Interleukin 6 Levels and Disease Activity in Takayasu Arteritis: A Systematic Review With Meta-analysis.

BACKGROUND: Various studies have suggested interleukin 6 (IL-6) as a potential biomarker for detecting disease activity in Takayasu arteritis. METHODS: A systematic review and meta-analysis was performed to assess differences in IL-6 levels in patients with active (aTA) and inactive Takayasu arteritis (iTA), as well as healthy controls (HCs), using validated activity scores. Study quality and the risk of bias were assessed using STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) and the Newcastle-Ottawa and Joanna Briggs checklist, respectively. For the meta-analysis, we pooled the raw mean IL-6 levels in each group and then estimated and pooled the crude mean differences between the groups. We applied a random-effects model in all analyses. RESULTS: Of the 93 eligible articles, 10 were included after removing duplicates and studies that met the exclusion criteria. Overall, 1825 patients with a mean age ranging from 24 to 40.6 years were included. The pooled levels of IL-6 increased depending on disease activity as follows: HCs: 3.08 (95% confidence interval [CI], 0.88-5.28), iTA: 7.21 (3.61-10.82), and aTA: 22.67 (12.44-32.91) pg/mL. Patients with aTA had higher IL-6 levels than HCs (21.52 [95% CI, -0.43 to 43.47]) and those with iTA (16.69 [95% CI, 5.32-28.06]), whereas IL-6 levels were not different between HCs and patients with iTA (3.62 [95% CI, -13.18 to 20.42]). CONCLUSIONS: Interleukin 6 levels are significantly increased in patients with aTA compared with those with iTA and HCs but not in patients with iTA compared with HCs. More studies are needed to establish the IL-6 cutoff value for assessing disease activity.

Clinical disease activity index applicability in lupus arthropathy: Unraveling underdiagnosed joint activity.

INTRODUCTION: Lupus arthropathy (LA) ranges from arthralgia and non-deforming arthritis to severe forms such as Jaccoud-type deformities and mutilating arthritis. Considering the evolving concept of LA, measuring arthritis activity in lupus patients may require a more practical and sensitive tool other than the classical composite scores. METHODS: In this cross-sectional study, we evaluated the articular pattern of a sample of SLE patients which were divided into those that scored in articular domain on Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and those with activity arthritis using the Clinical Disease Activity Index (CDAI). After all, we analyzed the association between CDAI and arthritis by SLEDAI-2K as well as its association with the presence or not of Jaccoud-type arthropathy (JA). RESULTS: A total of 127 patients with SLE were evaluated. According to SLEDAI-2K, 17 (13.4%) patients have scored in its joint criteria and 32 patients (25.19%) were considered to have some articular activity by CDAI. A total of 16 patients (50%) who scored some activity on CDAI did not score in articular domain of SLEDAI-2K. Also, the presence of Jaccoud-type arthropathy was significantly associated with arthritis activity according to the CDAI score (p = .014) but not with SLEDAI-2K joint criteria (p = .524). CONCLUSION: The CDAI was not directly associated with the presence of arthritis by the joint criteria of SLEDAI-2K and the presence of JA was significantly associated with the CDAI but not with arthritis at SLEDAI-2K.

Prevalence of subclinical systolic dysfunction in Takayasu's arteritis and its association with disease activity: a cross-sectional study.

