Long-standing Systemic Lupus Erythematosus Research Articles

Basal Cell Carcinoma in a Case of Systemic Lupus Erythematosus – A Case Report

Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune with autoantibody production, complementactivation, and immune complex deposition. SLE patients have higher mortality rates as compared to the general populationEven though there have been improvements in finding and treating diseases, cancer is still a major reason for death in this group.The most common type of cancer is Basal cell carcinoma, and it is widespread. The risk of malignancy in SLE is of considerableinterest because the immune and genetic pathways underlying the pathogenesis of SLE and the immunosuppressant drugs (ISDs)used in its management may mediate this altered risk. Here, we present a case of long-standing SLE in an elderly female who wason treatment for the same. She presented with a chronic ulcer over her scalp. She got checked, and a biopsy showed she hadBasal Cell Carcinoma on her scalp. Doctors removed it with surgery and covered the open area with skin from another part ofher body. At her first checkup, everything looked good, and the new skin was healing well. Even though skin cancers are knownto happen in people with certain skin conditions, getting Basal Cell Carcinoma with lupus is very uncommon. In various cohortstudies of adults with SLE and overall risk of malignancy, ranging from 1992 to 2017, it was found that though cutaneousmalignancies did occur, not a single study reported Basal Cell Carcinoma.

Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort.

Tackling active disease to prevent damage accrual constitutes a major goal in the management of patients with systemic lupus erythematosus (SLE). Patients with early onset disease or in the early phase of the disease course are at increased risk of developing severe manifestations and subsequent damage accrual, while less is known about the course of the disease in the long term. To address this issue, we performed a multicentre retrospective observational study focused on patients living with SLE for at least 20 years and determined their disease status at 15 and 20 years after onset and at their last clinical evaluation. Disease activity was measured through the British Isles Lupus Assessment Group (BILAG) tool and late flares were defined as worsening in one or more BILAG domains after 20 years of disease. Remission was classified according to attainment of lupus low-disease-activity state (LLDAS) criteria or the Definitions Of Remission In SLE (DORIS) parameters. Damage was quantitated through the Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI). LLAS/DORIS remission prevalence steadily increased over time. In total, 84 patients had a late flare and 88 had late damage accrual. Lack of LLDAS/DORIS remission status at the 20 year timepoint (p = 0.0026 and p = 0.0337, respectively), prednisone dose ≥ 7.5 mg (p = 9.17 × 10−5) or active serology (either dsDNA binding, low complement or both; p = 0.001) were all associated with increased late flare risk. Late flares, in turn, heralded the development of late damage (p = 2.7 × 10−5). These data suggest that patients with longstanding SLE are frequently in remission but still at risk of disease flares and eventual damage accrual, suggesting the need for tailored monitoring and therapeutic approaches aiming at effective immunomodulation besides immunosuppression, at least by means of steroids.

A Woman With Fatigue and Orthostatic Hypotension Who Had Run Out of Steroids

A 36-year-old woman presented to the rheumatology clinic for review of long-standing systemic lupus erythematosus, for which she used long-term steroids. She had run out of her personal supply of medications and did not have any steroids in the preceding 10 days. Physical examination demonstrated multiple inflammatory skin and joint changes compatible with a lupus flare. She appeared clinically unwell and had a blood pressure of 100/60 mm Hg, with a 10 mm Hg postural drop. Chest radiography revealed a narrowed cardiothoracic ratio (Figure 1) compared to a chest radiograph taken several months previously during another unrelated hospital visit (Figures 2 to 4),

