Diagnosis Of Systemic Lupus Erythematous Research Articles

Unusual presentations in systemic lupus erythematosus with concurrent IgA nephropathy lesion: a rare case report from Eastern Nepal

Introduction: Systemic lupus erythematous (SLE) is an autoimmune disease involving multiple organs. While lupus nephritis (LN) is seen in SLE, concurrent IgA nephropathy lesion is rare. Uncommon manifestations like cutaneous ulcers and orbital involvement present diagnostic challenges and this case from Nepal emphasizes careful diagnostic approach in such cases. Case presentation: A 42-year-old female presented with bilateral lower limb swelling, gum bleeding, and epistaxis. Initial evaluation revealed pancytopenia and suspected renal involvement. Renal biopsy showed IgA nephropathy lesions, but clinical and laboratory findings favored lupus nephritis. Treatment with immunosuppressive agents was initiated. Despite therapy, the patient developed cutaneous ulcers and orbital cellulitis. Decreasing anti-ds DNA levels were noted during course of treatment. Discussion: The diagnosis of lupus nephritis in the presence of IgA nephropathy lesions emphasizes the complexity of SLE diagnosis. Treatment with immunosuppressive agents targeting the underlying autoimmune process, yet development of cutaneous ulcers and orbital cellulitis highlights the importance of timely intervention in managing SLE complications. In resource-limited settings, clinicians should initiate interventions based on clinical and lab findings while awaiting detailed biopsy results. Conclusion: This case highlights diagnostic challenges in SLE and emphasizes the necessity for careful monitoring and timely intervention in managing complications. The interplay between SLE and IgA Nephropathy (IgAN) suggests that SLE may trigger or exacerbate it, complicating disease management. Further exploration is needed to enhance understanding and management of complex autoimmune disorders like SLE.

Longitudinal study of patients with discrepant results in CLIFT and a solid-phase dsDNA antibody assay: does a gold standard dsDNA assay exist?

ObjectiveAntidouble-stranded DNA (dsDNA) antibodies are essential for diagnosis and follow-up of systemic lupus erythematous (SLE). To ensure the best diagnostic approach, most healthcare laboratories opt for a combination of highly...

Prevalence of substance use disorders among patients with systemic lupus erythematous and major depressive disorder

AbstractBackgroundMajor depressive disorder (MDD) has a higher prevalence among patients with systemic lupus erythematous (SLE). Patients with SLE struggle with stressors such as social stigma and reduced employment. Furthermore, substance use disorders (SUD) are strongly associated with MDD.ObjectivesThis research investigated whether comorbid MDD among patients with SLE is associated with an increased prevalence of SUD diagnoses. Rates of various SUD diagnoses were compared among patients with SLE.MethodsThis was a retrospective comparison study of the medical record. All data were extracted from I2B2, a deidentified dataset from an academic hospital in an urban setting. Upon stratifying patients with SLE into two groups with or without MDD, chi‐squared test comparing SUD was completed. An additional analysis stratified patients with MDD into two groups with or without comorbid SLE and completed the chi‐squared test to compare SUD.ResultsPatients with SLE and comorbid MDD were more likely to have diagnoses of tobacco, alcohol, and opioid disorders, among others. The most common SUD was tobacco (prevalence 59.94%). Furthermore, the diagnosis of SLE among patients with MDD was not associated with increased rates of SUD diagnosis. However, patients diagnosed with comorbid SLE and MDD were more likely to have tobacco use and opioid use disorder diagnosis.ConclusionsThis study demonstrates a consistently higher rate of SUD diagnoses among patients diagnosed with SLE and comorbid MDD versus SLE alone. Rates of both tobacco and opioid use disorder were higher among patients with MDD and comorbid SLE when compared to MDD alone. Our study is limited by its retrospective analysis and does not include an analysis of the treatment of comorbid depression on SUD. For clinicians who take care of patients with SLE, when they have comorbid MDD, SUD should be screened.

