Positive Anticardiolipin Antibodies Research Articles

Anti-phospholipid antibodies nephropathy is associated with an increased risk of kidney failure: a systematic literature review and meta-analysis.

Anti-phospholipid antibodies nephropathy (aPL-N) is a complex feature of anti-phospholipid syndrome due to microvascular lesions. Renal prognosis and predictors of outcome are not yet known. We performed a systematic review of the literature (February 2006-January 2024) using the PubMed, Scopus, Cochrane Library and EMBASE databases. Two reviewers independently conducted literature screening and data extraction in a blinded, standardized manner. A random effects model was used to pool odds ratios (ORs) [with 95% confidence interval (CI)] for the primary analysis, the risk of kidney failure. Subgroup analyses were performed for clinical and laboratory features that predicted renal outcomes. Heterogeneity was assessed by I2. Six records involving 709 patients were included in the meta-analysis. Biopsy-proven aPL-N was found in 238/832 (28.6%) patients. Acute kidney injury (AKI) was present at diagnosis in 20/65 (30.8%), while 73/233 (31.3%) patients with aPL-N developed chronic kidney disease (CKD)/end-stage kidney disease (ESKD) at follow-up. aPL-N was associated with an increased risk of CKD/ESKD [OR 6.89 (95% CI 2.42-19.58)] and AKI [OR 2.97 (95% CI 1-4-6.29)]. Arterial hypertension and positivity for lupus anticoagulant, anti-cardiolipin antibodies and anti-β2 glycoprotein I antibodies were associated with an increased risk of developing aPL-N [OR 3.7 (95% CI 1.9-7.23), OR 4.01 (95% CI 1.88-8.53), OR 2.35 (95% CI 1.31-4.21) and OR 19.2 (95% CI 2.91-125.75), respectively]. aPL-N is associated with poor renal outcomes. High blood pressure and aPL positivity have been identified as predictors of adverse renal outcomes. This up-to-date knowledge on renal outcomes and predictors of renal outcomes in aPL-N enables a personalized follow-up and therapeutic approach.

Comparison of Clinical and Laboratory Characteristics in Lupus Nephritis vs. Non-Lupus Nephritis Patients-A Comprehensive Retrospective Analysis Based on 921 Patients.

Background: Lupus nephritis (LN) is an inflammation of the kidneys that is related to systemic lupus erythematosus (SLE). This study aimed to evaluate the differences in clinical and laboratory characteristics between LN and non-LN SLE patients. Methods: We conducted a retrospective analysis of medical records collected from SLE patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022. All patients met the 2019 European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for SLE. Results: Among 921 SLE patients, LN was documented in 331 (35.94%). LN patients were younger at SLE diagnosis (29 vs. 37 years; p < 0.001) and had a male proportion that was 2.09 times higher than the non-LN group (16.62% vs. 7.97%; p < 0.001). They were more often diagnosed with serositis and hematological or neurological involvement (p < 0.001 for all). Hypertension and hypercholesterolemia occurred more frequently in these patients (p < 0.001 for both). LN patients exhibited a higher frequency of anti-dsDNA, anti-histone, and anti-nucleosome antibodies (p < 0.001 for all). Conversely, the non-LN group had a 1.24-fold (95% CI: 1.03-1.50; p = 0.021) increase in the odds ratio of having positive anti-cardiolipin IgM antibody results. LN patients were more frequently treated with immunosuppressants. The risk factors for experiencing at least three LN flares included female sex, younger age at the onset of LN or SLE, LN occurring later than SLE onset, the presence of anti-nucleosome or anti-dsDNA antibodies, and certain SLE manifestations such as myalgia, arthritis, proteinuria > 3.5 g/day, and pathological urinary casts in the urine sediment. Conclusions: LN patients differ from non-LN patients in the age of SLE diagnosis, treatment modalities, and autoantibody profile and have more frequent, severe manifestations of SLE. However, we still need more prospective studies to understand the diversity of LN and its progression in SLE patients.

