Audiologic evaluation in systemic lupus erythematosus patients and impact of hydroxychloroquine therapy

Aim of the work: To evaluate the hearing function in systemic lupus erythematosus (SLE) patients and assess the impact of chronic use of hydroxychloroquine (HCQ). Patients and methods: This study was conducted on 48 SLE patients and 12 matched controls. The SLE disease activity index (SLEDAI) was assessed. Otological manifestations and extended high frequency audiometry were assessed. SLE patients were divided into those receiving HCQ (n = 36) and those not (n = 12). Results: The mean age of the patients was 39.5 ± 9.7 years, 40 females and 8 males, median disease duration was 7 (1.5–20) years. The median (interquartile range) SLEDAI was 4 (2.75). The median daily dose of those receiving HCQ was 400 mg. There was a significant difference regarding patients reported otological manifestations such as tinnitus (p = 0.02), vertigo (p = 0.002) and hearing impairment (p = 0.04) compared to controls but not for deafness or ear buzzing in one or both ears. There was a significant decrease in the hearing threshold frequencies between patients and controls (p < 0.001). Those receiving HCQ showed more hearing impairment at frequency 9000 and 20,000 Hz than those not (p = 0.004, p < 0.001 respectively). The otological manifestations were comparable between those receiving HCQ and those not. There was no difference between those receiving 400 mg/day (n = 31) HCQ and those receiving 200 mg/day (n = 5) Conclusion: Otological symptoms and sensorineural hearing loss are frequent among SLE patients. Chronic administration of HCQ may have an ototoxic effect.     

Systemic lupus erythematosus and antineutrophil cytoplasmic antibodies-associated vasculitis overlap in an elderly woman: A case-based literature review

BackgroundThe overlap of systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA) associated-vasculitis (AAV) is a rare entity. Aim of the work. To report a rare case of overlap SLE and AAV complicated by small bowel perforations and nephritis.Case presentationAn 81-years-old Chinese woman presented with a two-weeks history of progressive bilateral lower limb weakness and dysuria. An incidental uterine mass was found, and a total hysterectomy was performed with extensive small bowel adhesion and multiple enteric perforations discovered intra-operatively. SLE was diagnosed based on the presence of cutaneous vasculitis, positive antinuclear antibody, anti-double stranded deoxyribonucleic acid, consumed complements, thrombocytopenia, nephritis, and pleural effusion. Positive perinuclear-ANCA and histological findings of the resected small bowel led to evidence of co-existing AAV. Hence, these findings have led to a diagnosis of overlap SLE and microscopic polyangiitis (MPA). The patient received daily hydroxychloroquine (200 mg), azathioprine (50 mg) followed by intravenous (IV) hydrocortisone (200 mg/8 h) and cyclophosphamide (750 mg/m²). The patient’s condition deteriorated with respiratory failure and hypotension and was eventually intubated and ventilated. IV immunoglobulin (4 mg/kg/day) was given for 3 days with resolution of the vasculitic lesions. The renal function rapidly declined with hemodynamic and clinical deterioration and the patient died.ConclusionThis case demonstrates the diagnostic conundrum and complexity in the management of a late presentation of an overlap syndrome with rare life-threatening complications. To our knowledge, this is the first case diagnosed and managed in Malaysia and the oldest patient diagnosed with overlap SLE/AAV in the literature.     

Low estimated glomerular filtration rate is an independent risk factor for higher hydroxychloroquine concentration

Background

The aim of this study was to analyze the relationship of the estimated glomerular filtration rate (eGFR) to hydroxychloroquine (HCQ) blood concentrations in systemic lupus erythematosus (SLE) patients.

Method

Patients with SLE who had been taking HCQ for more than 12 months were recruited. All subjects gave written informed consent. Various clinical characteristics and laboratory values were examined. The blood concentration of HCQ was measured by high-performance liquid chromatography, and the relationship of eGFR to HCQ blood concentration was mainly investigated.