Cardiac tamponade revealing systemic lupus in an elderly subject: a rare case report

Systemic lupus erythematous (SLE) is a chronic autoimmune disease with a polymorphic clinical presentation that occurs mainly in young women. The particularity of this case report is that pericardial tamponade as the first manifestation of SLE and its late discovery in an elderly subject are rare. Few similar cases are reported in the literature. We report the case of a 60-year-old woman admitted to the cardiology department for dyspnoea evolving for 15 days, with a sensation of chest pressure. On admission, the patient was haemodynamically unstable. Echocardiography showed abundant circumferential pericardial effusion with variations in respiratory flow. A pericardiocentesis was performed with a sample of 900 ml of blood fluid. We started a symptomatic treatment of the pericardial effusion and an etiological assessment was carried out. The diagnosis of SLE was made according to the Systemic Lupus International Collaborating Clinics (SLICC) criteria for SLE. In our case, emergency pericardial puncture showed hematic-like pericardial effusion. Using the lab results and other investigations, we were able to make a diagnosis of SLE. The diagnosis of lupus allowed a curative treatment of the etiology of the pericardial effusion, to avoid the recurrence as well as to improve the prognosis. This case is interesting because lupus is rarely the cause of a very abundant pericardial effusion, even less so in an elderly subject. Cardiac tamponade is a life-threatening condition. Therefore, pericardiocentesis should be performed as soon as possible to avoid death. It should be kept in mind that SLE can be complicated by pericardial tamponade and know how to look for it after hemodynamic stabilization of the patient.

High-sensitivity cardiac troponin I is a biomarker for increased arterial stiffness in systemic lupus erythematous women with normal kidney function.

Cardiovascular disease (CVD) is a main cause of death in patients with systemic lupus erythematous (SLE). Algorithms for cardiovascular risk stratification in general population underestimate the risk for CVD in SLE. Our study aimed to determine whether serum high-sensitivity cardiac troponin I (hs-cTnI) might help to identify SLE patients with subclinical atherosclerosis. Arterial stiffness was assessed measuring the carotid-femoral pulse wave velocity (PWV) in 68 SLE women with a normal or almost normal kidney function and in 71 controls of similar characteristics. None of the participants had a history of an overt CVD. Serum hs-cTnI level was measured using the chemiluminescence method. Factors associated with an increased PWV (iPWV) were identified and multivariate analysis was performed. When detectable, patients tended to have had higher hs-cTnI levels than controls [2.9 (2.3-4.0) vs 2.4 (2.2-4.1); p = 0.098] and were more likely to have detectable hs-cTnI [50% vs 28%, odds ratio (OR) 7.0; 95% confidence interval (CI) 0.008-0.013]. Also, patients with iPWV were more likely to have detectable hs-cTnI than those with normal PWV (OR 6.4; 95% CI 0.019-0.026). In the multivariate analysis, the age at SLE diagnosis (OR 1.24; 95% CI 1.04-1.48), systolic blood pressure (OR 1.28; 95% CI 1.10-1.48) and detectable hs-cTnI level (OR 2.04; 95% CI 1.18-3.50) were independently associated with an iPWV. The negative predictive value of having an iPWV with undetectable hs-cTnI levels was 88%. Hs-cTnI may be a useful biomarker for the identification of SLE patients with iPWV as a surrogated marker of subclinical atherosclerosis. Specifically targeted prospective studies are needed to confirm this hypothesis.

Coexistentence of Morphea and DLE in a Patient with Beta Thalassemia Leading to a Diagnosis of Systemic Lupus Erythematous

Patients with autoimmune disorders are predisposed to developing a second autoimmune condition. This can be applied to cutaneous conditions as well. Morphea or localized scleroderma is an autoimmune inflammatory and fibrosing skin disorder due to increased collagen deposition.1,2 Patients with morphea are four times likelier of having a concomitant autoimmune disease.1 There have been cases of systemic lupus erythematosus (SLE) with morphea, but the co-occurrence of discoid lupus erythematosus (DLE) with morphea has been rarely reported, and never reported in patient with Beta-thalamessmia.1,2,7 Morphea and discoid lupus erythematosus can be found within one lesion or as separate diagnoses. In this case, we describe a patient with morphea and discoid lupus erythematous in the setting of beta thalassemia leading to diagnosis of SLE.

