Presentation Of Systemic Lupus Erythematosus Research Articles

Systemic lupus erythematosus presenting with bullous lesions, cutaneous vasculitis and laryngeal ulcerations-a rare association

<p>Bullous SLE is an uncommon complication of SLE with autoantibodies to type VII collagen of dermo-epidermal junction. We report a 31-year-old female who presented with vesiculobullous lesions in the photo exposed areas along with vasculitic lesions over palms and soles and ulcerations in the larynx. She also had history of photosensitivity and hair loss. Investigations showed significant anaemia and high titres of antibodies (ANA, anti-ds-DNA, anti Sm) confirming SLE. Histopathology showing sub epidermal blister with neutrophils and immunofluorescence favoured diagnosis of bullous SLE. The patient responded well to high dose parenteral steroids and dapsone. In view of high probability of renal involvement, patient was referred to nephrologist and is under regular follow up.</p><p><strong> </strong></p><p> </p>

Unusual Case Presentation of Systemic Lupus Erythematosus in a Young Woman

Systemic Lupus Erythematosus (SLE) is a chronic multisystem autoimmune disease. Serositis occurs in 16% of SLE patients, and while cardiac tamponade and acute peritonitis with ascites can occur during the course of the disease, they are rare as the first presentation. A 25-year-old woman presented to the emergency department in Tishreen Hospital with complaints of dyspnea, fever, chills, and chest and abdominal pain. Two months prior, she suffered from musculoskeletal pain, fatigue, anorexia, weight loss of about 15 kg, severe hair loss, and recurrent oral aphthous. On clinical examination, the patient was pale and tired with dyspnea and pitting edema (grade 3–4). Pericardiocentesis was emergently performed because there were signs of cardiac tamponade. Three days later, the patient developed an acute surgical abdomen due to acute peritonitis and ascites. Later, the patient was diagnosed with SLE after excluding malignant and infectious diseases. Consequently, methylprednisolone pulses, azathioprine, and hydroxychloroquine 200 mg/day were introduced immediately. The clinical status of the patient dramatically improved, and three months later, the patient was symptom-free with normal laboratory tests. In conclusion, although cardiac tamponade and acute surgical abdomen because of acute peritonitis and ascites as the initial presentation of SLE are very rare, they can occur coincidently.

Insights into the clinical presentation of juvenile systemic lupus erythematosus: the PRAGMA monocentric cohort of 177 patients.

The aim of this work is to describe the clinical manifestations at onset and during follow-up in a monocentric cohort of patients with juvenile systemic lupus erythematosus (jSLE) from the Paediatric Rheumatology group of the Milan area (PRAGMA). Patients were retrospectively included in case of i) SLE diagnosis according to the 1997 American College of Rheumatology or the 2012 SLICC classification criteria and ii) disease onset before 18 years. Among the 177 recruited patients (155 females), haematologic involvement was the most common disease manifestation (75%), followed by joint and cutaneous involvements (70% and 57%, respectively). Renal disease was observed in 58 patients (32.8%), neurological complications in 26 cases (14.7%). Patients presented most commonly 3 clinical manifestations (32.8%), while 2 organ involvements were identified in 54 patients (30.5%) and 4 in 25 subjects (14.1%). The 49 patients with disease onset <10 years had less commonly articular involvement (p=0.02), while patients aged >14.8 years displayed less neurological manifestations (p=0.02). At a median follow-up of 118 month, the disease progressed in 93 patients, with a median of 2 new manifestations per patient. Low complement at diagnosis predicted new clinical manifestations (p=0.013 for C3 and p=0.0004 for C4). The median SLEDAI at diagnosis was 13; SLEDAI was substantially similar at 6 months, decreased at 12 months to remain stable at 18 months and further reduce at 24 months (p<0.0001). These data from a large jSLE monocentric cohort allow gaining further insights into a rare disease with a still high morbidity burden.