BACKGROUND: Takayasu's arteritis (TA) is a vasculitis that affects the aorta and its branches and causes stenosis, occlusion, and aneurysms. Up to 60% of TA patients are associated with cardiac involvement which confers a poor prognosis. Global longitudinal strain (GLS) analysis is an echocardiographic technique that can detect the presence of subclinical systolic dysfunction. Hence, this study aimed to describe the prevalence of subclinical systolic dysfunction in patients with TA using the GLS method and to correlate this finding with disease activity using the ITAS-2010 (Indian Takayasu Activity Score). METHODS: Thirty patients over 18 years of age who met the American College of Rheumatology (ACR) 1990 criteria for TA were included. The sample was submitted for medical record review, clinical and echocardiographic evaluation, and application of ITAS-2010. The cutoff for systolic dysfunction was GLS > - 20%. RESULTS: Of the 30 patients analyzed, 25 (83.3%) were female, and the mean age was 42.6 years (± 13.2). The median time since diagnosis was 7.5 years [range, 3-16.6 years], and the type V angiographic classification was the most prevalent (56.7%). Regarding echocardiographic findings, the median ejection fraction (EF) was 66% [61-71%] and the GLS was - 19.5% [-21.3 to -15.8%]. Although half of the participants had reduced GLS, only two had reduced EF. Eleven patients (33.%) met the criteria for activity. An association was found between disease activity and reduced GLS in eight patients (P = 0.02) using the chi-square test. CONCLUSION: GLS seems to be an instrument capable of the early detection of systolic dysfunction in TA. The association between GLS and disease activity in this study should be confirmed in a study with a larger sample size.

Prevalence of subclinical systolic dysfunction in Takayasu's arteritis and its association with disease activity: a cross-sectional study

Abstract Background Takayasu's arteritis (TA) is a vasculitis that affects the aorta and its branches and causes stenosis, occlusion, and aneurysms. Up to 60% of TA patients are associated with cardiac involvement which confers a poor prognosis. Global longitudinal strain (GLS) analysis is an echocardiographic technique that can detect the presence of subclinical systolic dysfunction. Hence, this study aimed to describe the prevalence of subclinical systolic dysfunction in patients with TA using the GLS method and to correlate this finding with disease activity using the ITAS-2010 (Indian Takayasu Activity Score). Methods Thirty patients over 18 years of age who met the American College of Rheumatology (ACR) 1990 criteria for TA were included. The sample was submitted for medical record review, clinical and echocardiographic evaluation, and application of ITAS-2010. The cutoff for systolic dysfunction was GLS > - 20%. Results Of the 30 patients analyzed, 25 (83.3%) were female, and the mean age was 42.6 years (± 13.2). The median time since diagnosis was 7.5 years [range, 3-16.6 years], and the type V angiographic classification was the most prevalent (56.7%). Regarding echocardiographic findings, the median ejection fraction (EF) was 66% [61-71%] and the GLS was - 19.5% [-21.3 to -15.8%]. Although half of the participants had reduced GLS, only two had reduced EF. Eleven patients (33.%) met the criteria for activity. An association was found between disease activity and reduced GLS in eight patients (P = 0.02) using the chi-square test. Conclusion GLS seems to be an instrument capable of the early detection of systolic dysfunction in TA. The association between GLS and disease activity in this study should be confirmed in a study with a larger sample size.

Vasculitis induced by biological agents used in rheumatology practice: A systematic review.

Objectives: Biological medications have been used with an increasing frequency to treat rheumatological diseases. Autoimmune events can be induced by these drugs, such as psoriasiform lesions, alopecia, lupus and, vasculitis, which more often affects the skin (small-sized vessels) and eventually other organs. In this review, we describe the clinical profile of patients with vasculitis induced by the main biological agents used in rheumatology. Patients and methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. The PubMed database was used for searching eligible articles. We included case reports, case series, and letter to the editor of patients on anti-tumor necrosis factor-alpha (anti-TNF-a) molecules, as well as tocilizumab, ustekinumab, secukinumab, rituximab, and abatacept, who had vasculitis induced by these agents. Results: Eighty-one articles were included for final analysis (n=89). Twenty-seven patients were using infliximab, 20 adalimumab, 18 etanercept, seven secukinumab, four certolizumab, four rituximab, three golimumab, three ustekinumab, two abatacept, and one tocilizumab. Unspecific leukocytoclastic vasculitis (LCV) was the most common type of vasculitis (n=37), followed by anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (n=16). The medication was replaced with another biological molecule in 23 cases, with only four relapses. In six cases, the biological was maintained, but vasculitis worsened/persisted in one case, being necessary drug removal. Conclusion: Infections, infusion reaction, cancer, and autoimmune events are well-known side effects of biological therapy. This review demonstrates that vasculitis is another adverse effect of this type of therapy, particularly the anti-TNF-a molecules, and LCV the most reported type of vasculitis.