Late Onset Systemic Lupus Erythematosus: Different Clinical, Serological Presentations and Damage Compared to Adult Lupus in Egypt

Abstract Background Comparing cases of adult onset and late onset systemic lupus erythematous (SLE) reveals significant differences in clinical, serological, disease activity, and damage score. Objective This study aimed to analyze clinical manifestations, laboratory data, serological markers, and prognosis of late-onset SLE (L-SLE) and for comparing with adult- onset SLE. Patients and Methods One hundred fifty individuals with SLE were included in a cross-sectional study conducted at Ain Shams University Hospital divide into Group 1: 100 cases with adult-onset (age of onset ≥ 19 years and below 50 years). Group 2: 50 Patients with L-SLE (age of onset ≥ 50 years). All patients were subjected to medical history, physical examination, disease activity measured by the SLE disease activity index (SLEDAI-2K) and a damage score. Laboratory investigations as complete blood count (CBC), serum creatinine, anticardiolipin antibodies, lupus anticoagulant, protein creatinine ratio, serum complement (C3, C4), anti-dsDNA antibody, and antinuclear antibodies (ANA). Results Mucocutaneous manifestations, frequency of hematuria, proteinuria, urinary cast, consumed C3, positive anti-dsDNA antibodies, anti-cardiolipin antibody and lupus anticoagulant titers had considerably greater rates in-group 1 compared to group 2 (P-value &amp;lt;0.05) while group 2 had significantly more musculoskeletal symptoms (P-value &amp;lt;0.05). The SLEDAI scores of the two groups were equivalent, however the damage index was greater in group 2 (P-value 0.00). Neuropsychiatric, cutaneous, renal, and skin damage were more frequent in group 1, while musculoskeletal, endocrinal, pulmonary, cardiovascular and ocular damage were more frequent in- group 2. Conclusion L-SLE is different from adult onset SLE with more frequent damage.

An update on the endocrine manifestations of antiphospholipid syndrome.

Antiphospholipid Syndrome (APS), characterized by hypercoagulability and pregnancy morbidity, poses a significant clinical challenge when involving organ systems, such as the endocrine system. APS can directly and indirectly influence the anterior and posterior lobes of the pituitary gland. The thyroid gland exhibits involvement, especially in patients with positive anticardiolipin antibodies, yet the clinical significance of the relationship with APS remains elusive. The pancreas, often overlooked, manifests in diverse ways, from pancreatitis to implications in diabetes. Adrenal insufficiency emerges as a common endocrine manifestation of APS, with adrenal hemorrhage or infarction being a presenting manifestation. Adrenal gland involvement has also been reported in the context of catastrophic APS. Pregnancy complications and infertility might be effects of APS on the female ovaries, while testicular torsion and decreased sperm concentration and total sperm count have been reported as rare effects of APS on male testes.

Risk factors for thrombotic events in systemic lupus erythematosus patients with antiphospholipid antibodies: insights from morphometric measurements of carotid arteries.

