Presentation
Patients typically present with rash, fever, and arthritis, although the presentation may be unpredictable. [pedsinreview.aappublications.org]
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]
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. [merckmanuals.com]
Symptoms and signs are often nonspecific - eg, fatigue (can be severe and debilitating), malaise, fever, splenomegaly, lymphadenopathy, weight loss, arthralgia and fatigue, oral ulcers, photosensitive skin rashes, pleuritic chest pains, headache, paraesthesiae [patient.info]
Entire Body System
- Weight Loss
Fever, weakness, weight loss, and swelling of lymph nodes are several examples. [lecturio.com]
Kidney problems may become apparent when lupus patients feel ill with arthritis, have a rash, fever and weight loss. Less often, kidney disease may occur when there are no other symptoms of lupus. [my.clevelandclinic.org]
B-cell secretion -> formation of immune complexes with deposit in a variety of sites may be triggered by EBV long term risks: OP and CVS disease CLINICAL FEATURES History remitting and relapsing illness malaise, fatigue, myalgia, fever lymphadenopathy weight [lifeinthefastlane.com]
Constitutional symptoms or signs, as used in these listings, means severe fatigue, fever, malaise, or involuntary weight loss. [ssa.gov]
It generally begins with vague symptoms like weakness, joint pain, weight loss, fatigue and/or rashes. After a few months, more serious symptoms may set in. ISN. Personal Stories Lupus Personal Stories. [sclero.org]
- Severe Pain
[…] onset of abdominal pain, inability to bear weight or move a swollen joint due to severe pain, rapid swelling of one or more extremities (arms, legs, hands, or feet ). [emedicinehealth.com]
These NSAIDs may be suitable if your joint or muscle pain is mild. For more severe pain, you will need stronger medication prescribed by your GP. [nhsinform.scot]
In 2010, researchers who published a study on self-reported flares in the journal Rheumatology found that women with lupus report more severe pain and fatigue during menstruation. This suggests that flares may be more likely at this time. [medicalnewstoday.com]
which can vary in severity, is a common symptom in virtually all types of arthritis. [en.wikipedia.org]
- 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]
Jaw & Teeth
- Cheilitis
[…] findings frequently simulate other diseases, chiefly leukoplakia and lichen planus • Lesions usually affect the palate, buccal mucosa, and gingivae • Sometimes they appear as lichenoid reaction • It may also involve vermilion zone of the lower lip lupus cheilitis [slideshare.net]
[…] or ‘butterfly rash ’ ( erythema and oedema of cheeks, sparing nasolabial folds) lasting hours to days Erythematous papular rash on arms, sometimes forming large plaques and spreading widely Photosensitivity (a rash on all recently sun- exposed skin ) Cheilitis [dermnetnz.org]
- Aphthous Ulceration
SLE ulcers are typically seen on the hard palate and should be differentiated from the commonly occurring aphthous ulcers which are painful and are located on the buccal mucosa. 4. [rheumatologyadvisor.com]
ulcers, uveitis, and arthralgia but other systemic signs of SLE are not present. [ncbi.nlm.nih.gov]
ulcers, arthralgia, uveitis Recurrent oral ulcers plus two of the following: eye lesions, genital ulcers, skin lesions Chronic fatigue syndrome Persistent and unexplained fatigue that significantly impairs daily activities Tests to rule out other diseases [aafp.org]
- Aphthous Stomatitis
Cutaneous Manifestations Over 90 percent of patients with systemic lupus erythematosus eventually have a cutaneous manifestation of the disease, including malar rash, discoid lupus erythematosus, alopecia or aphthous stomatitis. [aafp.org]
Cardiovascular
- Thrombosis
Rapaport Thrombosis in patients with the lupus anticoagulant Ann Intern Med, 92 (1980), pp. 156-159 [15.] P. Jungers, F. Liote, M.D. Dauzenberg Lupus anticoagulant and thrombosis in systemic lupus erythematosus [16.] M.L. Boey, C.B. Colaco, A.E. [archbronconeumol.org]
[…] risk of recurrent arterial thrombosis may continue to need higher target ratios of 3.0-4.5. [ncbi.nlm.nih.gov]
High titers of IgG anticardiolipin (>50 IU) indicate high risk of thrombosis. [cancertherapyadvisor.com]
Histopathology can reveille the underlying cause, vasculitis vs. thrombosis, or mixture of both (secondary vasculitis in APS) [9, 28]. [hindawi.com]
Cerebral venography, which is more invasive, is rarely used to demonstrate dural venous sinus thrombosis. [pubs.rsna.org]