Systemic lupus erythematosus associated with sarcoidosis: Case report

BackgroundThe association between systemic lupus erythematosus (SLE) and sarcoidosis has been considered as extremely rare. Most often, sarcoidosis coexists with Sjögren's syndrome. Researching the literature, it seems that the association of SLE and sarcoidosis is much more frequent than previously thought. Aim of the workWe present a case of a Serbian woman who was diagnosed with coexisting sarcoidosis and long-standing SLE. Case presentationThe 40 years old SLE patient was in long-standing remission on oral prednisolone (10 mg/day) and hydroxychloroquine (HCQ) (400 mg/day). She presented with fatigue, chest pain, and dry cough. Chest computerized tomography (CT) showed hilar and mediastinal lymphadenopathy. The biopsy had been performed and results showed sarcoidosis. Diagnosis has been confirmed: eosinophilic granulomas without central necrosis morphologically corresponding to chronic granulomatous lymphadenitis like sarcoidosis. Thereafter, the patient was hospitalized. No new symptoms appeared, and the physical examination was unremarkable. Serum calcium was elevated (2.75 mmol/l), anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA) and anti-Ro antibodies were positive. Angiotensin converting enzyme (ACE) level was high normal (51 IU/L) and QuantiFERON-TB Gold test negative. The dose of prednisolone was increased to 40 mg/day with HCQ and the patient was discharged from hospital. On follow-up the patient improved with reduction of the steroid dose. ConclusionThe association of SLE and sarcoidosis should be considered even though it is reported to be extremely rare. May be the real incidence of these combinations is underestimated. More research about genetics and pathogenesis is needed to completely understand these conditions.

Assessment of EULAR/ACR-2019, SLICC-2012 and ACR-1997 Classification Criteria in SLE with Longstanding Disease.

Background: Different classification criteria for systemic lupus erythematosus (SLE) have been launched over the years. Our aim was to evaluate the performance of the EULAR/ACR-2019, SLICC-2012 and ACR-1997 classification criteria in a cohort of SLE patients with longstanding disease. Methods: Descriptive observational study in 79 patients with established and longstanding SLE. The three classification criteria sets were applied to those patients. Results: Of the 79 patients, 70 were women (88.6%), with a mean age of 51.8 ± 14 years and a mean disease duration of 15.2 ± 11.5 years. The sensitivity of the different criteria were: 51.9%, 87.3% and 86.1% for ACR-1997, SLICC-2012 and EULAR/ACR-2019, respectively. In total, 68 out of 79 patients (53.7%) met all three classification criteria; 11.4% did not meet any classification criteria and were characterized by low SLEDAI (0.6 ± 0.9), low SLICC/ACR Damage Index (0.88 ± 0.56) and fulfilling only skin domains, antiphospholipid antibodies or hypocomplementemia. To fulfill EULAR/ACR-2019 criteria was associated with low complement levels (p < 0.04), high anti-dsDNA levels (p < 0.001), presence of lupus nephritis III-IV (p < 0.05) and arthritis (p < 0.001). Conclusion: The EULAR/ACR-2019 classification criteria showed high sensitivity, similar to SLICC-2012, in SLE patients with longstanding disease. Patients with serological, articular or renal involvement are more likely to fulfill SLICC-2012 or EULAR/ACR-2019 criteria.

Superiority of cardiac magnetic resonance imaging over echocardiography in early detection of subclinical cardiac abnormalities in systemic lupus erythematosus patients

BackgroundCardiovascular disease (CVD) has become increasingly recognized as a cause of mortality, especially in patients with long-standing systemic lupus erythematosus (SLE). Aim of the workTo detect subclinical cardiac involvement and its relation to clinical characteristics, disease activity and damage. Patients and methodsTransthoracic echocardiography (TTE) was performed in 36 SLE patients. Cardiac magnetic resonance (CMR) sections were obtained. T1-weighted inversion recovery scout images were obtained after injection of gadolinium. ResultsThirty-six patients were included with a mean age of 32.4 ± 8.5 years; 35 females and 1 male; with disease duration of 7.9 ± 5 years. The frequent cardiac presentations on TTE were tricuspid regurgitation (TR) (41.6%), mitral regurgitation (MR) (36.1%), mitral thickening (25%), early diastolic mitral flow/mitral flow during atrial contraction (E/A) < 1 (19.4%). The most frequent cardiac presentations by CMR were MR (25%), pericarditis (25%), mitral thickening (13.9%), TR (13.9%), myocarditis (8.3). Neither SLE Disease Activity Index (SLEDAI) nor Systemic Lupus International Collaborating Clinics (SLICC) damage index, high- sensitivity-C reactive protein (hsCRP), C3 and C4 levels were significantly correlated with the ejection fraction (EF) by CMR. There was significant negative correlation between disease duration and EF by CMR (r = −0.36, p = 0.03). Using multiple regression, EF by CMR was strongly predicted by disease duration (p = 0.025). The analysis of EF and MR fraction by CMR and TTE showed acceptable moderate agreement. CMR and TTE showed 83.3% agreement in the detection of pericarditis. ConclusionCMR is superior to echocardiography in detection of subclinical abnormalities in SLE.

