effect of anticoagulation on the long-term survival of PAH patients. MethodsThe PubMed, EMBASE, Web of Science, and WanFang electronic database were searched for eligible studies. Pooled hazard ratios (HRs) with 95 % confidence intervals (CIs) were calculated for effect estimate regarding anticoagulation on the survival of PAH patients. ResultsFifteen cohort studies involving 4266 PAH patients were included. Approximately 45.8 % patients received anticoagulation. The mean follow-up period ranged from 2.1 to 14 years. Anticoagulation had a tendency to, however, did not significantly reduce mortality of PAH patients (HR: 0.86, 95 % CI: 0.73–1.02). In subgroup analysis, anticoagulation decreased the mortality risk as analyzed from retrospective studies (HR: 0.80, 95 % CI: 0.65–0.98), but not prospective studies (HR: 0.95, 95 % CI: 0.70–1.29). For both idiopathic PAH (IPAH) and connective tissue disease associated PAH (CTD-PAH), anticoagulation therapy did not significantly improve the long-term survival rate (HR: 0.83, 95 % CI: 0.65–1.07, and HR: 1.05, 95 % CI: 0.77–1.42, respectively), and this result remained unchanged when pooling data from either retrospective or prospective studies. Further analysis showed that anticoagulation had no advantage in reducing mortality in patients with systemic sclerosis associated PAH, systemic erythematosus lupus related PAH (free of antiphospholipid syndrome), or CTD-PAH of non-specified etiology. ConclusionAnticoagulation may not reduce the long-term mortality of PAH patients, including those with IPAH and CTD-PAH. In the management of PAH, anticoagulants should be prescribed with caution before comprehensive risk to benefit evaluation. Larger and more vigorously designed controlled trials are warranted.

CXCL10 predicts autoimmune features and a favorable clinical course in patients with IIP: post hoc analysis of a prospective and multicenter cohort study

BackgroundInterstitial pneumonia with autoimmune features (IPAF), which does not meet any of the criteria for connective tissue diseases (CTD), has been attracting an attention in patients with idiopathic interstitial pneumonia (IIP). However, the biomarkers that reflect the clinical course of these patients have not been fully elucidated.ObjectiveTo identify useful serum biomarkers reflecting CTD-related features and favorable prognoses in patients with IIP.MethodsThis was a post hoc analysis of a prospective and multicenter cohort study between 2015 and 2020. Newly diagnosed patients with IIP were consecutively enrolled, and 74 autoimmune features and autoantibodies were comprehensively checked during IIP diagnosis. Serum levels of CXCL10, CXCL1, CCL2, BAFF, angiopoietin-2, and leptin were evaluated at the time of IIP diagnosis.ResultsTwo hundred twenty-two patients (159 men and 63 women) with IIP were enrolled. The median observation duration was 36 months. The median age was 71 years old, and median %forced vital capacity (FVC) was 84.1% at the time of IIP diagnosis. The proportion of patients who met the classification criteria for IPAF was 11.7%. In patients with high serum CXCL10, changes in both %FVC and %diffusion lung capacity for carbon monoxide at one year were significantly higher than those in patients with low CXCL10 (p = 0.014 and p = 0.009, respectively), whereas these changes were not significant for other chemokines and cytokines. High CXCL10 levels were associated with acute/subacute onset (p < 0.001) and the diagnosis of nonspecific interstitial pneumonia with organizing pneumonia overlap (p = 0.003). High CXCL10 levels were related to a higher classification of IPAF (relative risk for IPAF was 3.320, 95%CI: 1.571–7.019, p = 0.003) and lower classification of progressive pulmonary fibrosis (PPF; relative risk for PPF was 0.309, 95%CI: 0.100-0.953, p = 0.027) compared to those with low CXCL10. Finally, survival was higher in patients with IPF and high CXCL10 (p = 0.044), and high CXCL10 was a significant prognostic factor in multivariate Cox proportional hazards models (hazard ratio 0.368, p = 0.005).ConclusionsHigh serum levels of CXCL10 are associated with CTD-related features, the favorable clinical course, and survival in patients with IIP, especially IPF.Clinical trial numberNot applicable.

