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SLE angioedema

P Kolkhir, D Pogorelov, O Olisova, M Maurer
Chronic spontaneous urticaria (CSU) is a common mast cell-driven disease characterized by the development of wheals (hives), angioedema (AE), or both for > 6 weeks. It is thought that autoimmunity is a common cause of CSU, which is often associated with autoimmune thyroiditis, whereas the link to other autoimmune disorders such as systemic lupus erythematosus (SLE) has not been carefully explored. Here, we systematically reviewed the existing literature for information on the prevalence of CSU in SLE (and vice versa) and we examined the possible clinical and pathogenetic relationship between CSU and SLE...
February 2016: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
Zahra Habibagahi, Jamshid Ruzbeh, Vahide Yarmohammadi, Malihe Kamali, Mohammad Hassan Rastegar
Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule...
July 2015: Iranian Journal of Medical Sciences
Daniel Bienstock, Louis Mandel
Non-medication-related acquired deficiencies of C1 esterase inhibitor (C1-INH) can cause the facial acquired angioedema (AAE) seen in systemic lupus erythematosus (SLE). The defect can originate from a lymphoproliferative disease (LPD) that catabolizes C1-INH or from circulating antibodies that inactivate C1-INH. This report describes a third and rare variety of facial AAE originating in SLE in which there was no LPD or circulating antibodies to impede C1-INH activity.
May 2015: Journal of Oral and Maxillofacial Surgery
Irène Gallais Sérézal, Laurence Bouillet, Robin Dhôte, Stéphane Gayet, Pierre-Yves Jeandel, Claire Blanchard-Delaunay, Ludovic Martin, Arsène Mekinian, Olivier Fain
Hereditary angioedema (HAE) is a rare genetic disorder that is primarily caused by a defect in the C1 inhibitor (C1-INH). The recurrent symptoms are subcutaneous edema and abdominal pain. Laryngeal edema, which can also occur, is life threatening if it goes untreated. HAE can be associated with some inflammatory and autoimmune disorders, particularly lupus. The aim of this study was to describe cases of lupus among HAE patients in France and to perform a literature review of lupus and HAE studies. Case detection and data collection (a standardized form) were performed, thanks to the French Reference Center for Kinin-related angioedema...
June 2015: Autoimmunity Reviews
John Nay, Christine O Menias, Vincent M Mellnick, Dennis M Balfe
Systemic diseases have many different presentations, including imaging findings in the bowel. Recognizing the imaging findings in these diseases is important in making the correct diagnosis. Although certain imaging features overlap, knowledge of specific findings along with the clinical presentation aid in narrowing the differential or in making an imaging diagnosis. The pictorial review will focus on the gastrointestinal manifestations of systemic diseases, including amyloidosis, angiotensin converter enzyme inhibitor-induced angioedema, celiac sprue, distal intestinal obstruction syndrome, graft-versus-host disease, hemolytic uremic syndrome, hemophilia, Henoch-Schönlein purpura, intestinal lymphangiectasia, mastocytosis, scleroderma, systemic lupus erythematosus, Wegener's granulomatosis, and Whipple's disease...
August 2015: Abdominal Imaging
Abhijeet Danve, Lisa Perry, Atul Deodhar
BACKGROUND: Pregnancy can lead to flares in systemic lupus erythematosus (SLE), and the presence of SLE in pregnancy could lead to a poor outcome for the mother and the fetus. OBJECTIVE: To describe a patient whose active SLE (including lupus nephritis) was managed with the use of belimumab throughout pregnancy. METHODS: A case report and review of relevant literature is presented. RESULTS: A 38-year-old Caucasian woman with SLE was seen for advice regarding planning a pregnancy and management of her active lupus (cutaneous lupus, angioedema, lupus nephritis, leukopenia, and anti-phospholipid antibody syndrome) that could only be controlled by mycophenolate, a drug contraindicated in pregnancy...
October 2014: Seminars in Arthritis and Rheumatism
Ahmed Mahfouz, Ahmed Naguib Mahmoud, Patel Ahmad Ashfaq, Khalid Hamed Al Siyabi
PATIENT: Female, 75 FINAL DIAGNOSIS: Hypertensive crisis with multi organ failure Symptoms: Anemia • general weakness • hypokalemia • nausea • tachycardia MEDICATION: - Clinical Procedure: - Specialty: Cardiology. OBJECTIVE: Unexpected drug reaction. BACKGROUND: Skin reactions are common adverse drug reactions and may include angioedema, erythroderma, Stevens-Johnson syndrome, and toxic epidermal necrolysis (TEN)...