To identify the correlation between thrombosis and atherosclerosis in systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPLs) (SLE/aPLs) through high-resolution magnetic resonance imaging (HR-MRI) of the carotid artery. A single-center, cross-sectional study was conducted. We collected consecutive patients with SLE/aPLs and healthy controls who underwent carotid HR-MRI examinations. The morphometric characteristics of the common carotid artery (CCA), internal carotid artery (ICA), external carotid artery (ECA), and carotid bulb (Sinus) were measured, and the differences in morphometric parameters between different groups were analyzed. A total of 144 carotid arteries were analyzed. Compared with the control group, the wall area, wall thickness (WT and WTmax), and normalized wall index of CCA, ICA, ECA, and Sinus were increased in patients with SLE/aPLs, and the total vascular area (TVA) of CCA, ICA, and Sinus, and the bifurcation angle (BIFA) of ICA-ECA were also increased. A negative lupus anticoagulant (LAC) (with or without positive anticardiolipin antibody (aCL) or anti-β2glycoprotein antibody (aβ2GPI)) contributed to illustrating lower increased TVA and thickened vessel walls of CCA and ICA in SLE/aPLs patients without thrombotic events. Logistic regression analysis showed that WTmaxSinus and WTmaxGlobal were independent risk factors for thrombotic events in SLE/aPLs patients. The receiver operator characteristic curve showed that the cut-off value of WTmaxSinus was 2.855 mm, and WTmaxGlobal was 3.370 mm. HR-MRI ensures the complete and accurate measurement of carotid morphometric parameters. Compared with the control group, the carotid artery in patients with SLE/aPLs is mainly characterized by diffusely thickened vessel walls, and the patients with thrombotic events showed additional higher vascular area of CCA and ICA, and BIFA of ICA-ECA without significant change in lumen area. The carotid arteries of SLE/aPLs patients with thrombotic events exhibited significant vessel wall thickening in all segments except ECA compared to those without thrombotic events. LAC-negative and non-thrombotic events distinguish relatively early atherosclerosis in the carotid arteries in patients with SLE/aPLs. Patients with SLE/aPLs that possess circumscribed thickened carotid vessel walls (>3.370 mm), particularly thickened at the Sinus (>2.855 mm), may require management strategies for the risk of thrombotic events.

The association between antiphospholipid syndrome and atrial fibrillation: a single center retrospective case-control study.

Antiphospholipid syndrome (APS) is a systemic autoimmune syndrome characterized by arterial or venous thrombosis, pregnancy complications and thrombocytopenia. The aim of this study is to investigate the association between APS and atrial fibrillation (AF) among patients in Peking University People's Hospital. A single center retrospective study was conducted. Cases were hospitalized patients diagnosed with AF by a cardiologist while the control group patients did not exhibit cardiac diseases. The results of the study revealed that in multivariable logistic regression, APS, anticardiolipin antibody (aCL) positivity and anti-beta-2-glycoprotein antibody (anti-β2GPI) positivity are independent risk factors of AF. APS, aCL positivity and anti-β 2GPI positivity are statistically different between AF patients and non-AF patients. Forthcoming studies are needed to clarify the potential link between APS and AF.

Hemoptysis and Deep Vein Thrombosis in a middle aged male due to Anti Phospholipid Antibody (APLA) Syndrome’’ - A Case Report

Abstract 36 Years old male, farmer by profession from rural Tamil Nadu presented with complaints of unilateral painful leg swelling for 1 month and cough with hemoptysis for 3 weeks. Chest skiagram revealed Hampton’s hump and ultrasound venous Doppler revealed deep vein thrombosis of the left leg. His computed tomography pulmonary angiogram revealed pulmonary embolism. Due to unusual presentation work up was done and we found positive anti cardiolipin antibody, lupus anticoagulant and anti β 2 glycoprotein antibody. Patient was diagnosed as APLA and was started on oral anticoagulant.

The Influence of Hyperthyroidism on the Coagulation and on the Risk of Thrombosis.

Introduction: Apart from the well-known fact that hyperthyroidism induces multiple prothrombotic disorders, there is no consensus in clinical practice as to the impact of hyperthyroidism on the risk of thrombosis. The aim of this study was to examine the various hemostatic and immunologic parameters in patients with hyperthyroidism. Methods: Our study consists of a total of 200 patients comprised of 64 hyperthyroid patients, 68 hypothyroid patients, and 68 euthyroid controls. Patient thyroid status was determined with standard tests. Detailed hemostatic parameters and cardiolipin antibodies of each patient were determined. Results: The values of factor VIII (FVIII), the Von Willebrand factor (vWF), fibrinogen, plasminogen activator inhibitor-1 (PAI-1), and anticardiolipin antibodies of the IgM class were significantly higher in the hyperthyroid patients than in the hypothyroid patients and euthyroid controls. The rate of thromboembolic manifestations was much higher in hyperthyroid patients (6.25%) than in hypo-thyroid patients (2.9%) and euthyroid controls (1.4%). Among hyperthyroid patients with an FVIII value of ≥1.50 U/mL, thrombosis was recorded in 8.3%, while in hyperthyroid patients with FVIII value ≤ 1.50 U/mL the occurrence of thrombosis was not recorded. The incidence of atrial fibrillation (AF) was significantly higher (8.3%) in the hyperthyroid patients compared to the hypothyroid patients (1.5%) and euthyroid controls (0%). Conclusions: High levels of FVIII, vWF, fibrinogen, PAI-1, and anticardiolipin antibodies along with other hemostatic factors contribute to the presence of a hypercoaguable state in patients with hyperthyroidism. The risk of occurrence of thrombotic complications is especially pronounced in patients with a level of FVIII exceeding 150% and positive anticardiolipin antibodies of the IgM class. Patients with AF are at particularly high risk of thrombotic complications due to a hyperthyroid prothrombotic milieu.

