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Weight loss may occur in patients with active SLE. Feb 2004;15(2):241-50. [Full Text]. 164(2):113-5. Philadelphia, Pa: Elsevier Saunders; 2017. Trends in the incidence and mortality of systemic lupus erythematosus, 1950-1992. This book is an invaluable resource for a broad medical audience, from the medical student to the experienced consultant. A quality indicator set for systemic lupus erythematosus. Safety and efficacy of rituximab in systemic lupus erythematosus: results from 136 patients from the French AutoImmunity and Rituximab registry. Lupus peritonitis is a less-common serositis that may be present, even in the absence of ascites. Photosensitive rash is often macular or diffusely erythematous in sun-exposed areas of the face, arms, or hands and generally persists for more than 1 day (see the image below). 2012 Apr. 2016 Apr 12. 18(7):639-44. Dermatology. Weening JJ, D'Agati VD, Schwartz MM, et al. 69(7):1269-74. Curr Opin Rheumatol. 2000 Feb. 39(2):133-41. Dubois' Lupus Erythematosus. Lupus patients may be functionally asplenic and may be at risk for encapsulated bacterial infections such as meningococcemia. Found inside – Page ii- Although the lupus erythematosus is primarily a skin disease, because it can affect other areas such as joints, kidneys, lungs, heart, liver, brain, and blood vessels this book is also useful to specialists in internal medicine. Kuhn A, Landmann A, Wenzel J. J Am Acad Dermatol. Found insideThis book focuses on the clinical evaluation and management of ulcers of the lower limbs. There are numerous causes for ulceration in the lower limbs and each variety requires careful clinical evaluation and management approach. Hidradenomas are usually circumscribed non-encapsulated multilobular tumors, centered on the dermis but sometimes extending into the subcutis. 2014 Sep 12. Always exclude an infectious etiology; patients with SLE are considered immunocompromised and are therefore at higher risk for developing infections and complications. 164(6):1335-41. 2010 Jan. 62(1):211-21. If all the underlying structures are affected, it is called . June 01, 2011 [ MEDLINE Abstract] Letter: Light-emitting diode photomodulation and radiation dermatitis. Factors predictive of serious infections over time in systemic lupus erythematosus patients: data from a multi-ethnic, multi-national, Latin American lupus cohort. [Medline]. It is a butterfly-shaped rash that can be flat or raised over the cheeks and bridge of the nose (see the images below). There are four main types of lupus, but these types of lupus can also have different variants. J Am Acad Dermatol. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Acute psychiatric manifestations in CNS lupus should be considered as a diagnosis of exclusion in an SLE patient. Arthralgia, myalgia, and frank arthritis may involve the small joints of the hands, wrists, and knees (usually symmetrical, polyarticular). The skin is from a patient with systemic lupus erythematosus and shows IgG deposit at 2 different places: the first is a band-like deposit along the epidermal basement membrane ("lupus band test" is positive); the second is within the nuclei of the epidermal cells (anti-nuclear antibodies). Development of autoantibodies before the clinical onset of systemic lupus erythematosus. Novel evidence-based systemic lupus erythematosus responder index. Efficacy of mycophenolate mofetil in antimalarial-resistant cutaneous lupus erythematosus. Lipsker D, Boeckler P, Hauptmann G. Cigarette smoking and lupus erythematosus. [Medline]. Long-term thalidomide use in refractory cutaneous lesions of lupus erythematosus: a 65 series of Brazilian patients. Mesangial proliferative lupus nephritis with moderate mesangial hypercellularity. Found inside – Page vThe Ninth Edition has been radically re-engineered to match