Result

In total, 115 patients with SLE receiving long-term HCQ therapy were included in the study. The median concentration of HCQ was 1096 ng/ml (range 116–8240 ng/ml). The eGFR was strongly associated with blood concentration of HCQ (P = 0.011, P < 0.05), when adjusted for age, sex, body mass index (BMI), weight-adjusted dose, prednisone use and immunosuppressive drug use. No statistically significant association were found between age, duration, BMI, weight-adjusted HCQ dose, corticosteroid use, immunosuppressant use and blood concentrations of HCQ.

Conclusion

We provided novel evidence that impaired renal function influenced the blood concentration of HCQ. Patients with low eGFR need to adjust the HCQ dosage according to the monitoring results of HCQ blood concentrations.

A 13-year-old systemic lupus erythematosus girl initially presenting with Raccoon eyes and neuropsychiatric manifestations

Background and aim of the workSystemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Although eyes are seldom affected, this case report presents a 13-year-old Iranian girl with Raccoon eyes and neuropsychiatric manifestations as an initial presentation of SLE.Case presentationThe patient was admitted to the pediatric rheumatology inpatient, Mofid Children’s Hospital, Tehran with swelling and ecchymosis around the eyes (Racoon eyes) as well as anorexia, abdominal pain, weight loss, mood disorders and hallucinations. Complete blood count showed normocytic, normochromic anemia, leukopenia, lymphopenia and thrombocytopenia. The C-reactive protein, erythrocyte sedimentation rate, liver and renal functional tests, and urine analysis were normal. The complement levels decreased, antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA) tests were positive. Bilateral pleural effusion and mild pericardial effusion were seen. Bone marrow aspiration showed mild hypocellularity without any evidence of malignancy. The diagnosis of neuropsychiatric SLE (NP-SLE) was held. She was treated by the pulse methyl prednisolone (30 mg/kg/d) and intravenous cyclophosphamide (500 mg/m²), oral prednisolone and hydroxychloroquine. Her appetite improved while hallucination and aggressive behavior decreased. Peri-orbital swelling and ecchymosis decreased. After one year, her appetite became normal; mood disorders, panic, phobic attacks and hallucinations were completely remedied. Swelling and ecchymosis around the eyes were eliminated. Oral prednisolone 10 mgday and hydroxychloroquine (5 mg/kg/day) were continued.ConclusionSLE may present with Raccoon eyes. Rapid detection and treatment of the disease based on clinical symptoms is critical for these patients. Prednisolone and cyclophosphamide are the best choice for treatment of the disease in children.     

Acute acalculous cholecystitis as the initial manifestation of systemic lupus erythematous: A case report

Rationale:Acute acalculous cholecystitis (AAC) is an extremely rare manifestation of systemic lupus erythematous (SLE). In previous reports, most of the patients were already diagnosed cases of SLE upon confirmation of AAC.Patient concerns:A 24-year-old female who initially presented with fever and acute right upper quadrant abdominal pain. She had no medical history.Diagnoses:Abdominal ultrasonography and computed tomography (CT) showed gallbladder thickening with pericholecystic edema without gallstones or sludge, demonstrating acalculous cholecystitis. She revealed discoid rash on the both shin. Laboratory tests revealed pancytopenia. The titer of antinuclear antibody (ANA) was 1:1280. Anti-dsDNA antibody, anti-phospholipid antibody, anti-Sm antibody test, and proteinuria in 24 hours were positive. Both C3 and C4 were low. Echocardiography and chest CT showed pericardial effusion and pleural effusion. Using the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, the score was 31. We thought AAC of this case that was one of the initial manifestations of SLE.Interventions:The patient was treated with high-dose prednisolone (1 mg/kg) and hydroxychloroquine 400 mg.Outcomes:After 4 days of administration of high-dose corticosteroid therapy, symptoms rapidly improved. After 35 days of the treatment, her symptoms and disease activity of SLE were markedly improved.Lessons:Although AAC being the initial manifestation of SLE is very rare, prompt diagnosis and management with corticosteroids precluded surgical intervention. Physicians need to be cognizant of AAC as a disease flare and as a rare initial manifestation of SLE.     

