scholarly journals An Unusual Presentation of Lupus in a Pediatric Patient

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Vimal Master Sankar Raj
Keyword(s):  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Organ Damage ◽  
Multiple Organ ◽  
Neurological Involvement ◽  
Childhood Onset ◽  
American Girl ◽  
African American Girl ◽  
Worse Prognosis ◽  
Inflammatory Tissue

Systemic lupus erythematosus (SLE) is an autoimmune disease causing inflammatory tissue damage. Multiple organ damage can ensue with renal and neurological involvement carrying the worse prognosis. In this case report we present a 10-year-old African American girl who presented with abnormal choreiform movements, headache, weight loss, and fatigue. Detailed clinical examination with laboratory and imaging studies clinched the diagnosis of SLE. Echocardiogram revealed the presence of Libman-sacks endocarditis. Patient showed rapid resolution of symptoms with steroid therapy. A brief discussion on childhood onset lupus along with the varied clinical presentation is discussed.

Cognitive and Psychiatric Manifestations of Childhood-Onset Systemic Lupus Erythematosus

2010 ◽  
Author(s):  
Deborah M. Levy ◽  
Gail S. Ross
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Organ Damage ◽  
Multiple Organ ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Disease Manifestation ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Similar Disease

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies directed against multiple organ systems. Individuals with SLE may have disease of only one organ system or of many organ systems at one time during a “flare” of disease activity. Systemic lupus erythematosus is characterized by multiple flares and remissions, and flares may lead to end-organ damage. The diagnosis of SLE is generally made by fulfilling four out of 11 of the American College of Rheumatology (ACR) 1997 Revised Classification Criteria for SLE (Hochberg 1997). Although the 1997 revised criteria have not been validated, the earlier 1982 criteria (Tan et al. 1982) have a sensitivity of 96% and specificity of 100%in childhood-onsetSLE(cSLE) (Ferraz et al. 1994). See Table 3.1 for these criteria. Systemic lupus erythematosus has a prevalence rate of 500 per million persons in the U.S. population (Klippel 1997) but may be as high as 130 per 100,000 persons (Uramoto et al. 1999). Approximately 15% of all SLE has its onset in childhood (prior to 18 years of age). Systemic lupus erythematosus occurs more commonly in non-Caucasians, with greater severity of SLE in children and adult Latino and African Americans than in non-Latino Caucasians (Michet et al. 1979).The female predominance of SLE in adulthood (10:1 female-to-male ratio) is less pronounced in childhood, and the ratio prior to puberty is more likely to be 5:1 or even 3:1.The majority of cSLE cases have onset in the peripubertal age period (10–15 years), which continues to suggest a link between estrogen and other hormones on the development of SLE. Childhood-onset SLE differs from adult-onset SLE in that the frequency of renal disease is higher, and the incidence and prevalence of neuropsychiatric SLE (NPSLE) is probably greater (Tucker et al. 1995; Sibbit et al. 2002). Children require aggressive immunosuppression, which on a dose per kilogram of body weight is generally higher than that required by an adult to treat a similar disease manifestation. Immunosuppression often includes corticosteroids. At least in adults, corticosteroids do not appear to cause cognitive impairment (Carbette et al. 1986; Denburg et al. 1997; Carlomagno et al. 2000).

scholarly journals Acute Kidney Injury and Acute Liver Failure in Leptospira Infection and Weil’s Syndrome

2020 ◽  
Vol 4 (2) ◽  
pp. 21-28
Author(s):  
Jonathan S Chávez-Iñiguez ◽  
Jose Said Cabrera-Aguilar ◽  
Guillermo Garcia-Garcia ◽  
Juan Armendáriz-Borunda
Keyword(s):  
Acute Kidney Injury ◽  
Clinical Presentation ◽  
Clinical Signs ◽  
Kidney Injury ◽  
Organ Damage ◽  
Multiple Organ ◽  
Serological Tests ◽  
Microbiological Criteria ◽  
Sanitary Conditions ◽  
The Impact