the modern day challenges faced by dermatologists. It is published as a combined digital and print resource, but with a new online platform enabling easier and faster navigation. [Medline]. [Medline]. Curr Opin Rheumatol. American College of Rheumatology. [Full Text]. Arthritis Rheum. DLE classically presents with erythematous-to-violaceous, scaly plaques with prominent follicular plugging that often results in scarring and atrophy (see the images below). [Full Text]. Combined oral contraceptives in women with systemic lupus erythematosus. Nodler J, Moolamalla SR, Ledger EM, Nuwayhid BS, Mulla ZD. Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital 8th ed. Sensory or sensorimotor neuropathies occur. Hornberger LK, Al Rajaa N. Spectrum of cardiac involvement in neonatal lupus. Environmental and exposure-related causes of SLE are less clear. This can lead to heart failure, valvular dysfunction, emboli, and secondary infective endocarditis. [Medline]. J Dermatolog Treat. Sheena Desai MD Candidate, Tufts University School of Medicine; MBA Candidate, Brandeis University, The Heller School for Social Policy and Management Kuo CF, Grainge MJ, Valdes AM, See LC, Luo SF, Yu KH, et al. Boddaert J, Huong DL, Amoura Z, Wechsler B, Godeau P, Piette JC. 6(3):248-53. Lupus. Although DLE may occur at any age, it most often develops in persons aged 20-40 years. Philadelphia: Lippincott Williams & Wilkins; 2007:1333-53. A retrospective seven-year analysis of the use of B cell depletion therapy in systemic lupus erythematosus at University College London Hospital: the first fifty patients. 5 (3):48-54. 2015 Sep 7. Arthritis Rheum. N Engl J Med. The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) diagnostic criteria are discussed in Workup. Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Interestingly, in men, SLE is more common in those with Klinefelter syndrome (ie, genotype XXY). Schmajuk G, Schneeweiss S, Katz JN, et al. N Engl J Med. Characteristic hand deformities are swan neck deformities that result from recurrent synovitis and inflammation of the joint capsule, tendons, and ligaments. Accelerated atherosclerosis in systemic lupus erythematosus: implications for patient management. Ann Rheum Dis. Ann Rheum Dis. Cutaneous lupus erythematosus (LE) is a diverse group of autoimmune connective tissue disorders localised to the skin that can be associated with systemic lupus erythematosus (SLE) to varying degrees. Pulmonary embolism, lupus pneumonitis, chronic lupus interstitial lung disease, pulmonary hypertension, complement-mediated pulmonary leukoaggregation, alveolar hemorrhage, or infection may be related to lupus disease. [Medline]. Early Life Factors Associated with Adult-Onset Systemic Lupus Erythematosus in Women. [Medline]. Lupus in lupus pernio is misleading because it suggests a connection with systemic lupus erythematosus; lupus pernio is actually a finding of the skin in sarcoidosis. 16(2):281-91. Links between complement abnormalities and systemic lupus erythematosus. Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA Jr, Jansen-McWilliams L, et al. 2012 Nov. 71 (11):1833-8. The American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) published new criteria for the classification of SLE in 2019. Acute viral infections in patients with systemic lupus erythematosus: description of 23 cases and review of the literature. Offers information on the symptoms, diagnosis, medications, side effects, alternative treatments of lupus along with advice on coping with lupus. The risk of progression to SLE in patients with DLE has been demonstrated to be higher than previously reported. [102], Source  (except as noted otherwise) :  Weening JJ, D'Agati VD, Schwartz MM, et al. Found insideSome dermatologic conditions are seen predominantly or only in richly pigmented skin. Ethnic Dermatology: Principles and Practice provides a practical approach to the dermatology of non-white skin. HCQ is recommended for all patients with LE in discoid LE (DLE), sub-acute LE (SCLE), lupus panniculitis (profundus), and SLE. Medscape Education, Appropriate Use of Glucocorticoids in the Treatment of SLE, 2002 [Medline]. Balluz L, Philen R, Ortega L, Rosales C, Brock J, Barr D, et al. 2011 Sep. 70(9):1569-74. 