Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study.

OBJECTIVE: To compare clinical and laboratory data obtained from patients with primary Antiphospholipid Syndrome (PAPS) with and without limb ischemia (LI). METHODS: A transverse study with 66 (83.3% female) PAPS patients was performed. All data were evaluated. Patients were subdivided into one of two groups: PAPS with LI and PAPS without LI and compared. RESULTS: Sixty-six primary APS were selected. PAPS with LI group exhibited a longer disease duration (p = .012) and more arterial events (p = .002). A lower frequency of venous events was observed in PAPS with LI (p = .007), and deep venous thrombosis (p = .016). Furthermore, PAPS with LI patients had more deficiency of protein C of coagulation (p = .015) than the others. CONCLUSION: PAPS and LI have a distinct clinical and laboratory spectra from those without LI and it is characterized by an increased frequency of protein C deficiency, and a lower frequency of venous events, deep venous thrombosis and IgM anticardiolipin.

Livedoid vasculopathy in 75 Brazilian patients in a single-center institution: Clinical, histopathological and therapy evaluation.

This study presents a single center experience with livedoid vasculopathy (LV). A rare disease that can lead to severe quality of life impairment. Characterize clinical data of LV patients at the Dermatology Division at the University of São Paulo. A retrospective and transversal study was conducted, from 1 January 2005 to 31 December 2019. About 75 patients diagnosed as LV and confirmed by skin biopsy were included. Epidemiology, clinical appearance, histopathology data, and treatment history were observed. There were 78.66% Caucasian women, with a mean age of 39.9 years. Frequent cutaneous manifestations were ulcers, atrophic blanche-like scars, hyperpigmentation, purpuras, telangiectasias, and livedo racemosa. Pain, pruritus, and hypoesthesia were the main symptoms. After treatment, almost 40% of cases relapsed during spring and summer months. About 66% of cases had thrombophilia factors associated, such as high levels of lipoprotein(a). Frequent treatments included acetylsalicylic acid, pentoxifylline, and diosmin with hesperidin. Not being a prospective study. This research provides useful data on Latin American LV patients, indicating multifactorial conditions involved in LV pathogenesis. An extensive work-up including autoimmune laboratory tests, thrombophilia factors, and other conditions associated with venous stasis should be part of LV investigation and controlled to improve treatment response.

Renal involvement in antiphospholipid syndrome.

This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries. On histology, APS nephropathy is characterized by TMA, but chronic vaso-occlusive lesions are also commonly observed (fibrous intimal hyperplasia, focal cortical atrophy, fibrous occlusions of arteries). Anticardiolipin and lupus anticoagulant are the most prevalent antibodies in patients with APS nephropathy. The spectrum of renal manifestations includes renal vein thrombosis, renal artery thrombosis/stenosis, TMA, increased allograft vascular thrombosis and malignant hypertension. Anticoagulation is the standard treatment of thrombotic events. In systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPL), kidney failure due to SLE nephritis (immune-complex disease) should be clearly distinguished from kidney failure due to APS-related TMA. In such cases, renal biopsy is mandatory. SLE nephritis requires immunosuppressive therapy, whereas APS nephropathy is usually treated with anticoagulants. Recently, eculizumab and sirolimus have been proposed as a rescue therapy. Based on our review, APS nephropathy appears to be a distinct clinical condition. TMA is a characteristic histopathological finding in APS and is strongly associated with the presence of aPL. This has important therapeutic implications and allows distinguishing APS nephropathy from lupus nephritis.

Diagnostic and classification criteria of Takayasu arteritis.

Takayasu arteritis (TA) is a chronic large vessel vasculitis that affects aorta, its main branches and pulmonary arteries. The inflammatory process results in stenosis, occlusion, dilation or aneurysm formation in the arterial wall. TA has been described in different parts of the world and affects predominantly young individuals (<50 years of age). Patients with TA may present constitutional symptoms, vascular pain (e.g. carotidynia) and typical features such as limb claudication, decreased or absent peripheral pulses, vascular bruits, hypertension, and reduction or discrepancies in blood pressure between arms. A proper diagnosis of TA is an important issue since delays may result in significant morbidity. The definition of TA was included in the 1994 and 2012 Chapel Hill Consensus Conference and TA was categorized as a large vessel vasculitis. The first diagnostic criteria for TA were developed by Ishikawa in 1988 and modified by Sharma et al., in 1995. Two sets of classification criteria were developed for TA to include patients in epidemiologic studies and clinical trials: the 1990 ACR Classification Criteria for TA and the Classification Criteria for childhood TA proposed by the European League Against Rheumatism (EULAR), the Pediatric Rheumatology European Society (PRES) and by the Pediatric Rheumatology International Trials Organization (PRINTO) to be used for patients younger than 18 years. The Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) is an international effort that is under way to develop a single classification system and a validated set of diagnostic criteria for systemic vasculitides using data-driven methods.