Life threatening macrophage activation syndrome as the initial presentation of systemic lupus erythematosus: A case report and review of the literature

BackgroundMacrophage activation syndrome (MAS) is characterized by activation of hemophagocytic cells and is a very rare initial manifestation of systemic lupus erythematosus (SLE).Aim of the workTo describe the features of a challenging case of SLE initially presented with MAS.Case reportA previously healthy 20-year-old female was referred to the Rheumatology clinic with a 25-days’ history of persistent fever, general fatigue, and significant weight loss. Clinical examination revealed a high-grade fever (41 °C), significant hair loss and general malaise. Laboratory investigations revealed leucopenia (1.5 × 10⁹/L), neutropenia (0.7 × 10⁹/L), lymphopenia (0.6 × 10⁹/L), normocytic normochromic anemia (9 g/dL), thrombocytopenia (95 × 109/L), elevated liver enzymes, hyperbilirubinemia (2.6 mg/dl); hypertriglyceridemia (306 mg/dL), hyperferritinemia (16000 ng/mL), elevated D-Dimer (4.7 mg/L), elevated lactate dehydrogenase (1379 U/L) and direct and indirect Coombs' tests were positive. Erythrocyte sedimentation rate was 28 mm/1st h. Immunological screening was positive for antinuclear antibody (ANA) and anti-double stranded deoxyribonucleic acid (anti-dsDNA). Serum C3 and C4 complement factors were low and C reactive protein was negative. Soluble CD25 was elevated (9800 pg/ml). Bone marrow biopsy showed hypocellular bone marrow with normal megakaryocytes and erythroid series with evidence of hemophagocytosis. A diagnosis of SLE initially presented with MAS was made. The patient received intravenous methylprednisolone for 5 consecutive days followed by prednisolone and cyclosporine A and intravenous immunoglobulin. The condition deteriorated with progressive coagulopathy, thrombocytopenia and hyperferritinemia. Subsequently, the conscious level of the patient deteriorated, multiorgan failure developed and the patient died.ConclusionMAS is a serious condition that may be the first presentation of SLE.     

Life-threatening onset of systemic lupus erythematosus coincides with Kikuchi disease in a Croatian patient

BackgroundKikuchi disease (KD) or histiocytic necrotizing lymphadenitis is a benign self-limited extremely-rare disorder of unkown etiology. It is characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. The recognition of KD is crucial as it can be mistaken for or be associated with systemic lupus erythematosus (SLE).Case reportWe present a 29-year-old Croatian female admitted to the Hematology and Oncology Department, General Hospital Dr. Josip Benčević, Croatia in a life threatening condition. She was feverish (40 °C) with chills and weight loss. On clinical examination the patient had bilaterally enlarged cervical, axillary and inguinal lymph nodes with sizes up to 3 cm. Our differential diagnosis was SLE, lymphoma, sarcoidosis, Still’s disease, hemophagocytic syndrome and KD. An extensive workup was done to confirm one of these diagnoses. Methylprednisolone 100 mg iv (1.5 mg/kg) was initiated for 5 days and as the patient’s condition was severe, steroids were maintained. Lymph node biopsy histopathology was compatible with KD. Antinuclear antibody and anti-double-stranded-DNA were positive. The patient fulfilled the classification criteria for SLE. A diagnosis of SLE associated with KD was held. On CT scan there was bilateral pleural effusion and ascites. Brain MRI was compatable with lupus cerebritis. On steroids plus hydroxychloroquine the patient remarkably improved and remained in remission after 3 months.ConclusionPrompt diagnosis and treatment with steroids may save the life of SLE patients with KD and leads to a favorable outcome. Raising the awareness towards this possibly serious association is important.     

HCQ prophylaxis in COVID-19 did not show any QTc prolongation in Healthcare workers