Early Detection of Silent Myocardial Impairment in Drug-Naive Patients With New-Onset Systemic Lupus Erythematosus: A Three-Center Prospective Study.

Cardiac magnetic resonance imaging (MRI) has enabled the assessment of myocardial features, from tissue characteristics to functional changes, in patients with systemic lupus erythematosus (SLE). Echocardiography, however, detects cardiac decompensation. This study was undertaken to investigate the use of cardiac MRI to explore early warning signs of silent cardiac involvement and determine treatment timing in SLE. Clinical assessment and cardiac MRI studies were performed in 50 drug-naive patients with new-onset SLE, 60 patients with longstanding SLE, and 50 healthy subjects in a 3-center prospective study. Analysis of cardiac enzymes, the presence and size of regional myocardial fibrosis as indicated by late gadolinium enhancement, strain changes, and biventricular ejection fraction did not indicate cardiac impairment in the patients with new-onset SLE. Native myocardial T1 and extracellular volume (ECV), which are extracellular matrix indices, were elevated in the patients with new-onset SLE (mean ± SD 1,369 ± 79 msec versus 1,092 ± 57 msec in the control group for native T1; 32 ± 5% versus 24 ± 3% in the control group for ECV; P < 0.001 for both). The elevation was independent of SLE disease activity. This is the first study to indicate that drug-naive patients with new-onset SLE, even those with inactive disease, are likely to have silent cardiac impairment. Structural and functional changes in the myocardium are related to SLE disease stage; this finding indicates the value of early detection of myocardial involvement. Native myocardial T1 values and ECV, rather than currently used clinical rheumatic and cardiac indices, could serve as early detection markers of myocardial injury before the presence of visual fibrosis and functional decompensation.

Correlation of anti-cardiolipin antibodies with right ventricular systolic strain in systemic lupus erythematosus patients

IntroductionThe association between anticardiolipin antibodies (aCL) and cardiac disease in the presence of systemic lupus erythematosus (SLE) has been reported in various clinical trials. However, the correlation between these auto-antibodies and right ventricular (RV) function has been inadequately investigated. ObjectiveThe present study investigated the possible correlation of the plasma anticardiolipin antibodies, as a marker of autoimmune phenomenon, with RV functions, assessed by right ventricular speckle tracking, in patients with systemic lupus erythematosus independent of significant pulmonary hypertension, systolic dysfunction or valvular disease. MethodsForty-six SLE patients and 20 healthy controls were enrolled in our study and submitted thorough history, complete clinical examination then clinical scoring according to SLEDAI-2K score and then laboratory investigations particularly plasma anticardiolipin Ig_G or Ig_M antibodies. Then echocardiography was done to assess cardiac dimensions, left ventricular systolic functions, right ventricular functions and lastly speckle tracking for assessment of the right ventricular systolic strain. ResultsMost of the study patients were young adult females with long-standing SLE (mean=26±3.1). All study patients had a high clinical SLE score (>6). All patients were normotensives and non-diabetics. No significant correlation was found between anticardiolipin titre and left ventricular dimensions or systolic functions. Significant negative correlation was found between RV strain and plasma level of both anticardiolipin Ig_M and Ig_G. ConclusionThe present study identified that with the use of 2D speckle tracking in patients with SLE, right ventricular systolic function was significantly diminished with rising plasma titre of autoimmune (Ig_G or Ig_M) antibodies independent of cardiovascular risk factors.