Cryofibrinogenemia: What Rheumatologists Should Know.

Cryofibrinogenemia refers to the presence of cryofibrinogen in plasma. This protein has the property of precipitating at lower temperatures. Cryofibrinogenemia is a rare disorder, clinically characterized by skin lesions, such as ulcers, necrosis, livedo reticularis, arthralgia, thrombosis, and limb ischemia. These features are most often observed in rheumatological practice and consist of differential diagnoses of antiphospholipid syndrome, primary vasculitis, thrombotic thrombocytopenic purpura, and cryoglobulinemia. Classical histopathological findings include the presence of thrombi within the lumen of blood vessels of the skin without vasculitis. To date, there are no validated classification criteria. Management includes corticosteroids, immunosuppressive therapy, anticoagulants, and fibrinolytic agents. This narrative review aims to make physicians, particularly rheumatologists, aware of the existence of this underdiagnosed condition. There are no epidemiological studies evaluating the prevalence of cryofibrinogenemia in different rheumatological disorders. Studies are also required to investigate if certain features of rheumatological diseases are related to the presence of cryofibrinogenemia.

Jaccoud-type lupus arthropathy.

BACKGROUND: Jaccoud's arthropathy (JA) is a condition characterized by joint deformities that have a "reducible" pattern, that is, they return to the normal appearance with a passive manoeuvre. JA was described in patients with rheumatic fever (RF) more than a century ago, and presently, the majority of the patients have systemic lupus erythematosus (SLE). PURPOSE: The aim of this review is to draw one attention to the epidemiology, pathogenesis, histopathology, clinical features, imaging, and management of JA in patients with SLE (Jaccoud-type lupus arthropathy). DATA COLLECTION: The search strategy included articles retrieved from PubMed utilizing the terms "lupus arthropathy", "lupus deforming arthropathy", "lupus hand", "lupus foot", "chronic postrheumatic arthropathy", "Jaccoud's" and "Jaccoud" from 1950 until March 2021, with no language restriction. RESULTS: The prevalence of Jaccoud-type arthropathy in SLE is approximately 5%. The aetiopathogenic mechanisms of JA are not yet known. The most common joint deformities of JA, are ulnar deviation, swan neck, and the "z" of the thumb. Unfortunately, none of the proposed classification criteria for JA have been validated so far. Characteristically, there is no bone erosion on plain radiographs of the joints, but more sensitive imaging methods, magnetic resonance imaging or high-performance ultrasound may reveal small bone erosions. There is no preventive measure against JA development, specific clinical treatment or convincing surgical approach for correcting the deformities. CONCLUSION: As daily activities and quality of life are compromised in patients with JA, other studies are urgently needed in this area.

Efficacy of hydroxychloroquine in the prevention of thromboembolic events: A systematic review and meta-analysis.

OBJECTIVE: To estimate the risk ratio (RR) of thromboembolic events in chloroquine and hydroxychloroquine users compared to non-users. METHODS: We systematically reviewed randomized controlled trials (RCTs), using MEDLINE and EMBASE databases from inception to the present, reporting thromboembolic events in chloroquine and hydroxychloroquine users compared to non-users. Four authors independently screened all the records obtained through our search strategy and later revised the selected full-text articles for eligibility, according to our inclusion criteria. The same four authors independently extracted relevant data through a customized data collection form while two other authors assessed the quality of the included RCTs using the Cochrane risk-of-bias tool (Version 2.0). All the disagreements were resolved through discussions among the authors. We calculated the risk ratio (RR) and its respective standard error of developing thromboembolic events in hydroxychloroquine users and non-users for each individual study and pooled the results using a random effects model meta-analysis. We assessed Heterogeneity using the Tau2 and I2, and publication bias using funnel plotting and Egger's regression. The protocol for this systematic review is registered at the PROSPERO database (CRD42021247902). RESULTS: Thirteen RCTs met our eligibility criteria and were included in our analysis (2663 patients). We found that hydroxychloroquine-no study on chloroquine was found-reduced the risk of thromboembolic events by 49% (RR 0.51[IC 95% 0.31-0.84]) with a medium heterogeneity (I2 = 67% and T2 = 0.4948). We did find some asymmetry in the inspection of the funnel plot, which was ruled out through an Egger's regression (p-value = 0.1025). CONCLUSION: Our data reinforce the idea that hydroxychloroquine reduces the risk of thromboembolic events.