HEMOTHORAX AND HEMOPERICARDIUM AS PRESENTING SIGNS OF SYSTEMIC LUPUS ERYTHEMATOSUS

HEMOTHORAX AND HEMOPERICARDIUM AS PRESENTING SIGNS OF SYSTEMIC LUPUS ERYTHEMATOSUS

S3395 Enterocolitis as the Initial Presentation of Systemic Lupus Erythematosus

S3395 Enterocolitis as the Initial Presentation of Systemic Lupus Erythematosus

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

S3495 What Do These Eosinophils Mean? Treatment Naive Systemic Lupus Erythematosus Presenting With Eosinophilic Ascites, Eosinophilic Pleural Fluid, and Enteritis of Unclear Etiology

Introduction: Eosinophilic ascites (EA), lupus enteritis (LE), and eosinophilic enteritis (EE) are rare systemic lupus erythematosus (SLE) complications. When they do occur, overlapping diagnostic criteria causes problems in distinguishing between a SLE-related etiology or EE. For example, intestinal eosinophil infiltration associated with EE is also consistently found in disseminated SLE autopsy case reports. Although both conditions are treated with steroids, therapies after treatment failure are different. We present a rare case of a SLE patient with this diagnostic dilemma with suboptimal response to SLE therapies, questioning whether an EE treatment algorithm may be more appropriate. Case Description/Methods: An afebrile 55 year old female with untreated SLE presented with constipation and a mildly tender distended abdomen. Imaging described moderate ascites, continuous small bowel thickening, ileus, and bilateral pulmonary infiltrates with pleural effusions. Significant results included a white blood cell count of 3.8 without eosinophils, erythrocyte sedimentation rate 5, C-reactive protein < 1, positive ANA (1:160), positive double stranded DNA, and low C3/C4 complement. Paracentesis yielded an exudative eosinophilic (71%) ascitic fluid with a low serum-ascites albumin gradient of 0.9 g/dL, high protein of 3.9 g/dL, and no evidence of malignancy or infection. She also had exudative eosinophilic pleural fluid. Endoscopy revealed mild cecal ischemia, mild gastritis, and a normal terminal ileum without eosinophils. Rheumatology diagnosed her with SLE serositis with peritoneal and pleural involvement complicated by LE, but they were unable to rule out EE. She responded to intravenous steroids but deteriorated when she was switched to oral prednisone. She then failed a regimen of hydroxychloroquine and oral prednisone. During her most recent hospitalization, the patient was transitioned to a regimen of hydroxychloroquine, mycophenolate mofetil, and oral prednisone. Discussion: It is unclear whether our patient’s sub-optimal therapy response is due to SLE or EE, with our case illustrating the difficulties in determining a diagnosis. Determining a diagnosis influences treatment and the patient's clinical course. In EE, treatment includes dietary therapy, antihistamines, leukotriene antagonists, and mast-cell stabilizers. However, our patient’s symptoms are attributed to serositis, leading to a focus on escalating her SLE immunosuppressive therapy rather than re-examine the diagnosis.

Systemic Lupus Erythematosus Presenting as Unilateral Frosted Branch Angiitis

A 28-year-old woman with a history of Hashimoto’s thyroiditis presented with hand motion vision of the left eye. Fundus examination showed unilateral frosted branch angiitis (Fig A). Angiography revealed blockage with late vascular leakage (Fig B). Lower extremity displayed an ulcerative rash (Fig C), with biopsy demonstrating leukocytoclastic vasculitis (Fig D). This can occur in various autoimmune and infectious conditions; however, systemic work-up was consistent with systemic lupus erythematosus with positive anti-nuclear, anti-Smith, and anti-double stranded DNA antibodies.