Leptospirosis is considered a zoonosis acquired predominantly from contaminated surfaces and water, more commonly in emerging countries with limited sanitary conditions. Leptospira in the host unleashes an immune response that explains the symptoms and clinical signs; once it reaches the kidney and liver tissue, it can manifest with alterations that lead to acute and chronic diseases in both organs. Weil’s syndrome is the best known clinical manifestation with jaundice and acute kidney injury that could lead to multiple organ failure and death. For its diagnosis, there are simplified scores such as the SPiRO score, the microbiological criteria by microscopy or serological tests; the treatment focuses on antibiotics and, if necessary, provides organic support until the infection is curtailed. The purpose of this review was to address the impact of Lep-tospira infection on the kidney and liver, the mechanisms of organ damage, the clinical presentation, and diagnosis and management of this disease.

scholarly journals Cytokine Networks in Systemic Lupus Erythematosus

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Hooi-Ming Lee ◽  
Hidehiko Sugino ◽  
Norihiro Nishimoto
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cytokine Production ◽  
Organ Damage ◽  
Multiple Organ ◽  
Serum Cytokine ◽  
Systemic Lupus ◽  
Pathological Conditions ◽  
Cytokine Levels ◽  
Cytokine Networks

Systemic lupus erythematosus (SLE) is an autoimmune disease more prominent in women and characterized by multiple organ damage. Imbalance in cytokine production and cytokine levels correlates with SLE progression, making the understanding of SLE cytokine networks very important for SLE treatment strategy and drug development. In this article, we review cytokine networks that may be involved in the pathogenesis of SLE by briefly describing abnormal cytokine production and serum cytokine levels in SLE patients. We also focus on the pathological roles of cytokines and their interactions in immunoregulatory networks and suggest how their disturbances may implicate in pathological conditions in SLE. Finally, we further discuss the influence of estrogen on these cytokine networks.

scholarly journals Sysyemic Lupus Erythematosus with Multiple Organ Damage

2020 ◽  
Vol 17 (5) ◽  
pp. 63-73
Author(s):  
Andreea Alexandra Nicola ◽  
Mădălina Dună ◽  
Ioana Miler ◽  
Nicoleta Petre ◽  
Denisa Predeţeanu
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Multiple Organ ◽  
Alveolar Haemorrhage ◽  
Interdisciplinary Management ◽  
Childbearing Age ◽  
Pulmonary Infarction ◽  
Pulmonary Manifestations

Abstract Systemic lupus erythematosus (SLE) is a heterogeneous rheumatic disease with various clinical manifestations and a multifactorial pathogenesis. Although the etiology of SLE is unknown, certain risk factors have been identified as promoters of an imbalance in the immune system with antibody formation and tissue damage secondary to the deposition of immune complexes. Women of childbearing age are predominantly affected, regardless of ethnicity. SLE affects the kidneys in about 50% of patients, lupus nephritis (LN) being a major risk factor for overall morbidity and mortality. The lungs are commonly involved later in the course of the disease, pulmonary involvement in SLE being extremely varied. It includes along with pleuritis which is the most common manifestation, acute lupus pneumonitis, pulmonary vasculitis, pulmonary embolism, diffuse alveolar haemorrhage; opportunistic pulmonary infections or drug toxicity could be other pulmonary manifestations. We present the case of a young woman with SLE complicated with lupus nephritis class V and pulmonary infarction due to thromobosis of a pulmonary artery. It is a case of SLE with multiple organ damage and life-threatening complications that require interdisciplinary management and appropriate treatment.

scholarly journals Clinical practice guidance for childhood-onset systemic lupus erythematosus—secondary publication

2021 ◽  
Author(s):  
Syuji Takei ◽  
Toru Igarashi ◽  
Tomohiro Kubota ◽  
Eriko Tanaka ◽  
Kenichi Yamaguchi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Practice ◽  
Survival Rate ◽  
Lupus Erythematosus ◽  
Pediatric Rheumatology ◽  
Organ Damage ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Objective Evidence ◽  
Onset Systemic Lupus Erythematosus