EXT1 and NCAM1-associated membranous lupus nephritis in a cohort of patients undergoing repeat kidney biopsies.

Exostosin 1/2 (EXT1/2) and neural cell adhesion molecule 1 (NCAM1) associated membranous lupus nephritis (MLN) may represent distinct disease phenotypes with prognostic significance. We searched our archives for patients with systemic lupus erythematous (SLE) and at least two kidney biopsies demonstrating MLN. Each biopsy was stained for EXT1 and NCAM1 and scored as positive or negative. Histopathologic and clinical data were reviewed. We identified 31 patients with a clinical diagnosis of SLE and at least two kidney biopsies with MLN. A total of 28 patients (90%) showed concordant staining for EXT1 and NCAM1 in both biopsies; 8 patients (26%) were EXT1 positive and NCAM1 negative, 18 patients (58%) were EXT1 negative and NCAM1 negative and 2 patients (7%) were EXT1 negative and NCAM1 positive. A total of three patients (10%) had discordant EXT1 staining between their first and last biopsies; two patients (7%) were EXT1 positive in their first biopsy and EXT1 negative in the last biopsy and one patient (3%) was EXT1 negative in his first biopsy and EXT1 positive in the last biopsy. Compared with the EXT1-negative cohort at the time of the first biopsy, the EXT1-positive cohort had a higher average estimated glomerular filtration rate (eGFR; 141 versus 108mL/min/1.73m2; P=0.04), lower average percent global glomerulosclerosis (0.5 versus 12%; P=0.05), lower average interstitial fibrosis and tubular atrophy (2.5 versus 11.7%; P=0.06) and lower average total National Institutes of Health (NH) chronicity scores (0.75 versus 2.33; P=0.05). On long-term follow-up, the rate of change in eGFR did not significantly differ between the two groups (P=0.24). One EXT1-positive patient (12.5%) developed stage 4 chronic kidney disease (CKD) or end-stage kidney disease (ESKD) compared with four patients (20%) in the EXT-negative group and two of the three EXT1-discordant patients (P=0.38). We performed the largest retrospective repeat-biopsy study to evaluate EXT1 and NCAM1 autoantigens in MLN. Our data demonstrate that EXT1 positivity is associated with better kidney function at the time of diagnosis and raises the possibility that EXT1 status may change throughout the disease course of MLN.

Tocilizumab for massive refractory pleural effusion in an adolescent with systemic lupus erythematosus

BackgroundPleural effusion in systemic lupus erythematous (SLE) is a common symptom, and recent studies demonstrated that IL-6 has a pivotal role in its pathogenesis.Case presentationWe report a case of a 15 years old Caucasian boy with a history of persistent pleural effusion without lung involvement or fever. Microbiological and neoplastic aetiologies were previously excluded. Based on the presence of pleuritis, malar rash, reduction of C3 and C4 levels and positivity of antinuclear antibody (ANA) and anti-double stranded DNA (dsDNA), the diagnosis of juvenile SLE (JSLE) was performed. Treatment with high dose of intravenous glucocorticoids and mycophenolate mofetil was started with partial improvement of pleural effusion. Based on this and on adults SLE cases with serositis previously reported, therapy with intravenous tocilizumab (800 mg every two weeks) was started with prompt recovery of pleural effusion.ConclusionTo the best of our knowledge, this is the first case of JSLE pleuritis successfully treated with tocilizumab.