P35 Lupus masquerading as Cholangiocarcinoma: One of the many faces of multifaceted lupus

Introduction/BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune condition which can present with a wide variety of clinical features. This variation can lead to delayed diagnosis or misdiagnosis. Gastroenetrological pathology in SLE is rare but primary autoimmune hepatitis, pancreatitis, lupus enteritis, cholestatic biliary disease and protein losing enteropathy have all been reported. We would like to share the first known presentation of SLE as cholangiocarcinoma.Description/MethodA 62-year-old, previously fit and well, gentleman presented with a 1 month history of painless jaundice, a palpable gallbladder (Courvoisier syndrome), dark urine, pale stool, generalised rash, pruritus, weight loss and anorexia. The presence of these red flag features raised concerns. Initial investigation with Endoscopic retrograde cholangiopancreatography (ERCP) revealed distal common bile duct (CBD) stricture which was further explored with computerised tomography of thorax, abdomen, pelvis and magnetic retrograde cholangiopancreatogram (MRCP) both raising the suspicion of a cholangiocarcinoma in the presence of constitutional symptoms. Several biopsies and brushings taken during multiple ERCP’s failed to identify clear mitotic cells, IgG 4 staining remained negative. Further investigation with Positron emission tomography – computerised tomography (PET-CT) showed significant FDG uptake around the upper part of the stent with final working diagnosis of Cholangiocarcinoma and a Whipple’s procedure was planned.In the interim, the patient developed swollen painful hands, scleritis and a yellow blistering rash. He was referred urgently onto the Rheumatology team after receiving the positive immunology results: ANA (1:1280), Anti double stranded DNA and Rheumatoid Factor (RF) along with hypocomplementemia (C3&C4).Discussion/ResultsSubsequent work up under rheumatology was done considering the possibilities of diagnosis of Systemic lupus erythematosus (SLE), Lupus related autoimmune hepatitis and reactive arthritis as a result of underlying malignancy. Patient’s immunology and other clinical features such as skin rash were strongly supportive of SLE as a primary diagnosis. He was initially treated with low dose prednisolone with dramatic improvement in arthritis and gradual improvement in liver function test. Following MDT discussions with the hepato-biliary team it was felt that lupus was the cause of the biliary stricture which settled well with steroid treatment. A final ERCP was arranged to remove the stent and take final brushings. Methotrexate was started at this point. His SLE remains well controlled on methotrexate and his liver function tests, including bilirubin, remain within the normal range. Patient has now been completely asymptomatic and is discharged from the care of hepatobiliary team with long term follow up for his SLE with rheumatology.Key learning points/ConclusionThis is extremely rare first presentation of SLE presenting with painless jaundice and biliary stricture which improved both biochemically and clinically with treatment of SLE. The key learning points included:Urgent review by rheumatology can facilitate timely diagnosis and preventable admissions/interventions such as major intraabdominal procedure in the form of whipple’s disease planned in our case.Cancer remains a major cause of mortality and morbidity in rheumatological conditions like SLE. Hence initial diagnosis can become extremely difficult if an overlap between these two happens.Treatment options can be limited initially and steroids remain the main stay of treatment till mitotic lesions have been ruled out and disease modifying agents can be used without any contraindications. But disease control for our conditions remains challenging in presence of cancers.

P33 The successful use of Belimumab in recurrent small bowel obstruction secondary to Lupus Enteritis

P33 The successful use of Belimumab in recurrent small bowel obstruction secondary to Lupus Enteritis

Sex differences in systemic lupus erythematosus (SLE): an inception cohort of the Chinese SLE Treatment and Research Group (CSTAR) registry XVII.