The risk of cancer in pediatric-onset immune-mediated inflammatory diseases – A nationwide study

Background and objectivesAdult-onset immune-mediated inflammatory disease (IMID) increases the risk of several cancers. However, data on pediatric-onset IMID (pIMID) remains scarce. We estimated the long-term cancer risk in pIMID and the association between medical treatment and specific cancers. MethodsWe used the nationwide Danish health registers to identify pIMID patients diagnosed from Jan 1, 1980 to Dec 31, 2018. Patients were matched with ten reference individuals based on age, sex, and residence. The primary exposure was pIMID, including autoimmune hepatitis, primary sclerosing cholangitis, Crohn's disease, ulcerative colitis, juvenile idiopathic arthritis, systemic lupus erythematosus, vasculitis, and connective tissue disease. Secondary exposures were immunomodulators and tumor necrosis factor-α antagonists (anti-TNFα). The primary outcome was cancer. Estimates are presented as hazard ratios adjusted for family income at diagnosis (AHR). ResultsWe included 12,664 pIMID patients and 109,274 reference individuals. Median follow-up time was 10.6 (interquartile range: 5.4–17.7) years for patients and 10.2 (interquartile range: 5.2–17.3) years for reference individuals. Patients with pIMID had a twofold higher cancer risk (AHR 2.2 [95 % confidence interval (CI): 1.8–2.6]) compared with reference individuals. Thiopurine treatment was associated with a higher risk of lymphoma (AHR 6.1 [95%CI: 2.2–16.8]) and skin cancer (AHR 6.1 [95%CI: 2.4–15.4]). Anti-TNFα treatment was associated with a higher risk of lymphoma (AHR 4.9 [95%CI: 1.1–22.6]). ConclusionsWe found an increased cancer risk in patients with pIMID followed into adulthood. Additionally, thiopurines and anti-TNFα were associated with increased lymphoma and skin cancer risks. This highlights the importance of individualized immunotherapy and cancer surveillance.

Nailfold Video-Capillaroscopy in Sarcoidosis: New Perspectives and Challenges.

Nailfold video-capillaroscopy (NVC) is a non-invasive cost-effective technique involving the microscopic examination of small blood vessels of the distal nailfold with a magnification device. It provides valuable information regarding the microcirculation including anomalies such as tortuous or dilated capillaries, hemorrhages, and avascular areas, which can characterize connective tissue diseases. The utility of NVC in the diagnosis and monitoring of systemic sclerosis (SSc) has been investigated in numerous studies allowing the distinction of the specific microvascular pattern of scleroderma from different conditions other than scleroderma (non-scleroderma pattern). Sarcoidosis (SA) is a systemic inflammatory disease that can affect various organs, including the lungs, skin, and lymph nodes. The purpose of our review was to evaluate the current state of the art in the use of NVC in the diagnosis of SA, to understand the indications for its use and any consequent advantages in the management of the disease in different settings in terms of benefits for patients. We searched for the key terms "sarcoidosis" and "video-capillaroscopy" in a computerized search of Pub-Med, extending the search back in time without setting limits. We provided a critical overview of the literature, based on a precise evaluation. After our analysis, we examined the six yielded works looking for answers to our questions. Few studies have evaluated that microcirculation is often compromised in SA, with alterations in blood flow and consequent tissue damage. Basing on highlighted findings, NVC appears to be a useful tool in the initial evaluation of sarcoidosis patients. Furthermore, capillaroscopy is useful in the evaluation of the coexistence of sarcoidosis and scleroderma spectrum disorder or overlap syndromes. In conclusions, no specific pattern has been described for sarcoidosis, and further re-search is needed to fully understand the implications of nailfold capillaroscopy find-ings in this disease and to establish standardized guidelines for its use in clinical practice.