Pregnancy in Takayasu arteritis: a cross-sectional study and review of literature.

OBJECTIVE: Takayasu arteritis (TA), a form of vasculitis affecting large-and medium-sized vessels; it mainly affects women of reproductive age. Although cardiovascular and hemodynamic changes during pregnancy represent a potential risk for TA, findings regarding risk in maternal and fetal outcomes are inconsistent. This study aimed to describe the prevalence and outcomes of pregnancies in patients with TA, along with a literature review of comparable studies on the subject matter. METHODS: This cross-sectional study was conducted between January and March 2020. We evaluated 20 women diagnosed with TA according to clinical and angiographic findings. RESULTS: The median age of the participants was 38 years. The median age at TA diagnosis was 26 years. Thirteen of the 20 participants reported at least one pregnancy. There were 38 pregnancies, including 26 deliveries (20 vaginal and six cesarean deliveries) and 12 abortions. The most common obstetric complication was spontaneous abortion (25%), followed by prematurity (7.89%), and eclampsia/preeclampsia (5.26%). Only one of our patients gave birth without any complications after being diagnosed with TA. In this case, the disease remained inactive throughout the pregnancy and postpartum periods. CONCLUSIONS: The most common obstetric complication encountered was spontaneous abortion. The maternal and fetal outcome findings were similar to those of previously published studies. The literature shows that patients with stable pregestational TA generally have a good prognosis.

High prevalence of latent tuberculosis using the QuantiFERON-TB Gold Plus test in Takayasu arteritis.

Objectives: This study aims to investigate latent tuberculosis using the QuantiFERON-TB Gold Plus method in patients with Takayasu arteritis (TA). Patients and methods: This case-control study included 22 patients with TA (3 males, 19 females; median age: 36.5 years; IQR, 32 to 50 years), 22 healthy individuals (3 males, 19 females; median age: 38.5 years; IQR, 32.5 to 50 years), and 66 patients with diffuse connective tissue diseases (DCTDs) (4 males, 62 females; median age: 41 years; IQR, 29.8 to 54 years). Two control groups were formed: (i) age- and sex-matched healthy individuals and (ii) patients with other DCTDs. Epidemiological data were collected, and the QFT-Plus test was performed. The QFT-plus positivity was compared among the groups. Results: A higher prevalence of QFT-Plus positive cases was observed in the TA group (8/22) than in the healthy control group (1/22) (p=0.020) or in the group with other DCTDs (3/66) (p=0.001). There was a statistically significant difference in the past pulmonary tuberculosis prevalence between the TA and DCTD groups (p=0.013). Conclusion: The prevalence of latent tuberculosis in TA patients (36.4%) was higher than that in both control groups and higher than the prevalence of latent tuberculosis among the general Brazilian population. Although a positive association was found, it is not possible to establish a direct cause-effect relationship. Given the increasing use of anti-cytokine therapies in TA, it is necessary to thoroughly screen patients with TA before initiating immunosuppressive therapy to avoid tuberculosis reactivation.

Phase III/IV, Randomized, Fifty-Two-Week Study of the Efficacy and Safety of Belimumab in Patients of Black African Ancestry With Systemic Lupus Erythematosus.