Predictors of musculoskeletal flares and Jaccoud׳s arthropathy in patients with systemic lupus erythematosus: A 5-year prospective study.

To investigate the prognostic value of US in predicting musculoskeletal flares and Jaccoud׳s arthropathy (JA) in systemic lupus erythematosus (SLE). A total of 80 out of 94 patients (76 female; age 45.5 ± 13.2 years) with non-deforming non-erosive (NDNE) arthritis and 48/60 healthy controls (42 female; age 49.6 ± 11.6 years) completed the 5-year follow-up study. Each patient was prospectively assessed for the occurrence of musculoskeletal flares using BILAG2004 and hand deformities according to Jaccoud׳s articular index. Baseline clinical, serological, semi-quantitative (0-3 scale) ultrasound (US) findings, PD-synovitis and PD-tenosynovitis scores were used as covariates to identify predictors of study outcomes. Short Form 36 v2 (SF36v2) health survey questionnaire was administered. Overall, 12 MS flares in 10 (12.5%) patients were recorded and the incidence rate was 3.0 per 100 patient-year. Baseline PD-synovitis score independently predicted MS flare (p < 0.001; RR = 2.0; 95% CI: 1.4-3.0) within 2 years since US examination. In all, 5 (6.2%) patients developed JA whose incidence rate was 1.25 per 100 patient-year. Independent risk factors for development of JA were higher longitudinal BILAG score in the musculoskeletal domain (p = 0.005; RR = 2.4; 95% CI: 1.3-4.6) and longer disease duration (p = 0.013; RR 1.2; 95% CI: 1.1-1.3). JA and active musculoskeletal inflammation (BILAG ≥ C), but not US erosions, were associated with lower results in SF36v2 physical and mental summary components. Performing musculoskeletal US can be useful in order to predict MS flares. Jaccoud׳s deformities may arise in patients with long-standing SLE and prolonged, even subclinical, joint and tendon inflammation.

Tiny Bubbles: Coronary Aneurysms and Lupus

PRESENTATION Among the cardiac complications that can be spurred by systemic lupus erythematosus, coronary aneurysms are a particularly challenging presentation. A 32-year-old woman with longstanding systemic lupus erythematosus was referred to us for treatment of New York Heart Association class III heart failure, including possible mitral valve replacement. She had diffuse proliferative lupus nephritis (class IV), chronic renal failure, hypertension, chronic anemia, and lung tuberculosis. She had no personal or family history of Kawasaki’s disease, rheumatic heart disease, or coronary artery disease. Her drug regimen consisted of ramipril, bisoprolol, prednisone, isoniazid, rifampicin and pyrazinamide. She did not complain of recent chest, back, or upper limb pain.

Digital Ulcer as a Rare Complication of Systemic Lupus Erythematosus: A Case Report

The two most common rheumatological diseases which accompany Raynaud’s phenomenon are systemic sclerosis and systemic lupus erythematosus (SLE). Digital gangrene formation has no definitive etiology and pathogenesis, and Raynaud’s phenomenon often accompanies the necrotic lesions associated with this skin condition. Macrovascular involvement is commonly found in systemic sclerosis, but is very rare in patients with SLE. In our case, we identified ulnar artery involvement in the absence of any other risk factors associated with digital gangrene formation. Raynaud’s phenomenon is fairly common in SLE, but digital ulcers are seldomly seen. The most common cause of digital gangrene in SLE is antiphospholipid antibody syndrome (AAS). The risk factors for digital ulcers include duration of lupus, male gender, atherosclerosis, vasculitis, and AAS. Herein, we present a case with long-standing SLE who also had digital ulcers.