A mysterious hematochezia in an adolescent boy: atypical presentation of childhood systemic lupus erythematosus

Thrombosis is a well-known entity in presence of antiphospholipid antibody (APLA) associated with systemic lupus erythematosus (SLE) as a hematological complication. Bleeding manifestations instead of thrombosis is hardly found in literature in presence of APLA seromarkers in SLE. Since these can range from minor bleeding like epistaxis to major life-threatening intracranial bleeding, early diagnosis and prompt treatment is essential to manage such condition. We report a 12 years old boy with no significant past history presented with hematochezia and epistaxis along with pallor requiring blood transfusion. Hematological investigations were normal except for elevated PT, aPTT and INR. Common causes of coagulopathy were ruled out. Upon suspecting systemic diseases, the investigations were carried out which revealed ANA 4+ along with high titre of dsDNA, low C3 and C4 complement and positive anti-β2 glycoprotein, anticardiolipin antibody and lupus anticoagulant. Diagnosis of SLE was made according to ACR-EULAR criteria with no renal involvement. Immunological basis was considered for coagulopathy in this child. He was started on oral prednisolone, hydroxychloroquine and methotrexate. He is now under close monitoring of the coagulation profile for titration of steroid dose. We want to create awareness about the uncommon hematological manifestation of SLE presenting as bleeding diathesis instead of thrombosis through this case report and that can be life threatening too if not treated promptly. A high index of suspicion and careful follow-up may help in preventing adverse outcome of the disease.

Severe Antiphospholipid Syndrome and Diffuse Glomerulonephritis After Adalimumab Treatment in a Patient With Ulcerative Colitis

Tumor necrosis factor alpha inhibitors (TNFi) are biological drugs used worldwide to treat various autoimmune disorders. Paradoxically, TNF‐α antagonists can also induce autoimmune diseases being systemic vasculitis, systemic lupus erythematosus, and psoriasis, the most common. We present a 22‐year‐old woman with ulcerative colitis (UC) who was started on adalimumab 40 mg subcutaneously every 2 weeks. After two doses of adalimumab, she developed gangrene of all toes and acute kidney injury requiring hemodialysis. Skin biopsy showed thrombi in the small vessels of the dermis. Renal biopsy disclosed diffuse proliferative glomerulonephritis (GN) and acute tubulointerstitial nephritis. Serologic work‐up showed positive IgG anticardiolipin (ACL) antibodies and low C3 levels. Antinuclear, anti‐dsDNA, anti‐Smith, anti‐SSA, anti‐SSB, anti‐RNP, antineutrophil cytoplasmic antibodies, ACL (IgA and IgM), and anti‐β2‐glycoprotein I (IgG, IgM, and IgA) antibodies were not elevated. Lupus anticoagulant test and cryoglobulins were negative. Adalimumab was discontinued, and she was treated with enoxaparin, intravenous (IV) methylprednisolone pulse, IV cyclophosphamide, and plasmapheresis followed by maintenance therapy with warfarin, prednisone, azathioprine, and hydroxychloroquine. She did not have further thrombotic events, and the acute kidney injury completely resolved. ACL IgG antibodies decreased to normal levels, and repeated tests were negative. After 7 years, anticoagulation and immunosuppressive drugs were discontinued. During a follow‐up of 24 months, she remained in complete clinical remission. This report highlights the occurrence of autoimmune disorders induced by TNFi. Thus, careful monitoring of adverse immune reactions to TNFi is highly recommended.