OBJECTIVE: Enrollment of patients of Black African ancestry with systemic lupus erythematosus (SLE) in phase II and phase III of the belimumab trials was not reflective of the racial distribution observed in the lupus population. This study was undertaken to assess the efficacy and safety of intravenous (IV) belimumab plus standard therapy in patients of self-identified Black race. METHODS: EMBRACE (GSK Study BEL115471; ClinicalTrials.gov identifier: NCT01632241) was a 52-week multicenter, double-blind, placebo-controlled trial in adults of self-identified Black race with active SLE who received monthly belimumab 10 mg/kg IV, or placebo, plus standard therapy. The optional 26-week open-label extension phase included patients who completed the double-blind phase. The primary end point of the study was SLE Responder Index (SRI) response rate at week 52 with modified proteinuria scoring adapted from the SLE Disease Activity Index 2000 (SLEDAI-2K) (SRI-SLEDAI-2K). Key secondary end points included SRI response rate at week 52, time to first severe SLE flare, and reductions in prednisone dose. RESULTS: The modified intent-to-treat population comprised 448 patients, of whom 96.9% were women and the mean ± SD age was 38.8 ± 11.42 years. The primary end point (improvement in the SRI-SLEDAI-2K response rate at week 52) was not achieved (belimumab 48.7%, placebo 41.6%; odds ratio 1.40 [95% confidence interval 0.93, 2.11], P = 0.1068); however, numerical improvements favoring belimumab were observed, in which the SRI-SLEDAI-2K response rates were higher in those who received belimumab compared with those who received placebo, especially in patients with SLE who had high disease activity or renal manifestations at baseline. The safety profile of belimumab was generally consistent with that observed in previous SLE trials. Adverse events were the primary reasons for double-blind phase withdrawals (belimumab 5.4%, placebo 6.7%). CONCLUSION: The primary end point of this study was not achieved, but improvement with belimumab versus placebo was observed, suggesting that belimumab remains a suitable treatment option for SLE management in patients of Black African ancestry.

Genetic Polymorphisms in Patients With Systemic Lupus Erythematosus and Jaccoud Arthropathy: A Pilot Study.

INTRODUCTION: Jaccoud arthropathy (JA) is a nonerosive and deforming arthropathy experienced frequently by patients with systemic lupus erythematosus (SLE). Although genetic polymorphisms are associated with SLE development, the association between genetic polymorphisms and JA has not been studied to date. The main objective of this study was to evaluate an association between HLA, STAT4, IRF5, and BLK polymorphisms and the presence of JA in Brazilian individuals with SLE. METHODS: Patients were selected from a cohort of individuals with SLE followed at 2 rheumatology reference centers in Salvador, Bahia, Brazil. The JA diagnosis was based on clinical and radiological criteria. The participants were genotyped for rs9271100, rs7574865, rs10488631, and rs13277113 polymorphisms in the HLA, STAT4, IRF5, and BLK genes, respectively, using real-time polymerase chain reaction. The presence of JA was correlated with allele frequencies, and clinical and laboratory data. RESULTS: One hundred forty-four individuals with SLE (38 with JA and 106 with SLE without JA) were studied. The mean age of the patients was 45 ± 12 years; the majority were women and had brown skin. Patients with JA had a longer disease duration than patients without JA. Serositis and neuropsychiatric manifestations were more frequent in the JA population. The A allele of rs13277113 in the BLK gene was associated with the presence of JA. CONCLUSIONS: The rs13277113 polymorphism in the BLK gene was found to be a possible genetic risk for JA development. However, further studies in larger populations should be performed to confirm this finding.

Pulmonary sarcoidosis with arterial involvement.