Ketamine in fibromyalgia: a systematic review.

OBJECTIVE: Fibromyalgia (FM) subjects are treated with antidepressant agents; in most cases, these drugs lose efficacy or have adverse effects. Ketamine is an anesthetic drug used in FM in some studies. This article aims to systematically review the safety and efficacy of ketamine in fibromyalgia (FM) patients. MATERIALS AND METHODS: We systematically searched articles on FM and ketamine published at Pubmed from 1966 to 2021. This study was registered at PROSPERO. RESULTS: There were only 6 articles published in this field, with a total of 115 patients. The female sex was predominant (88 to 100%). The age varied from 23 to 53 years old. Disease duration ranged from 1 month to 28 years. The dosage of ketamine changed from 0.1 mg/kg-0.3-0.5 mg/kg in intravenous infusion (4/5) and subcutaneous application (1/5). Regarding outcomes, the Visual analog scale (VAS) before ketamine was from 59 to 100 mm and after treatment from 2 to 95 mm. Most short-term studies had a good response. Only the study with 8 weeks of follow-up did not observe a good response. Side effects were common; all appeared during the infusion and disappeared after a few minutes of the ketamine injection. CONCLUSIONS: The present study demonstrates the effectiveness and safety of ketamine in FM patients in the short term. Although, more studies, including long-term follow-up studies, are still needed.

Ear Complaints in Fibromyalgia: A Narrative Review.

INTRODUCTION: Patients with fibromyalgia (FM) have innumerable complaints due to the central amplification of somatic stimuli. The aim of this paper was to review the ear complaints in patients with FM. METHODS: A review of articles published in PubMed/MEDLINE, Embase, Web of Science, and Scopus from 1966 to June 2023 was performed. RESULTS: Seventeen papers were included in the review. They showed that patients with FM have a higher hearing loss rate, mostly at high frequencies, and hyperacusis. The prevalence of vestibular symptoms (tinnitus, dizziness) and hyperacusis was higher than in the general population, reaching 87.0% of the sample. Subjective findings did not always correspond to objective results. In some studies, the degree of FM severity was associated with ear symptoms; in others, it was not. CONCLUSIONS: Ear complaints in patients with FM are linked to subjacent disease and may be related to stimuli central amplification.

Cannabis therapy in rheumatological diseases: A systematic review.

Cannabis has been used in rheumatic diseases as therapy for chronic pain or inflammatory conditions. Herein, the authors systematically review the rheumatological diseases in which cannabis has been studied: systemic sclerosis, fibromyalgia, osteoarthritis, rheumatoid arthritis, osteoporosis, polymyalgia rheumatica, gout, dermatomyositis, and psoriatic arthritis. We systematically searched PubMed for articles on cannabis and rheumatic diseases between 1966 and March 2023. Twenty-eight articles have been selected for review. Most of them (n=13) were on fibromyalgia and all of them but one showed important reduction in pain; sleep and mood also improved. On rheumatoid arthritis, two papers displayed decrease in pain and in one of them a reduction in inflammatory parameters was found. In scleroderma there was a case description with good results, one study on local use for digital ulcers also with good outcomes and a third one, that disclosed good results for skin fibrosis. In dermatomyositis a single study showed improvement of skin manifestations and in osteoarthritis (3 studies) this drug has demonstrated a good analgesic effect. Several surveys (n=5) on the general use of cannabis showed that rheumatological patients (mixed diseases) do use this drug even without medical supervision. The reported side effects were mild. In conclusion, cannabis treatment is an interesting option for the treatment of rheumatological diseases that should be further explored with more studies.