Determination of antinuclear antibodies: role in modern differential diagnosis of connective tissue autoimmune diseases

There is an increase in autoimmune rheumatic diseases among all age population groups globally. Diagnosis is often difficult, because the early stages of the diseases usually do not have specific symptoms. Often, clinical manifestations of autoimmune diseases and non-autoimmune pathologies have similar symptoms. Therefore, the differential diagnosis could be very difficult. Most often, the determination of antinuclear autoantibodies is used for laboratory diagnosis of connective tissue systemic diseases. Autoantibodies can be detected in patients long time before the appearance of symptoms. The correct diagnosis is very important because the therapy of various nosologies can be different. It is especially significant with the invention of targeted therapy. Further analysis of the diagnostic value of autoantibody determination is very important. This article presents examination and follow-up data of three children. The analysis of medical documentation was carried out. The role of determining autoantibodies in the differential diagnosis of autoimmune connective tissue diseases was analyzed. In the first patient with Sjögren’s syndrome, the clinical picture of the underlying disease developed at least 5 years after the detection of high levels anti-SS-A, SS-B and Ro-52 antibodies (immunoblotting) and the speckled pattern (indirect immunofluorescence assay). In the second patient, antibodies against centromeres (specific markers of systemic scleroderma) appeared at least 2 years before clinical symptoms. In the third patient, the specific markers of systemic scleroderma have been detected for 5 years. There were antibodies against centromeres (immunoblotting), high titer and the centromere pattern (indirect immunofluorescence assay). However, the patient has not developed any clinical symptoms of this disease during all time of observation. Thus, the analysis of the presented clinical cases shows that autoantibodies can be detected in patients long time before the onset of clinical manifestations of a specific autoimmune disease. In all three cases, the first immunological examination has been carried out in the background of the disease symptoms, but they were atypical. Identification of specific autoantibodies, is very important for differential diagnosis. In the absence of clinical symptoms, the presence of autoantibodies, is the reason for dynamic observation of the patients.

Systemic sclerosis associated with pulmonary arterial hypertension: Focus on the visceral form of the disease

Introduction. Systemic sclerosis is a connective tissue disease with the development of obliterating arteriolopathy and active fibrosis formation both in internal organs and in the skin. Pulmonary arterial hypertension is a life-threatening manifestation of systemic sclerosis, leading to death if diagnosed late. The search for predictors, as well as associated disease phenotypes, can facilitate early diagnosis and improve prognosis.Aim. To characterize the features of the visceral form in comparison with the limited variant in patients with pulmonary arterial hypertension associated with systemic sclerosis.Materials and methods. 14 patients with visceral and 63 with a limited variant of systemic sclerosis associated with pulmonary arterial hypertension were studied. The diagnosis of systemic sclerosis was established based on the 2013 ACR-EULAR criteria; pulmonary arterial hypertension was verified by right heart catheterization. In all patients, other causes of pulmonary hypertension – heart disease, lung disease, thrombophilia were excluded.Results. At the time of inclusion in the study, patients with visceral systemic sclerosis were younger (48 (35; 56) years) than those with limited systemic sclerosis (54 (49; 63) years, but the differences only approached significant (p = 0.057). All patients had the Raynaud’s syndrome, with limited systemic sclerosis, digital ischemic disorders were more often observed (41% compared to 14%, p = 0.11). Anticentromere antibodies caused by pulmonary arterial hypertension predominated; antibodies to topoisomerase-I were detected only in two patients with limited systemic sclerosis. The severity index was significantly higher in patients with limited systemic sclerosis (p &lt; 0.05). The clinical manifestations of pulmonary arterial hypertension in both groups were also the same. When studying central hemodynamics, no significant differences were found. The median follow-up of patients was 68 (39; 111) months. Survival also did not differ: with visceral systemic sclerosis it was 63 (40; 99) months, with limited systemic sclerosis – 69 (36; 116) months.Conclusion. A comparative analysis demonstrated the similarity of the two systemic sclerosis phenotypes, which suggests the universality of approaches to the early diagnosis of pulmonary arterial hypertension.