Necrotizing sarcoid granulomatosis (NSG) is a rare and under-recognized cause of granulomatous disease, described as a variant of typical nodular sarcoidosis. It can be asymptomatic when the patient has a single pulmonary nodule or may be accompanied by cough, fever, and dyspnea, or even symptoms due to the involvement of other organs such as the eyes, liver, and central nervous system. The histopathological analysis is essential for the differential diagnosis of other infectious and non-infectious causes of granuloma and to determine the appropriate treatment. NSG is characterized by the presence of a granuloma with extensive coagulative necrosis associated with the occurrence of vasculitis. We present the case of a patient diagnosed with NSG who had an unusual outcome with recurrent pulmonary thromboembolisms followed by hemodynamic instability and death.

Safety of treatment with chloroquine and hydroxychloroquine: A ten-year systematic review and meta-analysis.

OBJECTIVE: To estimate the incidence rate ratio (IRR) of adverse events (AE) in chloroquine or hydroxychloroquine users. METHODS: We systematically reviewed randomized controlled trials (RCTs), using MEDLINE (2010-2020) and EMBASE (2010-2020) databases, reporting AE in chloroquine or hydroxychloroquine users during treatment for lupus, rheumatoid arthritis, malaria and COVID-19. The protocol for this systematic review is registered at the PROSPERO database (CRD42020197938). The quality of the included studies was assessed using the Cochrane risk-of-Bias tool and relevant data were extracted though a customized data collection form, independently, by two authors. The IRR of AE was estimated using a random-effect model meta-analysis and heterogeneity was evaluated by T2 and I2. Subgroup analysis was performed, and publication bias was assessed by funnel-plot. RESULTS: Forty-six RCTs met our eligibility criteria and were included in our analysis (23132 patients). There was not a single death attributed to chloroquine or hydroxychloroquine use in the included RCTs. The IRR of general AE during antimalarial use was 1.15 [CI 95% 1.01-1.31]. COVID-19 patients treated with either antimalarial presented an 83% and 165% higher risk of developing general and gastrointestinal AE, respectively, in comparison with controls. The use of antimalarial increased the risk of developing dermatological AE by 92% in malarial studies and reduced by 65% in lupus studies. We did not find a significatively higher risk of cardiovascular nor ophthalmological AE in antimalarial users. CONCLUSIONS: Our data reinforces that chloroquine and hydroxychloroquine have a good safety profile though caution is advised when using higher than usual doses in hospitalized COVID-19 patients.

Biological therapy in rheumatoid vasculitis: a systematic review.

Rheumatoid vasculitis (RV) is one of the most severe extra-articular manifestations of rheumatoid arthritis, with significant morbidity and mortality, requiring aggressive treatment with corticosteroids and/or immunosuppressants. Recently, biological drugs were included in its therapeutic armamentarium. The objective of this study was to perform a systematic review on the use of biological drugs in the treatment of RV. A systematic literature review was performed based on PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations and searching articles in MEDLINE/PubMed, Cochrane, SciELO, Scopus, and Virtual Health Library electronic databases. Secondary references were also evaluated. The methodological quality of the selected studies was evaluated by the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) criteria. Altogether, five articles, assessing the use of biological drugs, were included. Globally, 35 patients participated in the studies, of which 21 were treated with rituximab (RTX) in cycles of 1000 mg every 2 weeks; 9 used infliximab 5 mg/kg; 3 used infliximab 3 mg/kg; and 2 used etanercept 25 mg twice/week. In general, an improvement in clinical picture, reduction of the mean daily dose of corticosteroids, and improvement in the Birmingham Vasculitis Activity Score was achieved by the end of the treatment. Complete remission occurred in almost 70% of the cases. The adverse effect rate was 34%, mainly due to infections. There were two deaths, one due to sepsis and the other due to uncontrolled vasculitis, after the biological drug withdrawal, following the development of sepsis. Based on the results of the present review, we believe that the use of biological therapy such as RTX and anti-tumor necrosis factor α can be beneficial in treating this complication.

Bronchoalveolar Lavage in Systemic Sclerosis Patients: A Systematic Review.