2014: American Journal of Case Reports
Getaw Worku Hassen, Ting Jia Tu, Daniel Hsiang Wei, Albert Hwang, Romus Lamothe, Ana Costea, Lydia Liyun Liu, Tennyson Smith, Felicia Mualim, Paul Johnston, Jennifer Ming Wai Ng, Shakeel Usmani, Hossein Kalantari
BACKGROUND: Approximately 2% of angioedema (AE) patients have a hereditary or an acquired deficiency of the complement 1 (C1) esterase inhibitor (C1 INH) gene. Some case reports indicate an association between angiotensin-converting enzyme inhibitor (ACEI) use and exacerbation of hereditary AE (HAE). OBJECTIVE: The aim of this retrospective study is to investigate the association between HAE and ACEI use in a larger patient population. METHODS: A retrospective chart review of patients who presented with AE and patients with diagnostic serum assays for functional C1 INH, C1 INH antigenic protein, C1q, C1q immune complex (C1q IC), and complement 4 (C4) regardless of medical complaint...
October 2013: Journal of Emergency Medicine
Carlo Perricone, Netta Shoenfeld, Nancy Agmon-Levin, Caterina de Carolis, Roberto Perricone, Yehuda Shoenfeld
The sense of smell is an ancient sensory modality vital for sampling and perceiving the chemical composition of surrounding environments. Olfaction involves a pathway of biochemical and electrophysiological processes, which allows the conversion of molecular information into sensations. Disturbances in the olfactory function have been investigated mainly in neurological/neurodegenerative disorders such as Alzheimer's and Parkinson's diseases; impaired sense of smell has been associated with depressed mood. Only recently, smell capability was tested in other diseases, particularly autoimmune diseases...
August 2013: Clinical Reviews in Allergy & Immunology
C Chattopadhyay, N Chakrabarti
BACKGROUND: Cutaneous drug reactions are a common impediment in therapy, the incidence ranging from 2% to 8%. This cross-sectional study was designed to compare different trends of cutaneous drug reaction in two different socio-economic groups of patients in the same region. AIMS: The aim was to evaluate common drugs implicated in causing reactions, describe the adverse cutaneous drug reactions, study the characteristics of patients presenting with the reactions...
April 2012: Nigerian Journal of Clinical Practice
V Kotnik
Complement is one of the most important mechanisms of natural resistance preventing infections in humans and animals. It is actively involved in the pathogenesis of several diseases, including skin diseases, characterized by the presence of autoantibodies, foreign microorganisms, altered tissue cells, and the presence of mannan. Complement is intended to kill invading microorganisms but it can also destroy the organism's own damaged or altered cells. It is characterized by vigorous activity and is also potentially harmful for the host if triggered in its own body...
2011: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
Vilson Furlanetto, Karina de Souza Giassi, Fabrício de Souza Neves, Adriana Fontes Zimmermann, Gláucio Ricardo Werner Castro, Ivânio Alves Pereira
Acquired angioedema is caused by different drugs and lymphoproliferative diseases, and rarely it has also been related to the presence of auto-immune disorders. We report the case of a 47 year old female with systemic lupus erythematosus (SLE) and severe cutaneous involvement who developed recurrent localized angioedema of the face, including lips and eye lids, upper limbs, and thorax, not associated with urticaria, and with reduced levels of C1 esterase inhibitor. Treatment with antimalarials, glucocorticoids, and pulse therapy with methylprednisolone associated with azathioprine did not improve her condition...
January 2010: Revista Brasileira de Reumatologia
D Lipsker, G Hauptmann
In this review we address the main cutaneous manifestations and diseases associated with deficiencies in components of the complement system. The first part is devoted to hereditary angioedema, in which acute, sometimes life-threatening recurrent attacks of acute swelling, usually associated with gastrointestinal symptoms, occur. It is related to a structural or functional deficiency of C1 esterase inhibitor. Patients usually have lowered C4 levels, and diagnosis relies on determination of antigenic and/or functional C1 inhibitor level...