The onset and clinical presentation of systemic lupus erythematosus (SLE) are sex-related. Few studies have investigated the distinctions in clinical characteristics and treatment preferences in male and female SLE patients in the initial cohort. This study aimed to improve the understanding of Chinese SLE patients by characterizing the different sexes of SLE patients in the inception cohort. Based on the initial patient cohort established by the Chinese SLE Treatment and Research Group, a total of 8713 patients (795 men and 7918 women) with newly diagnosed SLE were enrolled between April 2009 and March 2021. Of these, 2900 patients (347 men and 2553 women) were eligible for lupus nephritis (LN). A cross-sectional analysis of the baseline demographic characteristics, clinical manifestations, laboratory parameters, organ damage, initial treatment regimens, and renal pathology classification was performed according to sex. In the SLE group, as compared to female patients, male patients had a later age of onset (male vs. female: 37.0 ± 15.8 years vs. 35.1 ± 13.7 years, P = 0.006) and a higher SLE International Collaborative Clinic/American College of Rheumatology damage index score (male vs. female: 0.47 ± 1.13 vs. 0.34 ± 0.81, P = 0.015), LN (male vs. female: 43.6% vs. 32.2%, P < 0.001), fever (male vs. female: 18.0% vs. 14.6%, P = 0.010), thrombocytopenia (male vs. female: 21.4% vs. 18.5%, P = 0.050), serositis (male vs. female: 14.7% vs. 11.7%, P = 0.013), renal damage (male vs. female: 11.1% vs. 7.4%, P < 0.001), and treatment with cyclophosphamide (CYC) (P < 0.001). The frequency of leukopenia (male vs. female: 20.5% vs. 25.4%, P = 0.002) and arthritis (male vs. female: 22.0% vs. 29.9%, P < 0.001) was less in male patients with SLE. In LN, no differences were observed in disease duration, SLE Disease Activity Index score, renal biopsy pathological typing, or 24-h urine protein quantification among the sexes. In comparisons with female patients with LN, male patients had later onset ages (P = 0.026), high serum creatinine (P < 0.001), higher end-stage renal failure rates (P = 0.002), musculoskeletal damage (P = 0.023), cardiovascular impairment (P = 0.009), and CYC use (P = 0.001); while leukopenia (P = 0.017), arthritis (P = 0.014), and mycophenolate usage (P = 0.013) rates were lower. Male SLE patients had more severe organ damage and a higher LN incidence compared with female SLE patients; therefore, they may require more aggressive initial treatment compared to female patients.

Isolated Gastrointestinal Symptoms as Initial Presentation in Systemic Lupus Erythematosus and Its Differential Diagnosis

Initial clinical presentation of Systemic Lupus Erythematosus (SLE) is varied as it affects various organs in the body. While the typical presentation of SLE is mucocutaneous, musculoskeletal and haematological manifestation, gastrointestinal (GI) manifestation is a rare initial presentation of SLE. We discuss the case of a 13-year-old girl who was diagnosed with SLE after she presented with isolated gastrointestinal symptoms. She presented with vomiting, diarrhoea, mild colicky abdominal pain and bilateral ankle oedema; and was treated as acute gastroenteritis. She returned after one week with worsening symptoms. Her full blood count showed bicytopenia; urinalysis had proteinuria and haematuria, and renal profile revealed acute on chronic kidney injury which triggered suspicions of a more serious disease rather than simple viral gastroenteritis. Further investigations of positive anti-nuclear antibody, low complements and positive Coombs’s test supported the diagnosis of SLE. The diagnosis of SLE was confirmed when her renal biopsy reported crescentic lupus nephritis ISN/RPS Class IV. Additional investigation to investigate the cause of her gastrointestinal symptoms included an ultrasound abdomen which showed minimal ascites and bilateral renal parenchymal disease. She was planned for colonoscopy but due to the unavailability of paediatric endoscopy, colonoscopy was postponed. However, her symptoms markedly improved with intravenous Cyclophosphamide which supported the diagnosis of GI SLE. This case report is to highlight that a patient with symptoms of simple viral gastroenteritis might have a more serious underlying disease. Even though rare, SLE can present with gastroenteritis symptoms and is one of the differential diagnoses that should be considered.

Systemic lupus erythematosus: rarer in men than women but more severe

Although systemic lupus erythematosus (SLE) is rarer in men than women, it is more severe. The clinical presentation of SLE is variable and there are sex differences. Given the relative rarity of SLE in men, clinicians should have a low threshold for investigation to avoid delayed diagnosis.