A comprehensive Bayesian analysis assessing the effectiveness of lymphocyte immunotherapy for recurrent spontaneous abortion

Abstract Recurrent spontaneous abortion (RSA) affects 2%–5% of couples worldwide and remains a subject of debate regarding the effectiveness of lymphocyte immunotherapy (LIT) due to limited retrospective studies. We conducted a comprehensive Bayesian analysis to assess the impact of LIT on RSA. Using data from the Shenzhen Maternity and Child Healthcare Hospital (2001–2020, n = 2316), a Bayesian generalized linear model with predictive projection feature selection was employed. Our analysis revealed a significant improvement in live birth rates for RSA patients undergoing LIT. Notably, LIT had a greater impact compared to the other 85 factors considered. To mitigate research bias, we conducted a Bayesian meta-analysis combining our dataset with 19 previously reported studies (1985–2021, n = 4246). Additionally, we developed an empirical model highlighting the four key factors, which are the LIT result, age, paternal blood type, and anticardiolipin antibody. Younger age (19–27), paternal blood type B, and a positive anticardiolipin antibody (IgM) were associated with better therapeutic outcomes in LIT for RSA. These findings aid clinicians in identifying suitable candidates for LIT and improving treatment outcomes.

Rare Association of Takayasu Arteritis and Antiphospholipid Syndrome with Severe Thrombocytopenia

The association of Takayasu arteritis (TA) with antiphospholipid syndrome (APS) has rarely been described in the literature. This paper reports the first documented case of TA and APS in a 24-year-old woman in Bangladesh. This patient had claudication pain in upper and lower limbs for four years and Raynaud’s phenomenon in right hand for eight months. During the course of her illness, she suddenly developed deep vein thrombosis in left superficial femoral, popliteal and posterior tibial veins. Conventional angiography revealed total occlusion of right subclavian artery and 60-70% stenosis of right common iliac artery. CT angiography of right upper limb also supported these findings along with increased wall thickening in the 2nd part of right subclavian artery and moderate narrowing of the 1st part of right axillary artery. She had persistently positive anticardiolipin antibodies in high titers, positive lupus anticoagulant (LA), prolonged activated partial thromboplastin time (APTT) and severe thrombocytopenia. We started high dose prednisolone (1mg/kg daily). Her platelet count increased rapidly. Based on review of 10 case reports, we considered the rare association of TA and APS. Both conditions should be determined promptly for the sake of early institution of the appropriate therapy. Bangabandhu Sheikh Mujib Med. Coll. J. 2023;2(1): 59-63

Coexistence of Systemic Lupus and Antiphospholipid Syndrome in a Male Patient: Atypical Case Presentation

Abstract Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) are two distinct autoimmune disorders characterized by dysregulated immune responses, organ involvement, and the presence of autoantibodies in the bloodstream. Primary Antiphospholipid Syndrome (APS) can occur among individuals who are generally healthy and have no history of an underlying systemic autoimmune illness. In contrast, several other systemic autoimmune diseases, including SLE, have the potential to cause secondary Antiphospholipid Syndrome (APS). While Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) affect women of childbearing age, there are only a few reported case studies documenting the presence of SLE or APS individually in male patients. However, the coexistence of both conditions in a male patient is extremely rare, with only a few documented cases reports available to date. This case report presents a unique occurrence of concurrent SLE and APS in a forty-year-old male patient, highlighting the rarity of such a presentation. The patient presented with per rectal and mucosal bleeding and had been on warfarin therapy for 16 years due to a history of recurrent Deep Vein Thrombosis (DVT), but was not evaluated further. Immune-related laboratory results revealed positive antinuclear antibodies, anti-dsDNA antibodies, anticardiolipin antibodies, lupus anticoagulants, and direct Coombs test. Concurrent SLE and APS were diagnosed by meeting the classification criteria for both diseases. This report adds to the medical literature and emphasizes the significance of considering concurrent SLE and APS as a potential diagnosis in males. Further research is needed to enhance our understanding of the underlying mechanisms and the best approaches for managing this rare coexistence.