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

Thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disease and often occurs as a complication of systemic lupus erythematous (SLE). However, this probable causal relationship has not been completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a case of a 32-year-old woman with antecedents of epilepsy since she was 12 years old. The patient was admitted to the emergency room with a clinical picture of headaches, fever, paleness in the skin and mucosa, confused state, paresthesia, and transient spasticity of the extremities. The laboratory results revealed Coombs negative direct autoimmune hemolytic anaemia, severe thrombocytopenia, significant elevation of the enzyme lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film. In addition, positive antinuclear antibodies and positive anti-native DNA in titers of 1/320 and 1/160, respectively, were found. Renal function was conserved. We concluded that it was a case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response (normalization of biomarker levels, health condition) after the second session of plasmapheresis. Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions, may lead to good clinical response.

S2881 Lupus Enteritis as the Unique Manifestation of Lupus Flare - Report of 2 Cases

INTRODUCTION: Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematous (SLE) but real GI involvement is rare. Lupus enteritis refers to either vasculitis or inflammation of the bowel wall. It is present in 0.2–5.8% of patients usually in the context of high-SLE activity. The symptoms are nonspecific and can be life-threatening especially if the treatment is delayed. We present two cases of lupus enteritis as the sole manifestation of SLE flare. CASE DESCRIPTION/METHODS: Case 1. 19-year-old female with no prior SLE diagnosis was admitted with four months of persistent diarrhea, vomiting, and epigastric pain. Workup revealed Mg 0.5 mg/dl, K 2.5 mEq/l, Hb 11.2 g/dl, CRP 1.40 mg/dl, and subnephrotic proteinuria. Infectious workup was negative. CT scan showed diffuse thickening and enhancement of the small and large bowel wall. Upper and lower endoscopy with biopsy revealed nonspecific edema and chronic inflammation. Further evaluation showed low complement levels, positive ANA ( > 1:160), anti-dsDNA ( > 300 IU/ml), SSA, SSB, anti-histone Ab, and lupus anticoagulant. IV methylprednisolone was started with marked improvement, later transitioned to hydroxychloroquine, azathioprine, and prednisone with taper dose. Case 2. 49-year-old female, SLE diagnosed in 2017, no treatment for 2 years was admitted with one month of persistent vomiting and abdominal pain. Evaluated initially with upper and lower endoscopy which revealed Barret’s esophagus. Workup revealed Na 132 mEq/L, Mg 1.6 mg/dl, lymphopenia (200 k/uL), elevated CRP (0.67 mg/dl), and subnephrotic proteinuria. Negative infectious workup. Further evaluation showed ANA ( > 1:160), Anti Smith ( > 8), anti-dsDNA (4 IU/ml), SSA ( > 8), and low complement levels. CT scan with diffuse thickening and enhancement of the small bowel and entire colon and rectum. IV methylprednisolone and a short course of IV erythromycin was started with significant improvement; later transitioned to hydroxychloroquine, azathioprine, and prednisone with taper dose. Both patients did not present recurrence of symptoms during the next 3 months. DISCUSSION: Lupus enteritis is very rare as an unique feature of SLE flare. Current knowledge is scarce. CT scan is the test of choice. It usually responds to steroids. Steroid-sparing agents are required in severe-relapsing cases. Our cases highlight the difficult diagnosis of this disease. Identifying this condition is crucial to avoid complications as bowel necrosis or perforation.Figure 1.: Contrast enhanced abdomen CT scan coronal axial views revealed “target” sign that represent circumferential thickening of the intestine wall. In this case affecting both small and large intestine.Figure 2.: Contrast enhanced abdomen CT scan showing diffuse thickening of the small and large intestine wall. "Target" sign can also be appreciated here.