August 2010: Lupus
C Perricone, N Agmon-Levin, N Shoenfeld, C de Carolis, M D Guarino, G Gigliucci, I Milana, L Novelli, G Valesini, R Perricone, Y Shoenfeld
BACKGROUND: Hereditary angioedema (HAE) is an autosomal-dominant disorder resulting from C1-inhibitor (C1INH) deficiency. Smell impairments were found in patients affected with systemic lupus erythematosus, that, similarly to HAE, is characterized by the activation of the classical complement pathway with C4 consumption. In this study, we aimed at evaluating the sense of smell in patients with HAE. METHODS: Thirty patients with HAE and 30 healthy age- and sex-matched controls were evaluated for olfactory functions using the 3-stages Sniffin'-Sticks kit (threshold, discrimination, and identification [TDI])...
January 2011: Allergy
M Nittner-Marszalska, M Krasnowska, K Solarewicz-Madejek, D Jedrzejuk, A Szuba
Melkersson-Rosenthal syndrome (MRS) is an idiopathic, rare disorder manifested by facial swelling, congenital plicated tongue and recurrent peripheral facial nerve palsy. Labial involvement alone is referred to as cheilitis granulomatosa. Differential diagnosis of MRS includes allergic angioedema, bacterial, viral or filarial infections as well as autoimmunological inflammation in the course of systemic lupus erythematosus, dermatomyositis, and others. We present 4 patients who experienced periodically painless edema of the face and/or lips...
March 2010: Lymphology
Kanokvalai Kulthanan, Meethawee Cheepsomsong, Sukhum Jiamton
Out of 64 patients diagnosed with urticarial vasculitis (UV), 49 (76.6%) presented with their first attack of UV. The others experienced recurrent attacks with a mean number of 3.3 past recurrences. Fifteen patients had angioedema (23.4%) and 16 (25%) suffered systemic involvement. The most common abnormal laboratory finding was an increased erythrocyte sedimentation rate. Six of 62 patients (9.7%) had decreased C3 levels. A cause could be identified in 19 patients (29.7%). The most common identified cause was infection; other causes included drugs, malignancy and systemic lupus erythematosus (SLE)...
June 2009: Asian Pacific Journal of Allergy and Immunology
Young Hee Rho, Jin-Hyun Woo, Seong Jae Choi, Young Ho Lee, Jong Dae Ji, Gwan Gyu Song
Lupus is a systemic autoimmune disease of an unknown origin, and systemic lupus erythematosus (SLE) can be triggered by numerous stimuli. Bee venom therapy is an alternative therapy that is believed to be effective for various kinds of arthritis. We present here a case of a 49-year-old female who experienced a new onset lupus after undergoing bee venom therapy, and this looked like a case of angioedema. The patient was successfully treated with high dose steroids and antimalarial drugs. We discuss the possibility of bee venom contributing to the development of SLE, and we suggest that such treatment should be avoided in patients with lupus...
September 2009: Korean Journal of Internal Medicine
Carine Kieffer, Bernard Cribier, Dan Lipsker
We conducted the current study to define within the spectrum of the neutrophilic dermatoses a group of patients with an urticarial rash clinically and a neutrophilic dermatosis histopathologically. We reviewed the literature on neutrophilic urticaria and we report here a series of patients with this unique presentation. We reviewed all cutaneous biopsies submitted to our department between 2000 and 2006 in which histopathologic evaluation was compatible with this entity. We then retrieved the patient medical records and obtained information about follow-up and associated diseases...
January 2009: Medicine (Baltimore)
S M Weimer, S D Levine, A Gedalia
We describe a 10(1/2)-year-old black female who had severe angioedema, possibly triggered by penicillin, with respiratory distress as the initial presentation of systemic lupus erythematosus. In this patient, the Cl esterase inhibitor quantitative and functional serum levels were normal. Although there are several case reports with similar presentations in adults, this is the first case report in the pediatric age group.
August 1995: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
A-S Frot, S Barbarot, S Poignant, C Guyot, J-F Stalder
BACKGROUND: Hypocomplement urticarial vasculitis syndrome may be the presenting sign of systemic lupus erythematosus. Hypocomplement urticarial vasculitis presents as atypical urticaria associated in 50% of cases with angioedema. On laboratory investigation, hypocomplementaemia is the characteristic feature, with reduced C3, C4 and C1q. This disease is very rare in children. PATIENTS AND METHODS: An eight-year-old girl was hospitalised for relapsing urticaria with ecchymotic angioedema present for one year, in a setting of impaired general health and fever...
August 2008: Annales de Dermatologie et de Vénéréologie
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