2010 Jun. Atra E, Sato EI. Eur J Dermatol. 377(9767):721-31. 1992 Sep 26. Helper (CD4+) T cells are increased. [Image from Wikipedia: http://en.wikipedia.org/wiki/File:Histology-kidney.jpg], Table 1. Ginzler EM, Wofsy D, Isenberg D, Gordon C, Lisk L, Dooley MA. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. N Engl J Med. Clin Exp Rheumatol. J Rheumatol. 2016 Apr. Gordon H, Chandran A, Vandal AC, Yung A, Jarrett P. The relationship between disease severity and quality of life in discoid lupus erythematosus. 6:22341. Petri M, Kim MY, Kalunian KC, Grossman J, Hahn BH, Sammaritano LR, et al. [Medline]. Estrogen use in postmenopausal women appears to increase the risk of developing SLE. [Full Text]. [Medline]. 32(7):1877-84. [1] : In adults, Raynaud pleuritis and sicca are twice as common as in children and adolescents. Arthritis Rheum. A case-control analysis of the teratogenic effects of co-trimoxazole. Mortality and causes of death in systemic lupus erythematosus. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cDovL2VtZWRpY2luZS5tZWRzY2FwZS5jb20vYXJ0aWNsZS8zMzIyNDQtb3ZlcnZpZXc=, Kidney involvement, proteinuria, urinary cellular casts, Constitutional (eg, fatigue, fever, arthralgia, weight changes), Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis, avascular necrosis), Dermatologic (eg, malar rash, photosensitivity, discoid lupus), Renal (eg, acute or chronic kidney failure, acute nephritic disease), Neuropsychiatric (eg, seizure, psychosis), Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, interstitial lung disease), Gastrointestinal (eg, nausea, dyspepsia, abdominal pain), Hematologic (eg, cytopenias such as leukopenia, lymphopenia, anemia, or thrombocytopenia), Corticosteroids (eg, methylprednisolone, prednisone), short-term use recommended, Nonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine, Nonsteroidal anti-inflammatory drugs (NSAIDS; eg, ibuprofen, naproxen, diclofenac), Biologic DMARDs (disease-modifying antirheumatic drugs): Belimumab, rituximab, anifrolumab, and/or IV immune globulin, Childhood exposure to agricultural pesticides, Lipid disorders (dyslipidemia), atherosclerosis, and coronary heart disease, Bone-related conditions: Osteoporosis; avascular bone necrosis, Malignancies (eg, non-Hodgkin lymphoma, lung cancer, hepatobiliary cancer). [Medline]. 2009 Apr 15. [Full Text]. [Medline]. Immune complexes form in the microvasculature, leading to complement activation and inflammation. Systemic lupus erythematosus (SLE) is the prototypic systemic autoimmune disease characterized by diverse multisystem involvement and the production of an array of autoantibodies. 1990. 123 (6):1386-94. Prystowsky SD, Gilliam JN. Special consideration should be given to infectious causes (bacterial, viral [eg, CMV]), because of immunosuppression. Other forms of chronic cutaneous lupus include lupus profundus, chilblain lupus, and lupis tumidus. [82]  is characterized by erythema over the cheeks and nasal bridge (but sparing the nasolabial folds, which is in contrast to the rash of dermatomyositis) (see the image below). 2011 Oct. 20(10):1090-4. Its presentation and course are highly variable, ranging from indolent to fulminant. J Am Coll Cardiol. Long AA, Ginsberg JS, Brill-Edwards P, et al. [Medline]. J Rheumatol. [Full Text]. Curr Opin Rheumatol. 1345-67. 2016. 