Skin
- Skin Disease
National Institute of Arthritis and Musculoskeletal and Skin Diseases. Fast Facts About Lupus. Available online at http://www.niams.nih.gov/Health_Info/Lupus/lupus_ff.asp. Accessed March 2014. MayoClinic.com. Lupus. [labtestsonline.it]
Alternative medications for skin disease include dapsone and retinoic acid (Retin-A). Retin-A is often effective for an uncommon wart-like form of lupus skin disease. [medicinenet.com]
Secondary end points with respect to the glucocorticoid dose and the severity of skin disease, but not counts of swollen and tender joints and the annualized flare rate, also showed a significant benefit with anifrolumab. [nejm.org]
Not all affected individuals display all of these problems A chronic inflammatory connective tissue disease marked by skin rashes, joint pain and swelling, inflammation of the kidneys, inflammation of the fibrous tissue surrounding the heart (i.e., the [icd9data.com]
Musculoskeletal
- Arthralgia
[…] 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]
Arthralgias are usually controlled with NSAIDs. [msdmanuals.com]
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]
- 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]
swelling (arthritis) pleurisy, pericarditis, or inflammation of the pericardium Kidney inflammation with protein and blood in the urine seizures or psychoses (not due to taking medication or metabolic disorders) Unclear persistent decrease in red or [medizinicum.de]
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]
- Morning Stiffness
stiffness Serositis Typical pleurisy for more than 1 day OR pleural effusions OR pleural rub Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day OR pericardial effusion OR pericardial rub OR pericarditis by [rheumtutor.com]
stiffness is a common complaint. 1,11 Jaccoud arthropathy, which is characterized by reducible, nonerosive joint subluxations (eg, swan neck deformities, ulnar deviation, boutonniere deformities, and z-shaped thumbs), can be seen in SLE patients. 3 When [mdedge.com]
General: tiredness, malaise, chronic pain, fever with flares Joints: arthritis or synovitis causing swelling, pain and morning stiffness Lungs: pleurisy or pleural effusions Heart: pericarditis or pericardial effusions Kidneys: protein, casts in urine [dermnetnz.org]
Morning stiffness may also be reported. The severity of the pain ranges from mild to severe. Low-grade fever occurs in 90% of patients. It is usually caused by the inflammatory process of the disease, not by infection. [cancertherapyadvisor.com]
- Back Pain
Read More CE/CME Low Back Pain: Evidence-based Diagnosis and Treatment Author: Mike Roscoe, PhD, MPAS, PA-C Alyssa Nishihira Bs Bshs, PA-S Although accreditation for this CE/CME activity has expired, and the posttest is no longer available, you can still [mdedge.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]
Neurologic
- Neurologic Manifestation
In addition to constitutional symptoms, it most frequently involves the skin and joints, although serositis, nephritis, haematological cytopenias, and neurological manifestations may occur during the course of the disease. [bestpractice.bmj.com]
manifestations has a prognostic value because accrual of major neuropsychiatric events and CNS lesions is associated with increased mortality and morbidity. [medlink.com]
It frequently has been reported as a potential risk factor for stroke and described as a potentially prothrombotic factor. 1 Neurologic manifestations Despite the relatively common involvement of the CNS in SLE, presentation of this disorder with neurologic [epilepsy.com]
Arthritis and cutaneous manifestations are most common, but renal, hematologic and neurologic manifestations contribute largely to morbidity and mortality. [aafp.org]
- Hyperactivity
Lupus erythematosus (LE) is a collection of autoimmune diseases that is the result of immune system hyperactivity and consequent destruction of normal, healthy tissues. [news-medical.net]
In lupus, the body's immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal tissues and organs, including the skin, joints, kidneys, brain, heart, lungs, and blood. [health.ny.gov]
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. [1] Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood [en.wikipedia.org]
Lupus is a long-term autoimmune disease in which the body's immune system becomes hyperactive and attacks normal, healthy tissue. Symptoms include inflammation, swelling, and damage to the joints, skin, kidneys, blood, heart, and lungs. [medicalnewstoday.com]