Endocapillary hypercellularity levels are associated with early complete remission in children with class IV lupus nephritis as the initial presentation of SLE

BackgroundEndocapillary hypercellularity (ECHC) is commonly seen in class IV lupus nephritis (LN), the most common and severe LN in children. Factors influencing early complete remission (CR) in pediatric class IV LN have been poorly described. We investigated the relationship between ECHC levels and early CR in pediatric class IV LN.MethodsPatients with newly, simultaneously diagnosed systemic lupus erythematosus (SLE) and class IV LN by renal biopsy from 2012 to 2021 were studied. In this retrospective study, two pathologists who were blind to clinical information reviewed all pathological data retrospectively and classified glomerular lesions according to the revised criteria of the International Society of Nephrology and the Renal Pathology Society (ISN/RPS). The demographics, baseline clinical characteristics, laboratory parameters, renal histopathological findings, treatment regimen and CR at 6 months after immunosuppressive therapy were analyzed. ECHC was categorized as: > 50% (group A), 25–50% (group B) and < 25% (group C). CR was defined as absence of clinical symptoms, 24-hour urinary protein < 0.15 g, and normal levels of serum creatinine and albumin.ResultsSixty-four patients were identified: 23, 15 and 26 in groups A, B and C, respectively. Group A had significantly higher levels of D-dimer, urine protein, and SLE disease activity index (SLEDAI) than groups B and C. Group C had a markedly higher estimated glomerular filtration rate (eGFR) than groups A and B. A substantially greater proportion of patients in group A had glomerular microthrombi and basement membrane thickening than in groups B and C. At 6 months post treatment, CR was achieved in 19 (82.6%), 5 (33.3%) and 11 (42.3%) in groups A, B and C, respectively (p < 0.05, group A vs groups B and C). Multiple logistic regression analysis revealed that ECHC and urine protein levels were significantly associated with CR.ConclusionECHC and urine protein levels may be valuable biomarkers for predicting early CR in pediatric class IV LN.

Recurrent abdominal pain and vomiting with elevated triglyceride and positive antinuclear antibody in a girl aged 12 years.

A girl aged 12 years and 2 months presented with recurrent abdominal pain and vomiting for more than 2 years and arthrodynia for 3 months. She was diagnosed with recurrent acute pancreatitis with unknown causes and had been admitted multiple times. Laboratory tests showed recurrent significant increases in fasting serum triglyceride, with elevated immunoglobulin and positive antinuclear antibody. The girl was improved after symptomatic supportive treatment. The girl developed arthrodynia with movement disorders 3 months before, and proteinuria, hematuria, and positive anti-double-stranded DNA antibody were observed. The renal biopsy was performed, and the pathological examination and immunofluorescence assay suggested diffuse lupus nephritis (type Ⅳ). She was finally diagnosed with systemic lupus erythematosus (SLE), lupus nephritis (type Ⅳ), and recurrent acute pancreatitis. Pancreatitis was suspected to be highly associated with SLE. She was treated with oral hydroxychloroquine sulfate and intravenous methylprednisolone sodium succinate and cyclophosphamide. Arthrodynia was partially relieved. She was then switched to oral prednisone acetate tablets. Intravenous cyclophosphamide and pump infusion of belimumab were regularly administered. Now she had improvement in arthrodynia and still presented with proteinuria and hematuria. It is concluded that recurrent acute pancreatitis may be the first clinical presentation of SLE. For pancreatitis with unknown causes, related immunological parameters should be tested, and symptoms of the other systems should be closely monitored to avoid delaying the diagnosis.

Bullous systemic lupus erythematosus in females.

Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.

A case report: extremely severe pulmonary artery hypertension as an initial presentation of systemic lupus erythematosus in pregnancy

A 33-year-old G2 P1001 female at 13 weeks of gestation presented with dyspnea, tachycardia, and hypotension. Initial assessment revealed cardiogenic shock and acute respiratory failure caused by extremely severe pulmonary artery hypertension (PAH). Multidisciplinary efforts led to the diagnosis of severe PAH as an initial manifestation of systemic lupus erythematosus (SLE). Team care included gestational termination via dilation and curettage (D&amp;C), followed by corticosteroids and vasodilator therapy, with excellent clinical and hemodynamic outcomes.