2010 Jan. 49(1):128-40. [Medline]. Lupus panniculitis: also called lupus profundus; may develop in normal skin or preexisting skin lesion of patient with systemic or discoid lupus erythematosus Physical and factitious panniculitis: due to blunt force, pinching, cold or injection of foreign substances for secondary gain; a diagnosis of exclusion [52], Worldwide, the prevalence of SLE appears to vary by race. VII [correction of VIII]. Genetic susceptibility to systemic lupus erythematosus in the genomic era. [Full Text]. [Medline]. Available at https://www.lupus.org/understanding-lupus-0?utm_expid=.7AO1HbsrR9KO2Z8jF1HYTw.1&utm_referrer=. [Medline]. Munoz LE, Gaipl US, Franz S, Sheriff A, Voll RE, Kalden JR, et al. Isenberg D, Appel GB, Contreras G, et al. [Medline]. 2011 Nov 17. Available at https://www.lupus.org/resources/what-causes-lupus. The chest x-ray from a patient with lupus demonstrates a right-sided pleural effusion (yellow arrow) and atelectasis with scarring in the left lung base (blue arrow). Arthritis Rheum. 2004 Dec. 44(6):1050-9. Helmick CG, Felson DT, Lawrence RC, Gabriel S, Hirsch R, Kwoh CK, et al. Accessed: August 4, 2021. 48(7):716-20. 377(9767):721-31. A postdiagnostic 5-year follow-up study showed that males had a higher prevalence of thromboses, nephropathy, strokes, gastrointestinal symptoms, and antiphospholipid syndrome and that females were more likely to present with arthralgia, hair loss, Raynaud syndrome, and photosensitivity. [Medline]. Report of an association between discoid lupus erythematosus and smoking. Kreuter A, Tomi NS, Weiner SM, Huger M, Altmeyer P, Gambichler T. Mycophenolate sodium for subacute cutaneous lupus erythematosus resistant to standard therapy. Petri M, Orbai AM, Alarcón GS, et al. in a genetic background predisposing to autoimmunity. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Yazdany J, Panopalis P, Gillis JZ, Schmajuk G, MacLean CH, Wofsy D, et al. 2017 Jul 21. Abdominal ultrasound can be a reliable first-line diagnostic tool in lupus enteritis, aiding early diagnosis of potentially life-threatening complications. Epidemiology of systemic lupus erythematosus and cutaneous lupus erythematosus in a predominantly white population in the United States. 61(3):370-7. Although the interphalangeal spaces are affected, the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints are spared. 10:133. . Bertsias G, Fanouriakis A, Boumpas DT. Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology FoundationDisclosure: Nothing to disclose. 7:103. Arbuckle MR, McClain MT, Rubertone MV, et al. 2017 Jun 1. Somers EC, Marder W, Cagnoli P, Lewis EE, DeGuire P, Gordon C, et al. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, with multisystemic involvement. 2010 Jan. 49(1):128-40. 2019 Sep. 71 (9):1400-1412. [68]. Acta Reumatol Port. Acute kidney injury or chronic kidney disease may cause symptoms related to uremia and fluid overload. Arthritis Care Res (Hoboken). A retrospective seven-year analysis of the use of B cell depletion therapy in systemic lupus erythematosus at University College London Hospital: the first fifty patients. The Rheumatologist. [78] In the Michigan Lupus Epidemiology and Surveillance program, the proportion of patients with kidney disease was 2.2-fold higher, and that of progression to end-stage kidney disease was 3.4-fold higher, in blacks than in whites. Characterized by remission and flares, lupus profundus . Rheumatology (Oxford). Mittal B, Hurwitz S, Rennke H, Singh AK. Hwang J, Kim HJ, Oh JM, et al. [Medline]. Salman-Monte TC, Torrente-Segarra V, Almirall M, Corzo P, Mojal S, Carbonell-Abelló J. Numerous studies have investigated the role of infectious etiologies that may also perpetuate autoimmunity. [Full Text]. Scand J Immunol. 2013. Initial disease severity, cardiovascular events and all-cause mortality among patients with systemic lupus erythematosus. The book is divided into eight sections; the first deals with ‘normal flora’ of the skin (microorganisms present under normal circumstances, without causing disease), whilst each subsequent chapter covers a specific type of ... Efficacy and safety of rituximab in patients with active proliferative lupus nephritis: the Lupus Nephritis Assessment with Rituximab study. 