P0457MALE LUPUS NEPHRITES: WHAT ABOUT RISK FACTORS FOR RENAL FAILURE ? EXPERIENCE OF ONE DEPARTMENT

Abstract Background and Aims The occurrence of renal involvement during the clinical course of systemic lupus erythematous (SLE) is generally considered to be the most important factor influencing the prognosis in terms of morbidity and mortality. The factors influencing prognosis in lupus nephritis (LN) are variable in literature. The aim of our study was to analyze the clinicopathological correlations, and risk factors associated with renal failure in male patients with LN. Method We retrospectively studied all male patients with kidney biopsy-proven lupus nephritis (LN) treated in our department during the period between 1979 and 2016. Looking for predictive factors related to renal prognosis, we analyzed clinical, biological and histological data by multivariate analysis using the comparison of the survival rates by the log-rank test. Results We collected 41 native renal biopsies showing LN. Patients were aged 32.17 years (17-65 years) at the time of diagnosis of LN. Diagnosis of SLE was made according to the criteria of the American College of Rheumatology. The most common clinical presentation was nephrotic syndrome (61%), and the most frequent pathological finding in sediment was proteinuria (85%). At the time of diagnosis, 17% of patients had hypertension and 39 % of patients had an eGFR under than 60 ml/min/1.73 m2. LN was of class I, II, III, IV and V in 7.5%, 12.5%, 27.5%, 47.5% and 41.5% of the cases, respectively. Fourteen patients developed end-stage renal failure. The presence of hematuria, renal failure at the time of diagnosis, nephrotic syndrome, low complement, proliferative glomerulonephritis (class IV), high activity index score of LN and thrombotic microangiopathy were significantly associated with poor renal prognosis with (p=0.0053), (p=0.0002), (p=0.0186), (p=0.0287), (p=0.0005), (p=0.058), (p=0.0117), respectively. Conclusion Renal failure at the time of diagnosis and active proliferative lesions should be diagnosed and treated as soon as possible because they influence the renal prognosis in lupus man.

TTP AS INITIAL PRESENTATION FOR AN 82-YEAR-OLD FEMALE WITH SYSTEMIC LUPUS

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Thrombotic Thrombocytopenic Purpura (TTP) can be idiopathic or acquired. Most common cause for TTP is illegal drugs, other causes are medications like Quinin, Gemcitabine, oxaliplatin, and quetiapine, or in rare cases it can be secondary to other diseases like connective tissue diseases mostly SLE. We are reporting a case of an 82 y/o Caucasian female who presented with TTP which was found to be secondary to underlying undiagnosed Systemic Lupus Erythematous (SLE). CASE PRESENTATION: 82 y/o F newly diagnosed schizophrenia who presented with generalized fatigue, she was found to have hemoglobin of 4.1 and platelet count of 4000 and creatinine level of 1.8. Lab work for hemolysis showed high LDH, low Haptoglobin, and schistocytes where seen on peripheral blood smear. ADAMTS 13 activity was mildly decreased at 38%. TTP diagnosis was made. She received 5 plasmapheresis rounds and she responded very well. Patient renal function was improved after the first round of plasmapheresis. Laboratory work up showed positive ANA and dsDNA with low complement level. Diagnosis of SLE was made. Patient was treated with Prednisone. Patient did very well and she was discharged home on Prednisone in a good condition. DISCUSSION: TTP is a disease of a broader spectrum diseases of Thrombotic Microangiopathy. TTP is defined by hemolysis, renal impairment, Microangiopathy hemolytic anemia, fever, and neurologic symptoms which all exist only in 5% of cases. ADAMTS 13 activity level is a helpful measurement to approach the diagnosis, but mild impairment of ADAMTS 13 level does not` exclude severe TTP. Acquired TTP incidence based on the Oklahoma TTP-HUS registry is three per one million case a year, 1-4% of these cases are related to SLE. SLE usually more common in Asians and African American females between 16-55 years of age and it is extremely rare in older than 65 years. CONCLUSIONS: • Connective tissue diseases should always be considered in the differential diagnosis when a patient presents with TTP • ADAMTS 13 activity is a helpful test for TTP diagnosis, but not always correlate with the severity of the disease Reference #1: 4. Reese JA, Muthurajah DS, Kremer Hovinga JA, et al. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features. Pediatr Blood Cancer 2013; 60:1676. Reference #2: 2. Pons-Estel GJ, Alarcón GS, Scofield L, et al. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum 2010; 39:257. Reference #3: 7. Froissart A, Buffet M, Veyradier A, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med 2012; 40:104. DISCLOSURES: No relevant relationships by Muhammad Farhan Khaliq, source=Web Response no disclosure on file for Hesham Mohamed; No relevant relationships by Dany Tager, source=Web Response

Marital status and age of systemic lupus erythematous diagnosis: the potential for differences related to sex and gender

ObjectivesChronic rheumatic diseases can challenge social and family relationships. We compared marital status in patients with systemic lupus erythematous (SLE) with their general population counterparts, stratified by sex and age...