INTRODUCTION: We performed a systematic review of the literature to determine the value of bronchoalveolar lavage (BAL) in evaluating the pulmonary involvement of systemic sclerosis (SSc). METHODS: Articles published between 1980 and 2019 were retrieved from the databases: PubMed and Scielo. The search was restricted to clinical trials published in English, utilizing the keywords "scleroderma, systemic sclerosis, interstitial lung disease, and bronchoalveolar lavage". RESULTS: Twenty-two papers were analyzed. A positive correlation was observed between increased BAL cellularity (alveolitis) and worsening clinical symptoms, pulmonary function, and radiological pattern in 2, 11, and 15 studies, respectively. The majority of BAL studies that evaluated interleukin levels, including TNF-α, IL-6, IL-7, and IL-8, observed higher levels in patients with impaired pulmonary function and increased lung involvement. CONCLUSIONS: Alveolitis and the increase of some cytokines/chemokines in BAL were related to more severe pulmonary disease in SSc in the majority of the studies and seemed to be markers of worse prognosis, but it is unknown whether BAL adds clinical value to the use of the other non-invasive diagnostic procedures.

Videocapillaroscopic Findings in Patients With Systemic Lupus Erythematosus With or Without Jaccoud Arthropathy.

BACKGROUND/OBJECTIVE: Systemic lupus erythematosus (SLE) is an autoimmune disease that can present changes in blood vessels, which can be evaluated by periungual nailfold videocapillaroscopy (VCP). This technique is important for the diagnosis of systemic sclerosis and to identify individuals with Raynaud phenomenon at higher risk of developing systemic sclerosis. This study aims to describe the videocapillaroscopic profile of a series of SLE patients and to investigate if the VCP pattern is different among those with Jaccoud arthropathy (JA) compared with those without. METHODS: Between September 2014 and March 2015, the patients in this study underwent VCP, clinical evaluation, and laboratory tests. The capillaroscopic patterns were defined as minor, major, and scleroderma (SD). The presence of capillaroscopic findings, such as elongated capillaries, tortuosity, ectasia, prominent venous plexus, neoangiogenesis, hemorrhage, and megacapillaries, were also observed. Associations were calculated using the χ2, Fisher exact, or Student t test. RESULTS: In a population of 113 females with SLE (67 without JA and 46 with JA), at least 1 alteration was observed in VCP in 89.40% of them, among which "nonspecific changes" were the most prevalent. Minor changes were seen in 39 (58.2%) and 26 (56.5%), major changes in 21 (31.3%) and 11 (23.9%), and SD pattern in 2 (3.0%) and 3 (6.5%), in the patients without and with JA, respectively (p > 0.05). CONCLUSIONS: The majority of patients with SLE demonstrated changes in the VCP examination, but this tool did not allow discrimination between those with or without JA.

Acute Peripheral and/or Cutaneous Ischemic Syndrome: What Rheumatologists Should Know.

ABSTRACT: In daily practice, the frequent appearance of limb and/or skin necrosis, which we term "acute peripheral and/or cutaneous ischemic syndrome" (APCIS), can be a manifestation of numerous underlying diseases, or it can sometimes be a clinical phenomenon whose etiology is undefined even after a wide investigation. The mechanisms for the development of APCIS include vessel wall abnormalities (atherosclerosis, vasculitis, and calciphylaxis), embolic processes (infectious endocarditis, atrial myxoma, and cholesterol emboli), local thrombotic injuries (genetic or acquired thrombophilias and heparin- and warfarin-induced ischemia), dysproteinemias (cryoglobulinemia and cryofibrinogenemia), or venous limb gangrene. Here, we report 5 illustrative cases of APCIS with different pathogenetic mechanisms, thereby highlighting some clinical conditions that cause APCIS that may be of special interest for rheumatologists, such as antiphospholipid syndrome, primary and secondary vasculitis, and cryoproteinemias. Furthermore, we describe a large spectrum of other causes of APCIS beyond the scope of rheumatology. Because there are no validated guidelines for APCIS, we tentatively propose an initial diagnostic workup and a therapeutic approach based on full-dose anticoagulation and immunosuppressive therapy.