Ear abnormalities in chronic fatigue syndrome (CFS), fibromyalgia (FM), Coronavirus-19 infectious disease (COVID) and long-COVID syndrome (PCS), sick-building syndrome (SBS), post-orthostatic tachycardia syndrome (PoTS), and autoimmune/inflammatory syndrome induced by adjuvants (ASIA): A systematic review.

Chronic fatigue syndrome (CFS), fibromyalgia (FM), silicone breast implants (SBI), Coronavirus-19 infectious disease (COVID), COVID-19 vaccination (post-COVIDvac-syndrome), Long-COVID syndrome (PCS), sick-building syndrome (SBS), post-orthostatic tachycardia syndrome (PoTS), and autoimmune/ inflammatory syndrome induced by adjuvants (ASIA) are a cluster of poorly understood medical conditions that have in common a group of ill-defined symptoms and dysautonomic features. Most of the clinical findings of this group of diseases are unspecific, such as fatigue, diffuse pain, cognitive impairment, paresthesia, tachycardia, anxiety, and depression. Hearing disturbances and vertigo have also been described in this context, the underlying pathophysiologic process for these conditions might rely on autonomic autoimmune dysbalance. The authors procced a literature review regarding to hearing and labyrinthic disturbances in CSF, FM, SBI, COVID, post-COVIDvac-syndrome, PCS, SBS, POTS, and ASIA. The PRISMA guidelines were followed, and the literature reviewed encompassed papers from January 1990 to January 2024. After the initial evaluation of the articles found in the search through Pubmed, Scielo and Embase, a total of 172 articles were read and included in this review. The prevalence of hearing loss, dizziness, vertigo and tinnitus was described and correlated with the diseases investigated in this study. There are great variability in the frequencies of symptoms found, but cochlear complaints are the most frequent in most studies. Vestibular symptoms are less reported. The main pathophysiological mechanisms are discussed. Direct effects of the virus in the inner ear or nervous pathways, impaired vascular perfusion, cross-reaction or autoimmune immunoreactivity, oxidative stress, DNA methylation, epigenetic modifications and gene activation were implicated in the generation of the investigated symptoms. In clinical practice, all patients with these autoimmune conditions who have any audiological complaint an ENT consultation followed by an audiometry are needed.

Sexuality and sexual dysfunction in patients with psoriatic arthritis: A cross-sectional study.

OBJECTIVE: Psoriatic arthritis (PsA) is a chronic inflammatory disorder affecting the joints, skin and entheses. Despite the importance of the topic, few studies have investigated the association between PsA and sexual function. The purpose of this study was to assess sexuality and the prevalence of sexual dysfunction (SD) in patients with PsA. METHODS: This was an observational, cross-sectional single-center study on 23 PsA patients (male=12; female=11) evaluated with 2 male questionnaires (MSQ= Male Sexual Quotient, and IIEF=International Index of Erectile Function) and 2 female questionnaires (FSQ= Female Sexual Quotient, and FSFI=Female Sexual Function Index) validated for Brazilian Portuguese, in order to determine changes in sexual function. Clinical parameters, musculoskeletal activity and skin activity were also analyzed to identify factors associated with SD. RESULTS: The mean age was 52.1±9.7 years (males) and 49.1±9.6 years (females). Clinically, the patients had low skin and peripheral joint disease activity or were in remission. The mean time of PsA was 10±6.2 years, and 65.2% had a steady sexual partner. The mean MSQ score was 75.8±16.8. The prevalence of SD was 91.7% in men (IIEF), with a predominance of mild SD. The mean FSQ score was 64.9±24.1. The prevalence of SD was 72.7% in women (FSFI), with low domain scores. Also, a significant association was found between female age and total and domain-specific FSFI scores. PASI (Psoriasis Area and Severity Index) and the general satisfaction domain (IIEF) were significantly correlated. CONCLUSION: This study found a high prevalence of SD in PsA patients. Age had a negative impact on female sexual function. Physicians need to be more aware of SD in this population to provide early multidisciplinary treatment and minimize the impact of the disease on the quality of life of patients and their partners.

Dietetic issues in antiphospholipid syndrome.

There are several issues regarding diet in patients with antiphospholipid syndrome. This article focuses on main factors such as vitamin K ingestion and its relationship with anticoagulants and healthy diet for obesity, dyslipidemia, hypertension, diabetes mellitus that are important risk factors for hypercoagulability. Last, diet rich in calcium is also important since these patients are under anticoagulants and heparins that are related to osteoporosis/osteopenia. The medical follow-up in combination with nutrition counseling was carried out for guiding patients to acquire healthy habits in order to improve their quality of life and gain control of the comorbidities.