BackgroundHCQ is a commonly recommended drug for the prophylaxis of COVID-19. One of its rare side-effect includes QTc prolongation.MethodsThis was a prospective, cross sectional and observational study conducted on Hydroxychloroquine (HCQ) among Healthcare Workers (HCWs) at Max Super Speciality Hospital, Saket, New Delhi, India. A 3-lead ECG (only limb leads, it does not require chest leads) was performed. The QTc cut offs were pre decided, QTC < 470 ms for males and <480 ms for females was considered within the normal limits and anything above this was regarded as QTc prolongation.ResultsThere were 274 HCWs enrolled into the study, including 175 males and 99 females. Majority of the HCWs were young and had a mean age of 32.19 ± 9.29 years. Out of these, 218 were taking HCQ as per the Indian Council of Medical Research (ICMR) guidelines. The median cumulative dose being taken was 1600 mg and the median QTc of these participants was 390 ms in males and 391.5 ms in females. Subsequently, 33 participants were followed-up and found to have a median QTc of 389 ms and a cumulative dose of HCQ as 2000 mg.ConclusionIn conclusion, ours is a first study in the middle of the pandemic which showed that HCQ prophylaxis in young HCWs without comorbidities did not show any QTc prolongation.     

Systemic lupus erythematosus presenting as hyponatremia-associated rhabdomyolysis: A case report

Rationale:Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organs and causes various clinical manifestations. Cases of rhabdomyolysis as the initial presentation of SLE are rare, and there are no reported cases of SLE presenting hyponatremia-associated rhabdomyolysis as the first manifestation. Herein, we report a case of SLE with lupus nephritis in a patient with acute hyponatremia-associated rhabdomyolysis.Patient concerns:A 44-year-old woman was admitted with complaints of altered consciousness, myalgia, and red-brownish urine that first appeared three days prior. Peripheral blood tests revealed elevated creatine kinase (19,013 IU/L) and myoglobin (5099 U/L) levels and severe hyponatremia (111 mEq/L) with no azotemia. Urinalysis showed nephritic sediments.Diagnosis:Whole-body bone scintigraphy showed increased uptake of radiotracer in the both upper and lower extremities. Serological evaluation revealed the presence of anti-nuclear (speckled pattern, 1:640), anti-double stranded DNA, and anti-Smith antibodies and absence of anti-Jo-1 antibody. A kidney biopsy demonstrated mesangial proliferative (class II) lupus nephritis.Interventions:Fluid therapy, including intravenous administration of 3% NaCl, was initiated. After three consecutive days of intravenous methylprednisolone (1 g/d), oral prednisolone (1 mg/kg/d), mycophenolate mofetil, and hydroxychloroquine were administered.Outcomes:On day 28, the patient was discharged with marked resolution of SLE-associated symptoms and laboratory findings. Lupus reactivation was not present during the subsequent six-month follow-up.Lessons:Hyponatremia-associated rhabdomyolysis can be the first manifestation of SLE. Moreover, prompt fluid therapy and timely administration of immunosuppressive agents in SLE patients presenting with hyponatremia and rhabdomyolysis can significantly help alleviate disease activity and improve clinical outcomes.     

Systemic lupus erythematosus after coronavirus disease-2019 (COVID-19) infection: Case-based review

BackgroundCoronavirus disease-2019 (COVID-19) is a novel infectious disease, which presents with various clinical manifestations. There is growing evidence of an association between COVID-19 infection and autoimmune diseases. The aim of this case report was to demonstrate the association of COVID-19 infection and the development of systemic lupus erythematosus (SLE).Case presentationA 38 year old Iranian woman presented with progressive icterus, pleuritic chest pain, palpitation, dyspnea, photosensitivity and arthralgia 18-days after COVID-19 symptoms proved by a positive polymerized chain reaction (PCR). The chest and abdomen computerized tomography (CT) scan showed pericardial and pleural effusion and enlarged liver and abdominal lymph nodes. Antinuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-ds DNA) antibody and perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) were positive. She was diagnosed as SLE and was successfully treated with prednisolone 30 mg daily, hydroxychloroquine 200 mg daily and azathioprine 150 mg daily and she remarkably improved. Repeated anti-ds DNA antibody was positive. Due to nausea and abdominal discomfort, azathioprine was discontinued and replaced with mycophenolate mofetil 1500 mg daily. In the article, similar cases were presented; the mean interval between COVID symptoms and SLE presentations was 24.86 days. Pulmonary and renal involvements were the most common presentations of SLE triggered by COVID-19. The most frequently reported autoantibody was ANAConclusionIt is necessary to be aware of the development of lupus disease in COVID-19 infected patients, because prompt diagnosis and treatment is very important to improve their outcome.     

