Presentation
To our knowledge this is the first case of bullous lupus erythematosus with this atypical acral presentation. [ncbi.nlm.nih.gov]
[…] areas (''maculopapular lupus rash'', generalized form); SCLE which presents with red, raised, scaly rash on sun-exposed areas of the body (annular/polycyclic form or papulosquamous form); CCLE which presents either as red to purple, well demarcated, [orpha.net]
We present a case of a 17-year-old adolescent girl who presented with a vesiculobullous eruption without detectable type VII collagen antibodies and without full criteria for SLE. [mdedge.com]
Immune System
- Splenomegaly
[…] neuropathies and thromboembolic disease Ophthalmologic manifestations, particularly keratoconjunctivitis sicca as well as more uncommon manifestations Haematologic manifestations, including cytopenias, thrombophilia, thromboembolic disease, lymphadenopathy and splenomegaly [els.net]
Enlargement of the spleen (splenomegaly) occurs in about 10% of people. [msdmanuals.com]
[…] numbers of special red blood cells (platelets) that assist in blood clotting functions (thrombocytopenia), low levels of other circulating red blood cells (anemia), low levels of certain white blood cells (neutropenia), and abnormally large spleen (splenomegaly [rarediseases.org]
Entire Body System
- Swollen Knee
One person with lupus may have swollen knees and fever. Another person may be tired all the time or have kidney trouble. Someone else may have rashes. Lupus can involve the joints, the skin, the kidneys, the lungs, the heart and/or the brain. [medicinenet.com]
Course
- Severe Clinical Course
Pediatric patients with SLE have a more severe clinical course in comparison with their adult counterparts. Patients typically present with rash, fever, and arthritis, although the presentation may be unpredictable. [pedsinreview.aappublications.org]
Musculoskeletal
- Arthralgia
The most common presenting symptoms are constitutional, such as fatigue, weight loss, and fever without a focal infection, occurring in up to 90% of patients. 9 Other common presenting symptoms include arthralgia and myalgia, which occur in up to 95% [aafp.org]
[…] manifestations, including cutaneous (malar rash, photosensitivity), ocular (keratoconjunctivitis sicca, retinopathy), gastrointestinal (oral ulceration, abdominal pain), cardiac (atherosclerosis, chest pain), pulmonary (serositis, pleurisy), musculoskeletal (arthralgia [orpha.net]
Systemic sclerosis characterizes by decreased joint mobility, arthralgia, myalgia, Raynaud phenomenon, and skin induration. It can distinguish from SLE testing for specific antibodies. [ncbi.nlm.nih.gov]
Patients may be treated symptomatically with nonsteroidal anti- inflammatory drugs (NSAIDs) for the arthralgias, myalgias, fever, and arthritis. Rashes may respond well to antimalarials or topical preparations. [epilepsy.com]
CASE REPORT Six female patients with arthralgias, elevated transaminases and additional symptoms were seen either at the I. [wjgnet.com]
- Joint Swelling
[…] common in patients taking glucocorticoids Raynaud's phenomenon Presentation Symptoms fever pain in multiple joints hip pain (osteonecrosis) Physical exam butterfly malar rash large joint swelling and synovitis hand and wrist manifestation are common [orthobullets.com]
Common symptoms include: severe fatigue joint pain joint swelling headaches a rash on the cheeks and nose, which is called a “butterfly rash” hair loss anemia blood-clotting problems fingers turning white or blue and tingling when cold, which is known [healthline.com]
- Knee Pain
Multiple bone infarcts in a 26-year-old woman with SLE and left knee pain. (a) Anteroposterior radiograph of the left knee shows sclerosis in the distal femur and proximal tibia. [pubs.rsna.org]
Workup
Systemic Lupus Erythematosus (SLE) Workup. (19 September 2016 updated.) Available online at http://emedicine.medscape.com/article/332244-workup. Accessed March 27, 2017. Fonti utilizzate nelle precedenti revisioni (2003 August). [labtestsonline.it]
Systemic Lupus Erythematosus (SLE) Workup. (19 September 2016 updated.) Available online at http://emedicine.medscape.com/article/332244-workup. Accessed March 27, 2017. Sources Used in Previous Reviews (2003 August). [labtestsonline.org]
The focus of this article is ACLE. [2, 3] (See Etiology, History, Physical Examination, and Workup .) Relationship of acute cutaneous lupus erythematosus (ACLE) to systemic disease. LE is lupus erythematosus. [emedicine.medscape.com]
HLA Type
- HLA-DR4
Besides patients with HLA-DR4 make anti-Smith antibodies. [ncbi.nlm.nih.gov]
Treatment
We also reviewed published case reports on skin lesions that developed after HCQ treatments, and propose strategies for early cutaneous eruptions after HCQ treatments. [ncbi.nlm.nih.gov]
Limited treatment options Despite enormous efforts in finding real and conclusive answers regarding the etiology and pathogenesis of systemic lupus erythematosus, treatment is still complex and treatment options remain limited. [adcreview.com]