Systemic Lupus Erythematosus and Hereditary Coproporphyria: Two Different Entities Diagnosed by WES in the Same Patient

Background Hereditary coproporphyria (HCP) is a rare autosomal dominant disorder caused by a partial deficiency of coproporphyrinogen III oxidase (CPOX), and systemic lupus erythematosus (SLE) is an autoimmune disease with a strong genetic predisposition. SLC7A7 (solute carrier family 7 member 7) may be associated with monogenic lupus disease; however, only 2 cases of concomitant HCP and SLE have been reported. Methods We report a 30-year-old woman with a six-year history of SLE presenting with abdominal pain, vomiting, dysuria, tachycardia, and hyponatremia. Whole exome sequencing (WES) and Sanger sequencing were carried out for the proband and members of her pedigree to detect the genetic background. The Gene Expression Omnibus (GEO) database was used to search the related gene expression profiles. Differentially expressed genes (DEGs) were identified using GEO2R. Result A novel heterozygous splicing mutation of CPOX (NM_000097): c.700+2 T > C (intron 2) was detected by WES in the proband, and it was considered likely pathogenic (PSV1+PM2). Sanger sequencing verified the heterozygous mutation of CPOX in the proband, although it was not detected in her father. WES also identified 62 other gene variants, especially two heterozygous variants in SLC7A7 (NM_001126106): c.250G > A (p. V84I) and c.625+1G > A (splicing). DEGs were detected from GSE51997, and the expression of CPOX was downregulated in SLE patients compared with normal controls (adj. P = 0.0071, logFC = −1.0975). Conclusion This study presents the first reported case of SLE coexisting with HCP in China; moreover, a novel splicing mutation of CPOX, i.e., c.700+2 T > C (intron 2), and two heterozygous mutations of SLC7A7 were reported. The simultaneous mutations of CPOX and SLC7A7 may explain the etiopathogenetic connections of HCP and SLE.

An Incidental CT Scan Discovery of a Bilateral Renal Infarction in a Patient with Systemic Lupus Erythematosus Presenting with Acute Pancreatitis

Although patients with systemic lupus erythematosus, especially those with antiphospholipid antibodies, have a high incidence of arterial and venous thrombotic events, renal infarcts have been infrequently reported in these patients and are likely to be underestimated. In this paper, we report a case of multiple bilateral renal infarctions in a patient suffering from systemic lupus erythematosus, provide a brief insight into the clinical features and especially highlight the radiological aspects of this diagnosis.

Successful treatment of bullous lupus with corticosteroids and belimumab: A case report.

Cutaneous lupus erythematosus (CLE) is one of the most common manifestations of systemic lupus erythematosus (SLE), although it can manifest as an independent entity as well. Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of SLE presenting as tense vesiculobullous eruptions in a photosensitive distribution. Pathophysiology is secondary to autoantibodies against noncollagenous domain 1 and 2 (NC1 and NC2) type VII collagen, and histopathology reveals dense neutrophilic infiltration of the dermis with direct immunofluorescence showing IgG deposition at dermoepidermal junction. There is lack of data on available therapeutic options to treat BSLE, and varying responses to dapsone, methotrexate, azathioprine and corticosteroids have been reported. Belimumab, a fully humanised Change to Immunoglobulin G1λ (IgG1λ) monoclonal antibody targeting soluble B lymphocyte stimulator protein, was the first Food and Drug Administration-approved drug for SLE and has been reported to be effective for CLE. We present the case of a 41-year-old black female with SLE presenting with BSLE, who was successfully treated with corticosteroids and belimumab and did not experience disease relapse even after discontinuation of corticosteroids. To our knowledge, this is the first reported case of successful treatment of BSLE with belimumab, and further research can help determine the role of belimumab in the treatment of BSLE.