Adrenal Hemorrhage in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report and Literature Review.

Antiphospholipid syndrome (APS) is an autoimmune disorder while adrenal hemorrhage could be its rare complication. Herein, we report the case of a 32-year-old unmarried woman with a history of systemic lupus erythematosus (SLE) who was hospitalized after complaints of upper abdominal pain, limb weakness, and loss of appetite for 2 weeks. Laboratory examination revealed hyponatremia, low plasma cortisol levels, increased adrenocorticotropic hormone levels, and a positive anticardiolipin antibody status. Furthermore, computed tomography (CT) revealed the presence of bilateral adrenal masses. Ultimately, based on dynamic changes in CT images, these masses were diagnosed as adrenal hemorrhage owing to APS. A computer-assisted literature search was conducted to identify cases of primary adrenal insufficiency associated with APS and/or SLE. The clinical features, laboratory examination, treatments, and outcomes of these cases were summarized. Our findings emphasize the importance of screening for adrenal insufficiency in patients with SLE or APS who present with abdominal complaints, asthenia, and hyponatremia. It is also recommended to test for APS all patients with adrenal hemorrhage.

The effect of anti-phospholipid syndrome on pregnancy outcomes in patients with habitual abortus

Objective: The aim of this study was to research the pregnancy outcomes of women with anti-beta2 glycoprotein 1 and anti-cardiolipin antibody positivity and to determine the association with pregnancy morbidity.&#x0D; Materials and methods: This retrospective study contained pregnant women with anti-beta2 glycoprotein 1 and anti-cardiolipin antibody positivity and a control group without these antibodies. Totally 190 serum samples sent from Obstetrics and Gynecology clinics between January 2019 and January 2023 were analyzed in the medical microbiology laboratory of xx.&#x0D; Results: In a patient population separated into antibody-positive and antibody-negative groups, the gravida was found to be 3.8±0.1 and 3.5±0.3 respectively (p=0.333). Parity was 1.1±0.1 and 0.8±0.1 (p=0.071), abortion rates were 2.3±0.1 and 2.5±0.2 (p=0.659), and gestational age was 35.7±0.8 and 34±1.5 (p=0.047). Intrauterine fetal death was found to be higher in the antibody-positive group compared to the antibody-negative group (p=0.03). There was no noteworthy distinction noted between the two groups regarding additional pregnancy complications such as intrauterine growth restriction, oligohydramnios, gestational diabetes, and gestational hypertension (respectively p=0.623, 0.074, 0.312, 0.626). However, smoking was significantly higher in the antibody-positive group (p=0.049).&#x0D; Conclusion: Antiphospholipid syndrome adversely affects pregnancy outcomes. During the initial visit, a thorough patient history should be obtained, and in pregnant women with a history of poor obstetric outcomes or habitual abortions, this syndrome should be considered.

The Impact Of Hepatitis B Infection On The Development Of Anti-Cardiolipin Antibodies In Najaf Province

Background: Hepatitis B Virus (HBV) is a partly double-stranded, hepatotropic, non-cytotoxic Hepadnaviridae virus. HBV is linked to many autoimmune diseases, including Rheumatoid Arthritis(RA), Cryoglobulinemia, and Antiphospholipid Syndrome(APS). Anticardiolipin antibodies (ACL) are a type of anti-phospholipid antibodies(APA) that target cardiolipin molecules. They're more common in systemic lupus erythematosus (SLE) and APS. APA may be caused by viral infections such as HBV. Aim of the study: Determination of Anticardiolipin antibody positivity among hepatitis B virus-infected patients in Najaf governorate. material, and methods: a cross-sectional study was conducted between September 2022 to March 2023 in Najaf, a non-random sampling was depended, the sera of 113 HBV-infected patients were collected from the main health facilities in Najaf, and all had no other possible disease that is associated with APA, then it was tested for HBS antigen, total HBc antibodies, IgM HBc antibodies, ACL antibodies by ELISA technique. SPSS version 26 was used to perform the statistical analysis processes. Results: anti-cardiolipin antibodies(ACL) prevalence was 10.6%, The type of HBV infection and ACL production were significantly correlated (p&lt;0.05), males had a higher percentage of ACL autoantibodies and middle age patients presented with the highest rate of ACL antibodies, however, there was no statistically significant relationship between ACL and age or gender. In Conclusion: hepatitis B virus (HBV) is one of the main viruses that lead to the production of ACL especially in middle-aged people males, the chronic type is more prevalent for ACL than the acute type.