Efficacy and safety of hydroxychloroquine in healthcare professionals with mild SARS-CoV-2 infection: Prospective,non-randomized trial

ObjectivesTo assess the efficacy and safety of hydroxychloroquine (HCQ) compared with no treatment in healthcare workers with mild SARS-CoV-2 infection.MethodsProspective, non-randomized study. All health professionals with confirmed COVID-19 between April 7 and May 6, 2020, non-requiring initial hospitalization were asked to participate. Patients who accepted treatment were given HCQ for five days (loading dose of 400 mg q12 h the first day followed by200 mg q12 h). Control group included patients with contraindications for HCQ or who rejected treatment. Study outcomes were negative conversion and viral dynamics of SARS-CoV-2, symptoms duration and disease progression.ResultOverall, 142 patients were enrolled: 87 in treatment group and 55 in control group. The median age was 37 years and 75% were female, with few comorbidities. There were no significant differences in time to negative conversion of PCR between both groups. The only significant difference in the probability of negative conversion of PCR was observed at day 21 (18.7%, 95%CI 2.0–35.4). The decrease of SARS-CoV-2 viral load during follow-up was similar in both groups. A non significant reduction in duration of some symptoms in HCQ group was observed. Two patients with HCQ and 4 without treatment developed pneumonia. No patients required admission to the Intensive Care Unit or died. About 50% of patients presented mild side effects of HCQ, mainly diarrhea.ConclusionsOur study failed to show a substantial benefit of HCQ in viral dynamics and in resolution of clinical symptoms in health care workers with mild COVID-19.     

Frequency,characteristics and outcome of corona virus disease 2019 (COVID-19) infection in Iranian patients with rheumatic diseases

Aim of the workTo investigate the frequency, clinical characteristics and outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in rheumatic diseases patients.Patients and methodsOne thousand patients with rheumatic diseases including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), psoriatic arthritis (PsA), axial spondyloarthritis (SpA), systemic sclerosis (SSc), Sjögren’s syndrome (SS), Behçets disease (BD), vasculitis, idiopathic inflammatory myositis (IIM), relapsing polychondritis, sarcoidosis and antiphospholipid syndrome (APS) were studied. The following data were collected: age, sex, disease diagnosis, rheumatic disease medication. Rheumatic diseases patients were divided into two groups of infected and non-infected patients with COVID-19 and collected data were compared.ResultsThe 1000 patients mean age was 43.4 ± 13 years and 84.1% were females. The main diagnosis was RA (37.1%), followed by SLE (23.8%), SpA (13.4%), SSc (12.4%), vasculitis, BD and rhupus in 2.4%, 2.3% and 2.2% respectively, SS and SSc in 0.7% each. Most patients were taking glucocorticoids (78.4%). A large majority of patients were taking at least one of the cDMARDs. 16.1% were taking biologic therapy. 221 rheumatic diseases patients with COVID-19 were identified. Of these, 38 patients (17.2%) were hospitalized and 9 patients (4.1%) died. No significant difference was observed for compared variables in patients with and without COVID-19 except for prednisolone >20 mg/d (0.64% vs 2.26%; p = 0.048).ConclusionMost rheumatic diseases do not seem to be a risk factor for developing COVID-19 infection and despite immunosuppressive therapies, there is no poorer outcome. Only, patients using prednisolone >20 mg/d are at higher risk of developing COVID-19 infection.     

Metabolic syndrome in patients with systemic lupus erythematosus from South India