The Predictive Value of Autoantibody Spectrum on Organ Damage in Patients With Systemic Lupus Erythematosus.

This study aims to investigate the positive detection rate and predictive value of autoantibodies, including anti-double stranded deoxyribonucleic acid (anti-dsDNA) antibodies, anti-histone antibodies (AHAs), anti-ribosomal (anti-Rib) P antibodies, anti-Smith (anti-Sm) antibodies, anti-U1 ribonucleoprotein (anti-U1RNP) antibodies, anti-Sjögren's syndrome type A antibodies and anti-Sjögren's syndrome type B antibodies, on organ damage in patients with systemic lupus erythematous (SLE). A total of 225 patients with SLE (37 males, 188 females; mean age 37.4±15.9 years; range, 7 to 80 years) were evaluated retrospectively. Statistical analysis was performed to obtain the positive detection rate of autoantibodies and to investigate the predictive value. There were statistically significant differences of positive anti-dsDNA antibodies in renal damage, photosensitization, hematological abnormalities and serositis (p<0.05) and a statistically significant difference of positive AHAs in photosensitization (p<0.05). There was statistically significant difference of positive anti-U1RNP antibodies in renal damage (p<0.05). There were also statistically significant differences of positive anti-Smith antibodies in renal damage, arthritis, photosensitization, oral ulcers, hematological abnormalities and serositis (p<0.05) and of positive anti-Rib antibodies in renal damage, arthritis, photosensitization, malar rash, hematological abnormalities and serositis (p<0.05). However, there were no statistically significant differences of positive anti-Sjögren's syndrome type B antibodies and anti-Sjögren's syndrome type A antibodies in renal damage, arthritis, malar rash, neuropsychiatric disorders, hematological abnormalities and serositis (p>0.05). Autoantibody spectrum is an important serological basis for SLE diagnosis. There are differences in the autoantibodies distribution of SLE patients with different organ damage, suggesting a certain clinical value for prediction of organ damage in SLE.

Clinical Manifestations of Systemic Lupus Erythematous in Children at Dr. Hasan Sadikin General Hospital Bandung, Indonesia

Background : Systemic Lupus Erythematous (SLE) is an autoimmune disease, characterized by production of autoantibodies, leading to damage caused by inflammation, directed toward multiple organs in the body. It is difficult to diagnose due to its mimicry with other diseases. The differential diagnosis of SLE is broad, including infection, malignancy and other inflammatory disorders. It is important to recognize the clinical manifestation of SLE from other possible diseases. Methods : A descriptive study was conducted on 56 SLE patientsfrom the Department of Child Health in Dr. Hasan Sadikin General Hospital, Bandung, Indonesia, in which the medical records was collected from 2010 to 2014. A total sampling method was used and incomplete records were excluded. Results : Out of the 56 subjects, 82.1% were female, with female to male ratio was 4.6:1. Most of the subjects (52%) was in the age category of 11 to 15 years old. Hematologic involvement was the most common clinical manifestation based on the diagnostic criteria (83.9%), and fever was the most common chief complaint (41.1%). Conclusions : From this study, fever, rash, and joint pain are the common chief complaints among SLE patients in children with hematologic and renal disorders are also found to occur.