Other Drugs Used for the Treatment of DLE Methotrexate In 1995, Bottomley and Goodfield22 found that methotrexate may be of help to patients with DLE resistant to conventional treatment; short-term treatment is unlikely to be complicated by any significant [jabfm.org]
Thus, with current knowledge, treatment of APS in the context of SLE should not differ from treatment of primary APS. [ard.bmj.com]
Prognosis
Prognosis • SLE patient prognosis is variable – 5-year survival rate: approx. 82% to 90%; – 20 years survival rate: 63% to 75% – Prognosis depends on which organs are affected and how frequently the disease is reactivated – Common cause of death is renal [slideshare.net]
The Committee on Prognosis Studies in SLE. Arthritis Rheum 1992 ; 35: 630 – 640. Google Scholar | Medline 10. Gladman, D, Ginzler, E, Goldsmith, C. [doi.org]
It is known commonly to affect females but has worse prognosis when males or blacks are affected. [ncbi.nlm.nih.gov]
Damage to the kidneys or nervous system is associated with a poor prognosis! [amboss.com]
Certain patient characteristics portend a worse prognosis in SLE: male sex, age (both young and old), low socioeconomic status, and black race. [visualdx.com]
Etiology
[…] due to the underlying etiology. [icd10data.com]
Etiology Etiology is still elusive but it is thought to be multifactorial, involving a genetic predisposition. [orpha.net]
Etiology The exact etiology is unknown, but several predisposing factors have been identified: Genetic predisposition: HLA-DR2 and HLA-DR3 are commonly present in individuals with SLE Genetic deficiency of classical pathway complement proteins (C1q, C2 [amboss.com]
The etiology and pathology of many diseases have been cleared up and the way leading to the elucidation of many mooted questions pointed out. [jamanetwork.com]
Epidemiology
Epidemiology of systemic lupus erythematosus. Best Pract Res Clin Rheumatol 2002 ; 16: 847 – 858. Google Scholar | Medline | ISI 2. Borchers, AT, Keen, CL, Shoenfeld, Y, Gershwin, ME. [doi.org]
SLE Epidemiology: Epidemiologic Subtypes and Risk Factors for Development Pages 1-12 The Immunopathogenesis and Immunopathology of Systemic Lupus Erythematosus Pisetsky, David S., M.D., Ph.D. [springer.com]
Table of Contents Epidemiology and Diagnosis 1. Epidemiology 2. Disease Activity Measures 3. Disease Outcome 4. Quality of Life and Economic Aspects Pathogenesis 1. Overview of the Pathogenesis of SLE 2. Genetics 3. Hormones 4. [elsevier.com]
Epidemiology Sex: ♀ >> ♂ (10:1) Peak incidence: women aged 20–40 years; no particular age of manifestation in men US prevalence: highest in African-American, Hispanic, and Asian populations References:[1] Epidemiological data refers to the US, unless [amboss.com]
The Congress will span the disciplines dedicated to improving outcomes in SLE, from basic and translational science through epidemiology and outcomes research. Learn More Here [lupusresearch.org]
Pathophysiology
The pathophysiology of SLE involve injury to the bodies own cells leading damaged cell proteins and DNA. Injured cells can subsequently lead to organ injury. [youtube.com]
Medscape Education Rheumatology, September 2017 Concepts in Systemic Lupus Erythematosus: From Pathophysiology to Practice Five leading experts in lupus research and treatment discuss the latest developments. [medscape.org]
PATHOPHYSIOLOGY OF SLE 8. [slideshare.net]
EDUCATIONAL OBJECTIVES • Describe the pathophysiology and explain the various clinical manifestations of systemic lupus erythematosus (SLE). • Define the differential diagnosis for SLE. • List the elements of the laboratory work-up used in the diagnosis [mdedge.com]
[3] Environmental factors: UV light;, stimulation of immune cells through infection with bacteria and viruses (in particular EBV;, which causes disease flares following infection), medications (e.g., procainamide, hydralazine) [4] References:[1][4] Pathophysiology [amboss.com]
Prevention
Prevention There is no way to prevent SLE since the cause is not known. [winchesterhospital.org]
Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can directly act to prevent the blood clots. [medicinenet.com]
Treat flares quickly: This will help prevent serious illness. Prevent a lupus flare: Eat healthy foods: Healthy foods include fruits, vegetables, whole-grain breads, low-fat dairy products, beans, lean meats, and fish. [drugs.com]
Prevent or reduce flares. Help the immune system. Reduce or prevent damage to joints. Balance the hormones. Treat problems related to lupus, such as high cholesterol, high blood pressure, or infection. Alternative treatments may improve symptoms. [niams.nih.gov]
If they take corticosteroids, they should be eating foods low in salt to help prevent high blood pressure and low in sugar to help prevent diabetes and weight gain. [printo.it]