ObjectivesTo find the prevalence of metabolic syndrome in systemic lupus erythematosus (SLE) compared to controls and to identify association of metabolic syndrome with SLE disease activity and damage.MethodsA total of 82 SLE and 82 healthy controls were studied. Metabolic syndrome was defined by National Cholesterol Education Program-Adult Treatment Panel III (NCEP ATP III), consensus definition for Asian Indian Adults and World Health Organisation (WHO) 1999 definition, and associations with lupus characteristics, disease activity, and damage were examined. Insulin resistance (IR) was estimated using the homeostasis model assessment for IR (HOMA-IR).ResultsMetabolic syndrome was present in 24.39% SLE and 12.19% controls (p < 0.04) by NCEP ATP III criteria; 29.26% SLE and 19.51% controls (p = 0.14) by consensus definition for Asian Indians; 18.2% SLE and 7.31% controls (p < 0.035) by WHO 1999 criteria.Hypertension and hypertriglyceridemia were more frequent in SLE than in controls. Mean body mass index, diastolic and systolic blood pressure, triglycerides, and total cholesterol were higher in SLE than in controls. HOMA-IR (median, range) was 1.31 (0.06–9.32) and 1.55 (0.01–7.92), p = 0.09 in SLE and controls, respectively. There was no association of metabolic syndrome with disease activity/damage and prednisolone use. SLE patients with metabolic syndrome had a significantly longer duration of disease compared to patients without metabolic syndrome.ConclusionSouth Indian SLE patients have higher prevalence of NCEP ATP III and WHO defined metabolic syndrome compared to healthy controls. SLE patients have an altered lipid profile, but there was no IR and no association of metabolic syndrome with disease activity or damage.     

Successful treatment with glucocorticoid for secondary Fanconi syndrome caused by sarcoidosis

A 77-year-old female with renal dysfunction, hypercalcemia, and hypercalciuria was presented. Systemic investigations including renal biopsy showed that the patient had Fanconi syndrome secondary to renal sarcoidosis. Treatment with 25 mg per day of prednisolone was initiated and her condition improved. Complication of Fanconi syndrome in patients with sarcoidosis is extremely rare. Although the pathological mechanism is still unknown, corticosteroid therapy was effective for ameliorating proteinuria, glycosuria, hypercalciuria, and aminoaciduria.     

Low-dose oral hydroxychloroquine led to impaired vision in a child with renal failure: Case report and literature review

Introduction:Hydroxychloroquine (HCQ) has received much attention in the treatment of coronavirus disease 2019 recently. However, it can cause irreversible vision loss. Few cases have been reported in pediatric patient with HCQ-related adverse reactions. Appropriate administration and early disease recognition are important for reducing the adverse drug reactions of HCQ.Patient concerns:We report a case of a 14-year-old Chinese girl who sought treatment for rapidly decreasing vision in the left eye over 3 days. The simulation results of the population pharmacokinetic model of HCQ revealed that the plasma concentration of HCQ abnormally increased before the visual acuity of the eye decreased.Diagnosis:She was diagnosed as HCQ related drug adverse reaction.Interventions:The daily dose of HCQ for this patient was adjusted from 100 mg/d to 50 mg/d.Outcomes:Follow-up for 6 months showed no more vision loss recurrence. However, the existing decreased visual acuity of the eye did not recover either.Conclusion:Although decreased visual acuity is an infrequent symptom, ophthalmologists should be aware of the possibility of HCQ concentration enrichment and consider minimizing HCQ use when a child with renal hypofunction seeks treatment for shortsightedness.     

Tofacitinib as a Steroid-Sparing Therapy in Pulmonary Sarcoidosis,an Open-Label Prospective Proof-of-Concept Study

This is a prospective, open-label, proof-of-concept study of tofacitinib, a Janus kinase inhibitor, as a steroid-sparing therapy in corticosteroid-dependent pulmonary sarcoidosis. Five patients with corticosteroid-dependent pulmonary sarcoidosis were treated with tofacitinib 5 mg twice daily. The primary endpoint was a ≥ 50% reduction in corticosteroids at week 16 with no worsening in pulmonary function or respiratory symptoms. 60% of patients (3/5) met the primary endpoint. One patient was lost to follow up prior to steroid taper, and another was withdrawn due to worsening of known neurosarcoidosis. The three patients who met the primary endpoint each tapered to ≤ 5 mg/day prednisone, respiratory symptoms improved, and spirometry remained stable. In this proof-of-concept study, the addition of a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids. JAK-inhibitors are a promising therapy for pulmonary sarcoidosis, which require further investigation in randomized trials.

Trial Registration clinicaltrials.gov NCT03793439; registered Jan 4, 2019.