OR8 A novel reactivity pattern with labscreen™ class I single antigen beads and its prevalence among SLE patients

Aim Previously, a distinct class II Luminex-single antigen bead (SAB) pattern was described among patients with systemic lupus erythematous (SLE) and attributed to antibodies targeting denatured antigens. In this study, we identify and describe a novel class I reactivity pattern shared among SLE patients. Methods Routine review of serum samples from transplant patients revealed a recurring pattern of reactivity on class I single antigen bead assays (One Lambda, Inc, lot 10). The pattern included the following beads: HLA-A∗33:03, -A∗36:01, -A∗80:01, -B∗54:01, -B∗53:01, -C∗06:02, -C∗07:02, -C∗18:02, -C∗14:02, -C∗03:03, -C∗03:04, and -C∗15:02. Further investigation was performed to determine the prevalence of this pattern and the specific characteristics of patients in which it occurred. Towards this end, all class I results performed in 2017 on renal transplant candidates/recipients were reviewed retrospectively for the presence of the above pattern. Patient diagnosis and demographic data was obtained from patient laboratory and hospital records. Results In total 5,992 samples from 3,027 patients were reviewed. We observed 105 (1.8%) samples from 58 patients displaying the class I pattern. Twenty-nine percent (n = 17) of these patients had no history of sensitization (e.g. blood transfusion, pregnancy, transplant) and only 29% of samples had distinct antibodies as identified by FlowPRA testing. Of the positive samples, 83% had reactivity against self-antigen involving 1 or more of the alleles in question, with HLA-C∗07:02 being the most common target (45%). Notably, 62% of these patients had a diagnosis of SLE. Conclusions To our knowledge, this is the first description of a distinct class I Luminex-SAB pattern with a prevalence in patients with SLE. This pattern was found in patients with no sensitization history, negative FlowPRA, and/or antibody to self-antigen(s). Epitope analysis failed to reveal a common determinant(s) to explain this pattern of reactivity. Given these findings, this pattern likely represents antibody targeting denatured antigen/unique peptide, molecular mimicry, autoimmunity, or a combination of some/all these factors.

Improving the quality of care and patient experience of care during the diagnosis of lupus: a qualitative study of primary care.

Purpose To better understand diagnostic delay and doctor-patient communication during the diagnosis of systemic lupus erythematous in patients without malar rash, we conducted a qualitative study of primary care providers' perceptions. Methods We conducted in-depth interviews with a purposive sample of eight primary care physicians in Kaiser Permanente Northern California. Telephone interviews were recorded, transcribed, reviewed, and coded for domains and themes. Results We identified five domains related to diagnosis: initial assessment and tests, initial diagnosis and empiric treatment, timeliness of diagnosis, communicating with the patient, and opportunities for improvement. In the absence of malar rash, the lupus manifestations are common while the disease is rare. Once the primary care provider believes that the disease may be autoimmune, they work with a rheumatologist, but this could take months. Initially, the physician assesses whether the condition is self-limiting or responds to empiric treatments. Over time, as empiric treatments fail or additional lupus manifestations emerge, the primary care provider makes a referral. Doctor-patient communication is critical to help the physician make sense of the symptoms, maintain trust, and assure the patient that he or she is receiving appropriate care. Patient persistence and communication are critically important. Continuing education was deemed essential by each physician. Conclusion In the absence of malar rash, a lupus diagnosis can be difficult. Enhanced doctor-patient communication, patient persistence, physician access to rheumatology and continuing education of primary care might improve time to diagnosis and the patient's experience with primary care. This knowledge is transferable to other rare, complex diseases.

Urinary clusterin—a novel urinary biomarker associated with pediatric lupus renal histopathologic features and renal survival