Overlap syndrome: Juvenile dermatomyositis with systemic lupus erythematosus and lupus hepatitis in a child: An uncommon presentation

Autoimmune connective tissue disease in children carries a unique management challenge to the treating physician due to the potential years of disease burden and resultant complications. Early diagnosis and treatment positively impact disease course. Overlap syndrome in children is a rare entity. We report a case of juvenile dermatomyositis with systemic lupus erythematosus who on follow-up developed lupus hepatitis. Lupus hepatitis in children with overlap syndrome is not a well-known entity and there is paucity of literature describing this association.

Pseudoxanthoma elasticum: An unique entity in the coronary surgery.

Pseudoxanthoma elasticum is a rare connective tissue disease which affects the synthesis of the elastic fibres. It is an area of uncertainty for patients affected by this disease requiring surgical revascularization. We present a case report of a 43-year-old gentleman was known to have pseudoxanthoma elasticum syndrome (PXE) and coronary artery disease. He underwent successful off pump LIMA to LAD. Interestingly, this is the first case in UK and second one in the literature managed with an off-pump strategy. The predilection of the histopathological changes in PXE patients, should not prevent the routine use in of the left internal mammary artery as a standard conduit in CABG. Further studies are required to standardize the practice for surgical revascularization in the patients with PXE.

Thyroid cancer and autoimmune connective tissue disorders.

There are substantial data confirming the association between autoimmune disorders, including connective tissue diseases (CTDs), and an increased risk of thyroid malignancy. CTDs and thyroid cancer may co-exist as 2 separate diseases because of their relatively high incidence rates in the population. They can arise from each other due to the increased risk of thyroid cancer in patients with idiopathic inflammatory myositis, rheumatoid arthritis, systemic sclerosis, primary Sjögren's syndrome, and systemic lupus erythematosus. Moreover, in some scarce cases, CTDs may act as the paraneoplastic syndromes of thyroid cancer. The presence of CTDs may impact the diagnostic process, especially distorting the results of imaging tests or falsely indicating the increase of thyroglobulin or calcitonin. Finally, TSH suppression is a crucial element of the treatment of differentiated thyroid cancer, which may decrease bone mineral density and increase the risk of osteoporosis by accelerating bone turnover and shortening the bone remodeling cycle. The aim of this review is to emphasise the vital aspects of this interrelationship. The authors discuss this phenomenon aiming at the explanation of possible linking mechanisms. The impact of selected CTDs on thyroid cancer management is presented, as well as the possible effects of cancer therapy on skeletal health.

Practical Use of Ultrasound in Modern Rheumatology-From A to Z.

During the past 20 years, the use of ultrasound (US) in rheumatology has increased tremendously, and has become a valuable tool in rheumatologists' hands, not only for assessment of musculoskeletal structures like joints and peri-articular tissues, but also for evaluation of nerves, vessels, lungs, and skin, as well as for increasing the accuracy in a number of US-guided aspirations and injections. The US is currently used as the imaging method of choice for establishing an early diagnosis, assessing disease activity, monitoring treatment efficacy, and assessing the remission state of inflammatory joint diseases. It is also used as a complementary tool for the assessment of patients with degenerative joint diseases like osteoarthritis, and in the detection of crystal deposits for establishing the diagnosis of metabolic arthropathies (gout, calcium pyrophosphate deposition disease). The US has an added value in the diagnostic process of polymyalgia rheumatica and giant-cell arteritis, and is currently included in the classification criteria. A novel use of US in the assessment of the skin and lung involvement in connective tissue diseases has the potential to replace more expensive and risky imaging modalities. This narrative review will take a close look at the most recent evidence-based data regarding the use of US in the big spectrum of rheumatic diseases.