Faraone and Andras Perl, Brief Report: Attention Deficit and Hyperactivity Disorder Scores Are Elevated and Respond to N‐Acetylcysteine Treatment in Patients With Systemic Lupus Erythematosus, Arthritis & Rheumatism, 65, 5, (1313-1318), (2013). [doi.org]
- Difficulty Concentrating
With central nervous system involvement, problems such as difficulty concentrating and remembering, headaches and moods changes may occur. In these cases, education plans need to be discussed. [printo.it]
[…] or difficulty concentrating, caused by your immune system disorder (including manifestations of the disorder) or its treatment, even if you are able to do some routine activities of daily living. [ssa.gov]
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]
[…] mixed connective tissue disease Sources: Gordon, 2017 ; Lam, 2016 ; Joseph, 2010 ; Karimifar, 2013 Complement Components Increased complement consumption is typical in active SLE, and complement testing is routinely performed as part of the serologic workup [arupconsult.com]
Serum
- Neutropenia
Hair loss (non-scarring alopecia) Blood-related problems, e.g. low red blood cell count (anemia), low white blood cell count (neutropenia) and low platelet cell count (thrombocytopenia). [bangkokhospital.com]
[…] common findings include liver disease, an abnormally large head circumference (macrocephaly), and/or low numbers of circulating blood platelets that assist in blood clotting functions (thrombocytopenia), white blood cells that help fight infection (neutropenia [rarediseases.org]
[…] of the kidneys Hematuria Proteinuria Kidney failure[5] Cutaneous System[edit | edit source] Calcinosis Cutaneous vasculitis Hair loss Raynaud's phenomenon Mucosal ulcers Petechiae Blood Disorders[edit | edit source] Anemia Thrombocytopenia Leukopenia Neutropenia [physio-pedia.com]
Intravenous high-dose pooled gammaglobulin and granulocyte-colony stimulating factor have a role in autoimmune thrombocytopenia and neutropenia. [patient.info]
- Hypoalbuminemia
Poor prognosis has been associated with high serum creatinine levels, hypertension, nephrotic syndrome, anemia, hypoalbuminemia, hypocomplementemia, antiphospholipid antibodies, male sex, ethnicity (African American, Hispanic with mestizo heritage), and [lecturio.com]
• Proteinuria in the nephrotic range without hypoalbuminemia (NP): patients with clinical–serological symptoms compatible with SLE and proteinuria> 3.5 g/24 h, without hypoalbuminemia, and with eGFR> 60 ml/min/1.73 m 2. • Nephrotic syndrome (NS): patients [revistanefrologia.com]
Treatment
However, all forms of the condition may respond to treatment and the passing of time. [dermcoll.edu.au]
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]
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]
All of these drugs have important side effects and should always be discussed with your physician prior to starting treatment. [americanskin.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
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]
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]
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]
The etiology of SLE is not entirely understood; however, there are several predisposing factors that may contribute to the pathogenesis of this disease. [news-medical.net]
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
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]
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]
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]
[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]
• Neuroimaging studies and novel laboratory tests for autoantibodies may aid the diagnosis of neuropsychiatric systemic lupus erythematosus and provide clues for the underlying pathophysiology. • The prevalence of cognitive dysfunction in systemic lupus [medlink.com]
Prevention
Prevention There is no way to prevent SLE since the cause is not known. [winchesterhospital.org]
From these advances, scientists hope to generate early-intervention or even disease-prevention strategies. [lupusresearch.org]
Q: What are some ways a person with lupus could prevent infections, or prevent an existing infection from becoming severe? A: It’s important to catch infections early. [healthline.com]
Prevention Because the cause of SLE is unknown, no one knows how to prevent it. [rheumatology.org]
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]