     

High sensitivity C-reactive protein (hsCRP): Association with clinical subsets in systemic lupus erythematosus (SLE) patients from Western India

BackgroundHigh-sensitivity C-reactive protein (hsCRP) is a marker of systemic inflammation. hsCRP have been related to disease presence and clinical activity in systemic lupus erythematosus (SLE).AimTo understand the association between hsCRP and SLE disease manifestations and other associated immune parameters.Material & methodsOne hundred and ten SLE patients were studied and SLE disease activity was evaluated by SLE Disease Activity Index (SLEDAI). Among these 42.7% patients had lupus nephritis (LN). hsCRP and complement levels were detected by nephelometer.ResultsA total of 40/110 (36.4%) had elevated hsCRP levels. High CRP was associated with presence of infection (p < 0.001) Patients having bacterial, parasitic and viral infections had elevated levels of hsCRP. LN patients showed slightly higher hsCRP levels (29.3 ± 29.9 mg/L than non-LN group (27.3 ± 21.3 mg/L) but this difference was not significant. Patients with raised hsCRP levels showed a higher prevalence of low complement levels as compared to patients with normal CRP levels (p = 0.03).ConclusionElevated hsCRP levels were found to be associated with infections in SLE patients and low complement levels. Elevated hsCRP levels can be used as a marker of active infection in SLE patients.     

Cardiometabolic and immune factors associated with increased common carotid artery intima-media thickness and cardiovascular disease in patients with systemic lupus erythematosus

Background and aimPatients with systemic lupus erythematosus (SLE) have a higher prevalence of subclinical atherosclerosis and higher risk of cardiovascular (CV) events compared to the general population. The relative contribution of CV-, immune- and disease-related risk factors to accelerated atherogenesis in SLE is unclear.Methods and resultsFifty SLE patients with long-lasting disease (mean age 44 ± 10 years, 86% female) and 50 sex- and age-matched control subjects were studied. Common carotid artery intima-media thickness (CCA-IMT) was used as a surrogate marker of atherosclerosis. We evaluated traditional and immune- and disease-related factors, assessed multiple T-cell subsets by 10-parameter-eight-colour polychromatic flow cytometry and addressed the effect of pharmacological therapies on CCA-IMT. In SLE patients, among several cardiometabolic risk factors, only high-density lipoprotein levels (HDL) and their adenosine triphosphate-binding cassette transporter 1 (ABCA-1)-dependent cholesterol efflux capacity were markedly reduced (p < 0.01), whereas the CCA-IMT was significantly increased (p = 0.03) compared to controls. CCA-IMT correlated with systolic blood pressure, low-density lipoprotein (LDL) cholesterol and body mass index (BMI), but not with disease activity and duration. The activated CD4⁺HLA-DR⁺ and CCR5⁺ T-cell subsets were expanded in SLE patients. Patients under hydroxychloroquine (HCQ) therapy showed lower CCA-IMT (0.62 ± 0.08 vs. 0.68 ± 0.10 mm; p = 0.03) and better risk-factor profile and presented reduced circulating pro-atherogenic effector memory T-cell subsets and a parallel increased percentage of naïve T-cell subsets.ConclusionHDL represents the main metabolic parameter altered in SLE patients. The increased CCA-IMT in SLE patients may represent the net result of a process in which ‘classic’ CV risk factors give a continuous contribution, together with immunological factors (CD4⁺HLA-DR⁺ T cells) which, on the contrary, could contribute through flares of activity of various degrees over time. Patients under HCQ therapy present a modified metabolic profile, a reduced T-cell activation associated with decreased subclinical atherosclerosis.     

A case of very-late-onset systemic lupus erythematosus

Abstract

A 93-year-old woman was admitted to our hospital because of fever. Radiographic findings revealed accumulation of pleural fluid. Moreover, blood tests revealed inflammation, lymphopenia, hypocomplementemia, positive for anti-nuclear antibody, and elevated anti-DNA antibody level. Therefore, the patient was diagnosed with pleuritis associated with systemic lupus erythematosus (SLE). Administration of prednisolone 20?mg/day resulted in a marked improvement in fever, pleuritis, and laboratory findings. We report a case of very-late-onset SLE that occurred at the age of 93.