Lupus nephritis (LN) is a major risk factor for systemic lupus erythematous (SLE)-related morbidity and mortality. With the aim of bypassing renal biopsy, we analyzed urinary biomarkers for their ability to predict renal histopathologic features and end-stage kidney disease (ESKD). Urinary albumin, ß2-microglobulin (B2M), cystatin C, kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein 1 (MCP-1), clusterin, calbindin, interleukin-18 (IL-18), neutrophil gelatinase-associated lipocalin (NGAL), trefoil factor 3 (TFF3), osteopontin, and glutathione S-transferase π (GST-π) levels were measured at time of renal biopsy. Renal histopathologies were carefully reviewed. Urine from 60 pediatric SLE cases with LN, 29 without and 22 healthy controls were collected. Median age at SLE diagnosis was 12.92years (range = 4.27-17.30years) and 10 cases progressed to ESKD during a period of 4.12±2.17years. Urinary albumin and clusterin were significantly elevated (p = 0.035 and 0.048, respectively) in patients with tubulointerstitial renal lesions. Urinary clusterin among all urinary markers, performed best at predicting ESKD with cutoff of 0.61 × 10-4 (AUC = 0.804; p = 0.002). Interestingly, elevation of urinary clusterin likely resulted from local over-expression in tubulointerstitial tissue since the level of serum clusterin was not concomitantly higher (p = 0.424). Urinary biomarkers are emerging as non-invasive indicators for lupus-related renal histopathology and renal outcome prediction in pediatric SLE patients. Urinary clusterin, a newly identified biomarker, is an indicator that shows an association with tubulointerstitial renal lesions and demonstrates the best ability to predict ESKD.

Scabies presenting as cutaneous nodules or malar erythema: reports of patients with scabies surrepticius masquerading as prurigo nodularis or systemic lupus erythematosus

Scabies surrepticius is a unifying term that represents non-classical presentations of scabies mite infestation. A patient with scabies surrepticius is described: a man with scabies masquerading as prurigo nodularis. The 91-year-old man had metastatic prostate cancer and presented with diffuse pruritic nodules. Prurigo nodularis was suspected; however, the biopsy revealed scabies mites in the stratum corneum. He was successfully treated with topical permethrin 5% cream and oral ivermectin. In addition, the features of a woman with scabies mimicking systemic lupus erythematosus are summarized. The 47-year-old woman had idiopathic thrombocytopenic purpura and presented with malar erythema and a positive antinuclear antibody (titer 1:320). A diagnosis of systemic lupus erythematous was entertained until skin scraping and mineral oil preparation revealed scabies mites; she was successfully treated with oral ivermectin. In conclusion, Sarcoptes scabiei infestation can present with atypical clinical morphology and an absence of classical lesions such as burrows conventionally distributed in the interdigital web spaces, volar wrists, periumbilical area, or genitalia. Scabies surrepticius is a term that has been designated to describe these unusual presentations. Prurigo nodularis and systemic lupus erythematosus can be added to the litany of conditions masquerading as scabies and are included amongst the guises of scabies surrepticius.

Frequency of dental caries in active and inactive systemic lupus erythematous patients: salivary and bacterial factors.

The objective of this study was to determine dental caries frequency and to analyze salivary and bacterial factors associated with active and inactive systemic lupus erythematous (SLE) patients. Also, a proposal to identify dental caries by a surface, teeth, and the patient was developed. A cross-sectional, blinded study that included 60 SLE patients divided into two groups of 30 subjects each, according to the Activity Index for Diagnosis of Systemic Lupus Erythematous (SLEDAI). The decayed, missing, and filled teeth (DMFT) index and Integrative Dental Caries Index (IDCI) were used for analyzing dental caries. The saliva variables recorded were: flow, pH, and buffer capacity. The DNA copies of Streptococcus mutans and Streptococcus sobrinus were estimated by real-time PCR. The caries frequency was 85% for SLE subjects (73.3% for inactive systemic lupus erythematous (ISLE) and 100% for active systemic lupus erythematous (ASLE)); DMFT for the SLE group was 12.6 ± 5.7 and the IDCI was (9.8 ± 5.9). The ASLE group showed a salivary flow of 0.65 compared with 0.97 ml/1 min from the ISLE group; all variables mentioned above showed a statistical difference (p < 0.05). The salivary pH was 4.6 (6.06 for ISLE and 3.9 for ASLE). The DNA copies of S. mutans and S. sobrinus were high; all variables mentioned above show a significant statistical difference (p < 0.05) between groups. SLE patients had high DMFT and IDCI scores that were associated with a decrease in salivary flow, pH, and buffer capacity. There were high counts of S. sobrinus and S. mutans species, and IDCI is a useful tool to provide more detail about dental caries in epidemiological studies.