Optic neuritis after infliximab therapy.

Demyelinating diseases were described in patients receiving anti-TNF agents. Optic neuritis (ON) induced by TNF blockers was already described in 22 cases in the literature. In this article, the authors report a 53-year-old woman with refractory rheumatoid arthritis that developed neuritis optica after the fourth dose of infliximab and had a good outcome after anti-TNF withdrawal, associated with glucocorticoid treatment. In addition, the previous cases of ON induced by anti-TNF agents were reviewed.

Seizures in systemic sclerosis.

The aim of this study was to evaluate the frequency of seizures in systemic sclerosis (SSc) and to determine the clinical and laboratory features associated with their occurrence. Thirty-four SSc patients (ACR criteria) were analyzed by a standard interview, physical examination, and review of medical charts. Risk factors for seizures, clinical manifestations, associated co-morbidities and current treatment were evaluated. We identified 3 (8.8 %) SSc patients with seizures. A higher median age [61 (35-64) vs. 48 (27-71) years, p = 0.0005] and higher activity score [4.75 (4.5-5.0) vs. 2.5 (0-5-5) years, p = 0.006] were observed in SSc patients with seizures. No other clinical or laboratory feature was associated with the occurrence of seizure in this cohort. This study demonstrated a higher prevalence of seizures in SSc when compared to general population. Seizures were associated with older age and higher activity score in this cohort.

Low dynamic muscle strength and its associations with fatigue, functional performance, and quality of life in premenopausal patients with systemic lupus erythematosus and low disease activity: a case-control study.

BACKGROUND: The purpose of the present study was to compare dynamic muscle strength, functional performance, fatigue, and quality of life in premenopausal systemic lupus erythematosus (SLE) patients with low disease activity versus matched-healthy controls and to determine the association of dynamic muscle strength with fatigue, functional performance, and quality of life in SLE patients. METHODS: We evaluated premenopausal (18-45 years) SLE patients with low disease activity (Systemic lupus erythematosus disease activity index [SLEDAI]: mean 1.5 ± 1.2). The control (n = 25) and patient (n = 25) groups were matched by age, physical characteristics, and the level of physical activities in daily life (International Physical Activity Questionnaire IPAQ). Both groups had not participated in regular exercise programs for at least six months prior to the study. Dynamic muscle strength was assessed by one-repetition maximum (1-RM) tests. Functional performance was assessed by the Timed Up and Go (TUG), in 30-s test a chair stand and arm curl using a 2-kg dumbbell and balance test, handgrip strength and a sit-and-reach flexibility test. Quality of life (SF-36) and fatigue were also measured. RESULTS: The SLE patients showed significantly lower dynamic muscle strength in all exercises (leg press 25.63%, leg extension 11.19%, leg curl 15.71%, chest press 18.33%, lat pulldown 13.56%, 1-RM total load 18.12%, P < 0.001-0.02) compared to the controls. The SLE patients also had lower functional performance, greater fatigue and poorer quality of life. In addition, fatigue, SF-36 and functional performance accounted for 52% of the variance in dynamic muscle strength in the SLE patients. CONCLUSIONS: Premenopausal SLE patients with low disease activity showed lower dynamic muscle strength, along with increased fatigue, reduced functional performance, and poorer quality of life when compared to matched controls.

Low-Dose Naltrexone in Rheumatological Diseases.

Background: Naltrexone has been approved for alcohol and opioid abuse by the FDA. At low-dose naltrexone (LDN) has been used in several diseases including chronic pain and autoimmune conditions, including rheumatic disorders. Aim: To review the use of LDN in rheumatic diseases: systemic sclerosis (SSc), dermatomyositis (DM), Sjögren's syndrome (SS), rheumatoid arthritis (RA), and fibromyalgia (FM). Methods: PubMed and Embase databases were searched for articles on LDN and rheumatic diseases between 1966 and August 2022. Results: Seven studies in FM have been identified: in this disease LDN has showed beneficial effects on pain and well-being. In SS, two articles with 3 cases description showed that LDN may be of help in the pain treatment. LDN relieved pruritus in scleroderma (a case description with a series of 3 patients) and dermatomyositis (description of 3 patients in two articles). In RA a study using Norwegian Prescription Database showed that LDN was associated to reduction in the use of analgesic and DMARDs. No serious side effects were detected. Conclusion: This review shows that LDN is a promising and safe therapy to be used in some rheumatic disease. However, the data is limited and needs to be reproduced in larger studies.