Analysis of incidence and clinical characteristics of osteonecrosis of femoral head in patients with systemic lupus erythematosus treated with glucocorticoid: A descriptive study based on a prospective cohort

To describe the disease characteristics of osteonecrosis of the femoral head (ONFH) in patients with systemic lupus erythematosus (SLE) who experiencing prolonged glucocorticoid (GC) exposure. Between January 2016 and June 2019, 449 SLE patients meeting the criteria were recruited from multiple centers. Hip MRI examinations were performed during screening and regular follow-up to determine the occurrence of ONFH. The cohort was divided into ONFH and non-ONFH groups, and the differences in demographic baseline characteristics, general clinical characteristics, GC medication information, combined medication, and hip clinical features were compared and comprehensively described. The age at SLE diagnosis was 29.8 (23.2, 40.9) years, with 93.1% (418 cases) being female. The duration of GC exposure was 5.3 (2.0, 10.5) years, and the cumulative incidence of SLE-ONFH was 9.1%. Significant differences ( P<0.05) between ONFH and non-ONFH groups were observed in the following clinical characteristics: ① Demographic baseline characteristics: ONFH group had a higher proportion of patients with body mass index (BMI)<20 kg/m 2 compared to non-ONFH group. ② General clinical characteristics: ONFH group showed a higher proportion of patients with cutaneous and renal manifestations, positive antiphospholipid antibodies (aPLs) and anticardiolipin antibodies, severe SLE patients [baseline SLE Disease Activity Index 2000 (SLEDAI-2K) score ≥15], and secondary hypertension. Fasting blood glucose in ONFH group was also higher. ③ GC medication information: ONFH group had higher initial intravenous GC exposure rates, duration, cumulative doses, higher cumulative GC doses in the first month and the first 3 months, higher average daily doses in the first 3 months, and higher proportions of average daily doses ≥15.0 mg/d and ≥30.0 mg/d, as well as higher full-course average daily doses and proportion of full-course daily doses ≥30.0 mg/d compared to non-ONFH group. ④ Combined medications: ONFH group had a significantly higher rate of antiplatelet drug use than non-ONFH group. ⑤ Hip clinical features: ONFH group had a higher proportion of hip discomfort or pain and a higher incidence of hip joint effusion before MRI screening than non-ONFH group. The incidence of ONFH after GC exposure in China's SLE population remains high (9.1%), with short-term (first 3 months), medium-to-high dose (average daily dose ≥15 mg/d) GC being closely associated with ONFH. Severe SLE, low BMI, certain clinical phenotypes, positive aPLs, and secondary hypertension may also be related to ONFH.

Changes in Clinical Manifestations and Course of Systemic Lupus Erythematosus and Secondary Antiphospholipid Syndrome over Three Decades.

Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome (APS), which potentially results in a more severe disease course and reduced life expectancy. Since the therapeutic guidelines have been refined in the last 15 years, we assumed that the diseases course has become more favorable. In order to shed light on these achievements, we compared the data of SLE patients diagnosed before and since 2004. In our retrospective study, we assessed a wide spectrum of clinical and laboratory data of 554 SLE patients who received regular follow-up care and therapy at our autoimmune center. Among these patients, 247 had antiphospholipid antibodies (APAs) without clinical signs of APS, and 113 had definitive APS. In the APS group, among patients diagnosed since 2004, deep vein thrombosis (p = 0.049) and lupus anticoagulant positivity (p = 0.045) were more frequent, while acute myocardial infarction was less frequent (p = 0.021) compared with patients diagnosed before 2004. Among the APA positive patients without definitive APS, anti-cardiolipin antibody positivity (p = 0.024) and development of chronic renal failure (p = 0.005) decreased in patients diagnosed since 2004. Our study demonstrates that the disease course has changed in recent years; however, in the presence of APS, we have to expect repeated thrombotic events despite adequate anticoagulant therapy.