ABSTRACT Childhood-onset systemic lupus erythematosus (cSLE) has been recognised as a more acute and severe autoimmune disease than adult-onset SLE. With the development of medications for the disease and supportive therapy, the mortality rate associated with cSLE has drastically improved; the 10-year survival rate among patients with cSLE between 1995 and 2006 in Japan was 98.3%. However, the 10-year survival rate without any permanent functional impairment remained low at 66.1%. Therefore, the current treatment goal for cSLE is to ensure that they can perform normal daily activities throughout their lives by preventing the occurrence and/or progression of organ damage. For this purpose, appropriate treatments and evaluations are required according to the severity and risk of organ damage; however, there are no established guidelines for cSLE. Therefore, the Pediatric Rheumatology Association of Japan and the Pediatric Rheumatology Subcommittee in the Japan College of Rheumatology developed a comprehensive guidance for clinical practice based on cSLE-related data collected from Japanese national surveys and relevant articles from both domestic and international sources. However, due to the lack of indications for defined and objective evidence quality levels, this guidance should be used on the basis of the judgement of the attending physicians for individual patients.

scholarly journals Childhood-Onset Systemic Lupus Erythematosus: Southeast Asian Perspectives

2021 ◽  
Vol 10 (4) ◽  
pp. 559
Author(s):  
Swee Ping Tang ◽  
Sern Chin Lim ◽  
Thaschawee Arkachaisri
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Economic Status ◽  
Healthcare Access ◽  
Organ Damage ◽  
Southeast Asian ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Childhood onset systemic lupus erythematosus is a rare disease that is more common amongst Southeast Asian children compared to the West. It is typified by a peripubertal onset and a female preponderance, which increases with advancing age. Organs commonly involved at diagnosis include haematological, renal, and mucocutaneous. Fever, malar rash, and cutaneous vasculitis are common. Lupus nephritis is typically proliferative especially Class IV and contributes to both disease activity and damage. Antinuclear antibody and anti-dsDNA positivity are both prevalent in this region. Disease activity is higher than Western cohorts at onset but responds to therapy reducing to low disease activity by six months. However, organ damage occurs early and continues to accumulate over the time, a consequence of both active disease (neurological and renal systems) and steroid-related complications especially in the eye (cataract and glaucoma) and musculoskeletal systems (avascular necrosis). Infections remain the leading cause of death and mortality in this region is highly variable contributed by the heterogeneity in social economic status, healthcare access, and availability of paediatric rheumatology expertise in the region.

scholarly journals Oral lesions as a clinical sign of systemic lupus erythematosus

2018 ◽  
Vol 51 (3) ◽  
pp. 147
Author(s):  
Eliza Kristina M. Munthe ◽  
Irna Sufiawati
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Symptoms ◽  
Organ Damage ◽  
Multiple Organ ◽  
Suspected Case ◽  
Oral Lesions ◽  
Systemic Lupus ◽  
Organ Systems ◽  
High Degree

Background: Oral lesions represent one of the most important clinical symptoms of systemic lupus erythematosus (SLE), an autoimmune disease with a high degree of clinical variability rendering it difficult to arrive at a prompt and accurate diagnosis. There are many unknown causes and multiple organ systems involved, with the result that permanent organ damage may occur before treatment commences. Purpose: The purpose of this case report is to discuss the importance of recognizing the lesions related to SLE which may help dentists to make an early diagnosis. Case: A 17-year-old female patient was referred by the Internal Medicine Department with a suspected case of SLE. Prior to admittance to the hospital, the patient was diagnosed with tuberculosis. A subsequent extraoral examination revealed ulceration with a blackish crust on the upper lip. An intraoral examination showed similar ulceration covered with a blackish crust on the labial mucosa accompanied by central erythema in the hard palate. Blood tests indicated decreased levels of hemoglobin, hematocrit and platelets, but increased levels of leukocytes. A diagnosis of oral lesions associated with SLE and angioedema was formulated. Case management: The patient was given 1% hydrocortisone and vaseline album for extraoral lesions, while 0.2% chlorhexidine gluconate and 0.1% triamcinolone acetonide was used to treat intraoral lesions. An improvement in the oral lesions manifested itself after two weeks of treatment. Conclusion: Early detection of oral lesions plays a significant role in diagnosing SLE. It is important for the dentist to recognize the presentation of diseases that may be preceded by oral lesions. A multidisciplinary approach and appropriate referrals are necessary to ensure comprehensive medical and dental management of patients with SLE.

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