340(8822):741-5. The discoid rash can present as erythematous patches with keratotic scaling over sun-exposed areas of the skin. Fessler BJ. Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. It is a chronic condition in which the body attacks in own cells and tissues, resulting in inflammation of the skin, joints, blood vessels, brain, lungs, and kidneys. Malar rash is a fixed erythema that typically spares the nasolabial folds. Photosensitivity in SLE may be either acute or chronic. The skin is from a patient with systemic lupus erythematosus and shows IgG deposit at 2 different places: the first is a band-like deposit along the epidermal basement membrane ("lupus band test" is positive); the second is within the nuclei of the epidermal cells (anti-nuclear antibodies). Deficits below a dermatomal level or spastic paraparesis should raise consideration of transverse myelitis. Chronic cutaneous lupus erythematosus (CCLE) can be further divided into three main types: discoid lupus erythematosus (DLE), tumid lupus, and lupus panniculitis. Lupus erythematosus is a polygenic autoimmune disease linked to various HLA subtypes, immune signaling, and environmental factors, which ultimately leads to autoantibody production and T-cell dysfunction. Vinet E, Clarke AE, Gordon C, Urowitz MB, Hanly JG, Pineau CA, et al. J Rheumatol. Although only approximately 50% of patients with SLE develop clinically evident kidney disease, biopsy studies demonstrate some degree of renal involvement in most patients. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system. [Full Text]. Rerknimitr P, Tekacharin N, Panchaprateep R, Wititsuwannakul J, Tangtanatakul P, Hirankarn N, et al. [Medline]. Alarcón GS, McGwin G, Bertoli AM, Fessler BJ, Calvo-Alén J, Bastian HM, et al. [Full Text]. 28(4):761-5. [98] Headache is the most commonly seen CNS finding in SLE, occurring in 39-61% of adults and 72% of children, 1368-88. Cooper GS, Dooley MA, Treadwell EL, St Clair EW, Parks CG, Gilkeson GS. [Medline]. 69 (5):1016-1027. [Full Text]. HARVEY AM, SHULMAN LE, TUMULTY PA, CONLEY CL, SCHOENRICH EH. 2011 Jan. 38(1):60-3. Der Lupus erythematodes hypertrophicus et profundus geht mit polygonalen violetten Plaques einher, die bevorzugt die Lippen, die Kopfhaut und die Akren befallen (s. [Medline]. [Medline]. 67 (6):817-28. [Medline]. The chronic steroids prescribed to patients also place them at increased risk for atypical infections. [Medline]. Translocation of a gut pathobiont drives autoimmunity in mice and humans. Lupus erythematosus profundus and enophthalmos. June 01, 2011 [ MEDLINE Abstract] A pigmented optic disc. [Full Text]. Prognostic factors from the 2008 European League Against Rheumatism (EULAR) recommendations included the following 2011 Jun. 2016 Jul. [Full Text]. Symptoms include inflammation of different parts of the body including the lungs, heart, liver, joints and kidneys. Lupus is a complicateddisorder that requires the regular care of a qualifiedphysician. Jennekens FG, Kater L. The central nervous system in systemic lupus erythematosus. J Rheumatol. Young KA, Munroe ME, Guthridge JM, Kamen DL, Niewold TB, Gilkeson GS, et al. International Society of Nephrology/Renal Pathology Society 2003 class III (×200, immunofluorescence). 2016 Jan 12. Part 2. [Medline]. Discoid lupus erythematosus is known to negatively impact patient quality of life. Arthritis Rheum. 2001 Apr. [Medline]. 2011 Sep-Oct. 29(5 Suppl 68):S126-9. Sle are less common are seen predominantly or only in richly pigmented skin deficits may represent discoid lupus erythematosus a! 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