Acute Herpes Simplex Virus 1 Pneumonitis in a Patient With Systemic Lupus Erythematosus

A woman with severe and longstanding systemic lupus erythematosus presented with a 1-week history of fever up to 38°C and pain in her right flank. Computed tomography scan of the chest revealed interstitial infiltrates and multiple nodules. Bronchoalveolar lavage did not show any inflammatory cells. Gram stain and cultures for aerobic and anaerobic bacteria, fungi, and Nocardia; acid-fast staining; polymerase chain reaction for tuberculosis, cytomegalovirus, herpesvirus 6, and parvovirus B19; and IF staining for pneumocystic and Legionella antigen were all negative. Transbronchial biopsy was nondiagnostic. Open lung biopsy with polymerase chain reaction and immunohistochemistry analyses revealed herpes simplex virus 1 infection. Acyclovir therapy was initiated and was followed by significant improvement. Herpes simplex virus 1 infection (although unusual) should be considered in patients with systemic lupus erythematosus with an atypical clinical presentation.

Bacterial peritonitis: The presentation of a cyclophosphamide-associated bladder carcinoma in a long-standing systemic lupus erythematosus patient

Background: Cyclophosphamide is an alkylating agent that is used in various neoplastic and non-neoplastic diseases, including lupus nephritis. Cyclophosphamide can have a toxic urothelial effect, with different clinical manifestations, ranging from minor irritative symptoms and microscopic hematuria to hemorrhagic cystitis, bladder necrosis and rarely urothelial carcinoma. Various cyclophosphamide-induced bladder cancers have been reported, which had occurred after a prolonged oral cyclophosphamide therapy and more than decades after cessation of cyclophosphamide intravenous therapy.

Tacrolimus monotherapy in a patient with lupus flare using once-daily administration protocol

Tacrolimus monotherapy in a patient with lupus flare using once-daily administration protocol

Extensive Fat Necrosis With Lipomembranous Changes and Calcification in Lupus Erythematosus Panniculitis Is Not Necessarily Associated With Systemic Lupus Erythematosus

Biswas A, 2007, EUR J DERMATOL, V17, P550; Fraga J, 2008, DERMATOL CLIN, V26, P453, DOI 10.1016-j.det.2008.06.002; FRAGA J, 2008, DERMATOL CLIN, V26, pR6; Martens PB, 1999, J RHEUMATOL, V26, P68; Ng PPL, 2002, INT J DERMATOL, V41, P488, DOI 10.1046-j.1365-4362.2002.01510.x; Suda T, 2007, EUR J DERMATOL, V17, P86; TUFFANELLI DL, 1982, HAWAII MED J, V41, P394; TUFFANEL.DL, 1971, ARCH DERMATOL, V103, P231, DOI 10.1001-archderm.103.3.231; Watanabe T, 1996, BRIT J DERMATOL, V134, P123, DOI 10.1111-j.1365-2133.1996.tb07850.x; Yamamoto T, 2007, CLIN EXP DERMATOL, V32, P278, DOI 10.1111-j.1365-2230.2007.02358.x

Delayed lupus nephritis

Objective:To describe and analyse the clinical and immunological characteristics of a large series of patients with delayed lupus nephritis (LN).Methods:A cross-sectional study was carried out. Patients with systemic lupus erythematosus...

Effective treatment of young patients with pediatric-onset, long-standing lupus nephritis with tacrolimus given as a single daily dose: an open-label pilot study

The objective of the current work is to report the preliminary experience with tacrolimus (TL) administered as a single-dose daily for maintenance therapy of young patients with pediatric-onset, long-standing systemic lupus erythematosus (SLE). Six consecutive patients with long-standing SLE were recruited for a 6-month open-label trial of single-dose-daily administration of tacrolimus (3 mg/day) without dose up of concomitantly administered prednisolone (PDN). TL treatment was started at the time of the most recent flares. Data on the clinical and serologic lupus activity were collected prospectively. The baseline characteristics of the patients were: mean age, 20 years; urinary protein/creatinine ratio, 1.22 +/- 1.94; serum C3 level, 70.8 +/- 21.2 (normal, 79-152 mg/dL); serum complement hemolytic activity (CH50), 22.2 +/- 10.3 (normal, 23-46 U/mL); serum anti-dsDNA antibody titer, 60.4 +/- 71.7 IU/mL (normal, < 12.0 IU/mL); serum creatinine, 0.55 +/- 0.11 mg/dL; European Consensus Lupus Activity Measurement (ECLAM) index, 5.2 +/- 2.6. Despite the gradual tapering of the PDN dose, marked improvement as compared with the baseline values was observed in the ECLAM index examined at one and three months and serological parameters examined at three months after the start of treatment. After a 6-months' therapy, complete response was achieved in all of the patients (serum CH50 value, 27.7 +/- 8.3 U/mL; serum anti-dsDNA antibody titer, 28.4 +/- 27.9 U/mL and the ECLAM index, 1.2 +/- 1.2 (P < 0.05), respectively), except in one patient who showed WHO class V lupus nephritis. No serious adverse effects were observed. These data suggest that TL, even when administered as a single-daily dose, is effective and safe for selected young patients with pediatric-onset, long-standing SLE. However, further studies on a larger number of patients are needed to confirm these results.