Dehydroepiandrosterone (DHEA) Supplementation in Rheumatic Diseases: A Systematic Review.

Background: Dehydroepiandrosterone (DHEA) is an adrenal hormone used to treat rheumatic conditions such as systemic lupus erythematosus (SLE), Sjogren's syndrome (SS), rheumatoid arthritis (RA) with controversial results. Aim: To review the results of DHEA use in rheumatic diseases. Methods: PubMed, Scielo, Scopus, and Embase databases were systematically searched for articles on the treatment of rheumatic diseases with DHEA between 1966 and April 2023. Results: Twenty-one studies were identified: 13 in SLE, 5 in SS, 2 in RA, and 1 in fibromyalgia. DHEA use in SLE has shown a mild to moderate effect on disease activity, a positive effect on bone mineral density (BMD), and improved fatigue. The studies on SS showed a decrease in symptoms of dry mouth, but its performance did not differ from placebo in disease activity. In RA, a questionable effect on disease activity was noted. The only study on fibromyalgia failed to show any improvement. The drug was well tolerated; mild androgenic effects were the most common complaints. Conclusion: DHEA seems to have a place in SLE treatment, where it improves BMD and disease activity. The use in RA, SS, and FM is questionable.

Melatonin supplementation improves rheumatological disease activity: A systematic review.

BACKGROUND: Melatonin is a pineal hormone with a complex role. It is linked to sleep, inflammatory, oxidative, and immunological processes. AIM: To review the use of melatonin supplementation in rheumatological diseases. METHODS: A systematic search of PubMed, Embase, and Scielo databases was performed, looking for articles on Melatonin and rheumatic diseases published between 1966 and August 2022. RESULTS: Thirteen articles were identified: in fibromyalgia (n = 5 articles), rheumatoid arthritis (n = 2), systemic sclerosis (n = 1), systemic lupus erythematosus (n = 1) and osteoporosis/osteopenia (n = 3) and osteoarthritis (n = 1). There were positive results of melatonin administration in fibromyalgia, osteoarthritis, and osteoporosis/osteopenia but not in rheumatoid arthritis and lupus. The drug was well tolerated with mild side effects. CONCLUSION: This review shows the efficacy of Melatonin in some rheumatic diseases. However, new studies are needed to elucidate the real role of this treatment in rheumatology.

Prevalence of Metabolic Syndrome and Insulin Resistance in Adult Patients with Acne: Association with Traditional Cardiovascular Risk Factors and Acne Severity.

Introduction: Acne is a chronic inflammatory disease that affects the pilosebaceous unit, and there are conflicting evidences regarding its association with metabolic syndrome (MS) and insulin resistance (IR). Methods: A cross-sectional study was performed with 162 acne patients, over 20 years of age, matched for age and sex with 78 healthy controls without acne. The measured parameters included waist circumference (WC), body mass index (BMI), systolic blood pressure, diastolic blood pressure, fasting blood glucose, fasting insulin, high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglycerides (TG), and total cholesterol. Acne severity was determined according to the Global Acne Grading System. The criteria used for the diagnosis of MS were those of the Harmonizing the Metabolic Syndrome Statement, adjusted for South Americans, and the IR was calculated using the HOMA-IR. Results: The prevalence of MS was significantly higher in cases, compared to controls (12.3% vs. 2.6%, P = 0.014), as was the prevalence of IR (11.7% vs. 3.8%, P = 0.047). In addition, MS and IR showed a positive correlation with the degree of acne severity (P = 0.011 and P = 0.021, respectively). HDL levels were significantly lower in cases (P = 0.012) and showed an association with acne severity (P = 0.038). In the logistic regression model, the risk factor that independently influenced both MS and IR in patients with acne was the WC (P = 0.001). Conclusions: Adults with acne, especially the most severe cases, are significantly more likely to have MS, IR, and lower HDL levels, compared to controls without acne.