Reduced circulating Tregs and positive pANCA were robustly associated with the occurrence of antiphospholipid syndrome in patients with systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a typical chronic immune disorder with clinical heterogeneity. The systemic abnormal immune response not only challenges the diagnosis and treatment of the disease itself but also the secondary antiphospholipid syndrome (APS), characterized by recurrent arterial or venous thrombosis, recurrent spontaneous abortion, or stillbirth. Clinical interest has primarily focused on primary APS's pathological and clinical features. However, differences in clinical features and laboratory indicators between SLE with or without APS are still lacking, especially differences between circulating lymphocytes, which are critical in the pathogenesis of SLE and its complications. In this retrospective study, we collected and analyzed clinical characteristics, general laboratory indicators, immunological indicators, and circulating lymphocyte subsets of SLE with or without APS. Systemic lupus erythematosus with APS (SLE-APS) had elevated SLEDAI scores, hospitalization costs and time, and frequencies of central nervous system symptoms and spontaneous abortion compared with those without APS. SLE-APS had higher positive anti-Cardiolipin antibodies, anti-β2 Glycoprotein 1 antibodies, and perinuclear antineutrophil cytoplasmic antibody (pANCA) than none-APS patients. Compared with healthy controls (HCs), the circulating lymphocyte subsets were altered to some extent in all patients, especially in patients with SLE-APS. Reduced Tregs and positive pANCA were independent risk factors for SLE secondary APS. The present study revealed a robust association between APS secondary to SLE and reduced Tregs and positive pANCA, which provides essential information regarding the diagnosis and therapeutic possibilities of APS secondary to SLE.

New-onset giant cell arteritis with lower ESR and CRP level carries a similar ischemic risk to other forms of the disease but has an excellent late prognosis: a case-control study.

Biopsy-proven giant cell arteritis (GCA) occasionally presents without acute-phase reaction. In this setting, GCA may be initially overlooked and glucocorticoid treatment unduly delayed, potentially increasing ischemic risk. From an inception cohort of patients with newly diagnosed, biopsy-verified GCA, we retrieved all cases without elevation of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level before starting glucocorticoid treatment. We compared the baseline features and outcomes of these patients and two additional patients recruited after GCA diagnosis with those of 42 randomly selected patients with high baseline ESR and CRP. Of 396 patients, 14 (3.5%) had lower baseline values of both ESR and CRP. Lower baseline ESR and CRP were associated with fewer American College of Rheumatology criteria met (p < 0.001, 95% CI -1.1; -0.9), and less jaw claudication (p = 0.06, 95% CI 0.8; 44.9), but similar rates of permanent blindness (p = 1.0). Patients with lower ESR and CRP also showed obvious differences regarding mean blood cell counts and mean hemoglobin level, but also less anti-cardiolipin antibody positivity (p = 0.04, 95% CI 0.8; ∞) and hepatic cholestasis (p = 0.03, 95% CI 1.0; 422). Patients with lower ESR and CRP had fewer GCA relapses (p = 0.03, 95% CI -1.1; -0.1), fewer glucocorticoid-induced complications (p = 0.01, 95% CI -2.0; -0.1), and successfully stopped glucocorticoids sooner than the other patients (18.3months vs 34months in average, p = 0.02, 95% CI -27;-0.9). Biopsy-proven GCA presenting with lower ESR and CRP is not an exceptional occurrence. It is clinically less typical but carries similar ischemic risk to other forms of the disease. Conversely, the late GCA prognosis of these patients is excellent.