Erstmanifestation einer diffusen proliferativen Lupus-Nephritis unter immunsuppressiver Langzeittherapie mit Mycophenolatmofetil

A now 34-year old female patient with long-standing systemic lupus erythematosus (SLE) had successfully been treated with mycophenolate mofetil (MMF) because of former pleuropericardial and pulmonal lupus manifestations when she was admitted with edema for the first time. Laboratory investigations of urine and blood showed findings compatible with lupus nephritis. Kidney biopsy revealed lupus nephritis type IV. Despite the initiation of cyclophosphamide bolus and corticosteroid therapy, the kidney function and renal hypertension worsened and the patient developed cerebral manifestations. Finally, synchronization of plasmapheresis with subsequent pulse cyclophosphamide led to remission of SLE. Although MMF is a promising innovative agent for induction and maintenance therapy of lupus nephritis that can be used instead of cyclophosphamide, there may be cases like the one reported here, in which MMF cannot prevent lupus flares in first manifestation of severe renal disease.

JC virus meningitis in a patient with systemic lupus erythematosus

The human neurotropic JC virus (JCV) is most commonly acquired during childhood, and, because no clinical illness has been associated with primary infection, is presumed to be asymptomatic. In the immunocompromised host, JCV is responsible for progressive multifocal leukoencephalopathy (PML). We describe a patient with longstanding systemic lupus erythematosus who presented with acute meningitis without encephalitis or PML. JCV was the only pathogen found in the cerebrospinal fluid suggesting a primary infection or symptomatic reactivation. Our observation demonstrates the expanding clinical importance of JCV in autoimmune diseases, and diagnostic tests for JCV should be included in the investigative work-up for meningitis or encephalitis in these patients.

Systemic lupus erythematosus and gouty arthritis: an uncommon association.

To examine the frequency of gouty arthritis in patients with systemic lupus erythematosus (SLE) and elucidate the clinical factors that predispose to this occurrence. A chart review of in-patients (1989-2001) and out-patients (1999-2001) with ICD9 billing codes for gouty arthritis/tophaceous gout and SLE was performed. Twenty-six patients were identified. Patients meeting American College of Rheumatism criteria for SLE and crystal-proven gout or a self-limited inflammatory arthritis felt to be crystal-induced (i.e. definite gout) were included. Ten patients were identified. Their mean age was 46.5 yr; 80% were African-American and 70% were women. Nine of the ten had lupus nephritis and four had undergone renal transplantation. An acute worsening of renal function unrelated to lupus activity preceded almost all gout flares. Lupus activity at the time of the first gout attack as measured by the Systemic Lupus Activity Measure (SLAM) was low. Eight patients were on prednisone (mean dose 8 mg/day) for their SLE at the time the attack of gout occurred. Gouty arthritis is uncommon in SLE; it occurs primarily in patients with long-standing SLE and nephritis. Worsening renal function usually preceded gout attacks, but SLE disease activity was minimal. Crystal-induced arthritis should be included in the differential diagnosis of a lupus patient presenting with acute inflammatory arthritis because the long-term treatment of the two conditions differs substantially.