Síndrome de Stickler: una causa de oftalmopatía pediátrica

Stickler's syndrome is a rare genetic disease of connective tissue, with dominant autosomal inheritance most of the time. It is characterized by the association of ophthalmopathy (myopia, retinal detachment and cataracts), orofacial abnormalities, hearing loss and arthropathy, with a wide variability in clinical expression. Diagnostic confirmation is obtained by genetic study. The prognosis varies depending on the severity of the manifestations. The absence of an etiological treatment reduces the therapeutic possibilities to a symptomatic treatment that is very often surgical. The importance of knowing its characteristics lies in an early clinical suspicion to try to avoid irreversible sequelae at an early age and provide the family with adequate genetic counseling.

Subcutaneous Granuloma Annulare: A Systematic Review on Current Treatments

Subcutaneous Granuloma Annulare (SGA) is a rare subset of Granuloma Annulare (GA), presenting most often in female children and young adults as a benign, nodular lesion. Etiology is unknown but can be linked to stress, immunologic disorders, connective tissue diseases and diabetes. We searched Scopus, PubMed, Embase, and OVID Medline to assess the current landscape of SGA treatment. We searched (“subcutaneous granuloma annulare” OR “pseudorheumatoid nodules” OR “deep granuloma annulare” OR “palisading granuloma”) AND (“treatment” OR “topical” or “injection”). Articles were limited to English language and human subjects. 302 unique studies were screened and assessed for eligibility by two reviewers working independently with disagreements resolved by a third reviewer’s decision. 33 articles met inclusion comprising case reports (n=27), case series (n=3) and retrospective case reviews (n=3) with a total of 149 patients involved. The most predominant treatment was surgical excision/biopsy (n=112) followed by steroid injection (n=6), topical steroid (n=5), and topical antibiotic (n=3). Reports of one patient responding to treatment (n=1) were reported for local hyperthermia, hydroxychloroquine, cyclophosphamide, oral isotretinoin, PUVA light therapy, and pentoxifylline. Of patients studied in these cases, 17 had spontaneous resolution of their lesions either after the treatment window or before starting treatment. One patient was refractory to targeted treatment but had resolution after achieving diabetic control. While surgical excision predominated, biopsy is standard for diagnosis but follow up was not consistent to assess recurrence. Our review calls for more trials and treatment options for SGA.

Impact of connective tissue diseases on complications following aesthetic surgery: A matched cohort study

BackgroundThe association between connective tissue diseases (CTD), including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma, and complications following aesthetic surgery is under-investigated. We hypothesized that the risk of complications following aesthetic surgery was higher in patients with these connective tissue disorders compared to matched non-CTD patients. MethodsAll patients diagnosed with RA, SLE and Scleroderma who underwent aesthetic surgery at our institution from 2003-2022 were reviewed. Demographic data, comorbidities, medications, procedures, and postoperative complications were collected. Non-CTD controls were identified for each procedure and matched 1:1 based on propensity scores derived from race, gender, BMI, smoking status, and comorbidities. ResultsSix hundred and thirty-eight patients were included, comprising 319 (50%) patients diagnosed with CTD, and 319 (50%) controls. The average age at surgery was 56.3 years. There were 129 complications. There were no differences between the CTD and non-CTD patients in number of total complications (69 versus 60, p=0.38), major complications (23 versus 16, p=0.25), or minor complications (46 versus 44, p=0.73). Complications were not significantly different between CTD patients and controls who underwent blepharoplasty (p=0.38), breast reduction (p=0.91), abdominoplasty (p=0.46), or rhytidectomy (p=0.50). CTD patients who underwent breast augmentation had significantly more complications compared to matched non-CTD patients on bivariate analysis (7 versus 0, p=0.018*) and multivariable logistic regression (OR: 10.2, 95% CI: 1.21 to 93.3, p=0.039*). ConclusionsMost aesthetic surgeries can safely be performed in patients with connective tissue diseases. Patients seeking breast augmentation should be counselled on a potentially increased risk of post-operative complications.

Safety analysis of fluoroquinolone drugs in elderly patients over 65 based on FAERS

ABSTRACT Objective This study investigates adverse drug event (ADE) reports from the FAERS related to FQs drugs in patients aged 65 and older. The findings aim to guide the rational clinical use of these drugs in elderly patients. Methods We employed Reporting Odds Ratio (ROR) and Proportional Reporting Ratio (PRR) methods to analyze ADE reports for the representative FQ drugs from Q1 2015 to Q4 2023, covering 36 quarters. Results The analysis identified 6883 ADE cases for ciprofloxacin, 5866 for levofloxacin, 1498 for moxifloxacin, and 317 for ofloxacin. Moxifloxacin showed higher incidences of Cardiac disorders and Psychiatric disorders ADEs (4.01%, 23.11%). Ciprofloxacin and levofloxacin showed higher ADE rates in musculoskeletal and connective tissue diseases (20.18% and 26.97%) compared to moxifloxacin (3.62%) and ofloxacin (9.25%). Additionally, moxifloxacin and ofloxacin showed higher ADE rates for eye disorders (10.61% and 15.03%). Conclusion Different FQs exhibit varying ADE profiles across cardiovascular, vascular and lymphatic, renal and urinary, psychiatric, musculoskeletal and connective tissue, and ocular systems. Patients with underlying systemic diseases should avoid FQs with higher ADE risks for their conditions. Personalized medication plans for elderly patients should also be strengthened.

The impact of Marfan syndrome and Ehlers-Danlos syndrome on the risk of penile fracture in patients between 18 and 45 years.

Despite knowledge of the pathophysiology and clinical complications of connective tissue diseases (CTD), little is known regarding their impact on men's sexual health disorders. To investigate the prevalence of penile fracture (PF) in patients with Ehlers-Danlos Syndrome (EDS) and Marfan syndrome (MFS) in comparison with disease-free controls between 18 and 45years of age. A multicenter, international, electronic health record network (TriNetX) was queried to identify adult male patients (between 18 and 45years) with or without EDS and MFS between 1993 and 2023 using ICD-10 codes. The prevalence of PF was compared between patients with and without the diseases of interest. Prevalence ratios (PR) were generated with 95% confidence intervals. Prevalence of PF in patients with EDS and MFS when compared to disease-free controls. The number of patients with EDS, MFS, and control groups was 8060, 8642, and 20 184 547, respectively, with a mean age of 27.8 ± 7.58, 28.6 ± 7.4, and 31.6 ± 8.04years. Men with EDS had a higher prevalence of PF (PR 30.18, 95% CI [17.08-53.19]; P < 0.0001). Similarly, men with MFS had a higher prevalence of PF (PR 23.4, 95% CI [12.6-43.7]; P < 0.0001). This study demonstrates an association between CTD and men's sexual health disorders. It may be important to counsel such men about the risks of PF. This is the largest study to date to demonstrate an association between CTD and men's sexual health disorders. While the large sample sizes in this study contribute to the robustness of the findings, the study is limited by the use of a claims-based dataset, which does not provide further details about disease course and complications, and the use of a univariate analysis only. Patients with EDS and MFS are possibly at an elevated risk for PF. Due to the limitations of the TriNetX database, the analysis was limited to a univariate one, thus limiting the ability to control for confounders and limiting the generalizability of these findings. Further prospective research is needed to corroborate these findings.

Nintedanib in the treatment of interstitial lung disease in patients with systemic connective tissue diseases

Nintedanib in the treatment of interstitial lung disease in patients with systemic connective tissue diseases