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Acquired Angioedema

RAE


Presentation

  • Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues.[ncbi.nlm.nih.gov]
  • We present a case of MCD in an HIV-positive patient that is unusual on two counts: our patient's MCD first presented in the context of an immune restoration inflammatory syndrome (IRIS), following the initiation of highly active antiretroviral therapy[ncbi.nlm.nih.gov]
  • In the present paper, we report the case of a male patient with symptoms of AAE and recurrent deep venous and arterial thrombosis.[ncbi.nlm.nih.gov]
  • CASE REPORT: A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody.[ncbi.nlm.nih.gov]
  • Abstract An unusual case of angioedema is presented. The initial presentation occurred at 84 years-of-age without a family history of this condition.[ncbi.nlm.nih.gov]
Lymphocyte Disorder
  • This disruption can be caused by a B-cell lymphocyte disorder, an autoimmune disease, or cancer. In this type, the swellings can occur several years before other symptoms, making the cause difficult to determine.[angioedemanews.com]
Orthopnea
  • Case Report Clinical history A 45 yo male presented to the Emergency Room with dyspnea, orthopnea, fatigue, and lower extremity edema. Medical history included hypertension, treated with lisinopril.[alliedacademies.org]
Abdominal Pain
  • INTRODUCTION: Acquired angioedema (AAE), an acquired deficiency of C1esterase inhibitor, is a medically treatable condition which can cause severe abdominal pain mimicking an acute surgical abdomen.[ncbi.nlm.nih.gov]
  • Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease.[ncbi.nlm.nih.gov]
  • Abstract Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema.[ncbi.nlm.nih.gov]
  • Sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract.[checkorphan.org]
  • People with AAE can have edema of the lining of the digestive tract, which can cause abdominal pain and nausea, as well as edema of the upper airway, which can be life-threatening. [1] Swelling episodes may have various triggers, such as mild trauma ([rarediseases.info.nih.gov]
Nausea
  • People with AAE can have edema of the lining of the digestive tract, which can cause abdominal pain and nausea, as well as edema of the upper airway, which can be life-threatening. [1] Swelling episodes may have various triggers, such as mild trauma ([rarediseases.info.nih.gov]
  • Severe swelling in the intestines or digestive system can cause nausea, vomiting, and intense pain in the abdomen. When severe swellings occur in the mouth or throat, they can block the airways and make it difficult to breathe.[angioedemanews.com]
  • The GI tract is often involved, with manifestations that suggest intestinal obstruction, including nausea, vomiting, and colicky discomfort.[merckmanuals.com]
  • Associated symptoms include nausea, vomiting, anorexia, and even distention. Some patients present with diarrhea alone. Rarely, obstruction or toxic megacolon occurs. Physical examination findings vary from mild tenderness to peritoneal signs.[mdmag.com]
  • […] tissues occur throughout the body without the presence of urticaria Typically involves arms, legs, hands, trunk, face, mouth, larynx, airway, genitals, and tongue Gastrointestinal tract often involved, with recurrent episodes of cramping, abdominal pain, nausea[arupconsult.com]

Workup

  • A literature review was completed for the purpose of assessing the diagnostic value of C1q when used in the workup of AAE.[ncbi.nlm.nih.gov]
  • Workup reveal low complements 3 and 4 (C3, C4), low normal C1q level, elevated ESR, and cryoglobulin which favor the diagnosis of acquired angioedema (AAE).[shmabstracts.com]
  • Although no association between the CLL and angioedema was initially recognized, further workup showed her to have low C1-INH levels. Chemotherapy helped prevent subsequent episodes, but three years later she redeveloped angioedema.[ecancer.org]
  • Workup for AAE included evaluation for both lymphoproliferative and autoimmune disease.[hoajonline.com]
Albuminuria
  • UPEP showed albuminuria, a kappa band, likely a Bence Jones protein, and increased gamma staining. Serum free kappa light chains resulted 408.43 mg/L, free lambda 14.02 mg/L, ratio of kappa to lambda of 29.13.[alliedacademies.org]
Ventricular Hypertrophy
  • Trans-thoracic echocardiogram demonstrated left ventricular hypertrophy, with heavy trabeculations, ejection fraction of 41%, and moderately enlarged left atrium. Cardiac MRI failed to meet criteria for non-compaction cardiomyopathy and amyloidosis.[alliedacademies.org]

Treatment

  • CONCLUSION: In our experience, rhC1INH is a beneficial treatment for patients with preference for a C1INH that is not plasma derived and it is suitable for home treatment.[ncbi.nlm.nih.gov]
  • BACKGROUND: Administration of C1-inhibitor concentrate is effective for prophylaxis and treatment of severe angioedema attacks caused by C1-inhibitor deficiency.[ncbi.nlm.nih.gov]
  • C1-INH concentrates and attenuated androgens are used for short-term prophylactic treatment. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics.[ncbi.nlm.nih.gov]
  • Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and[ncbi.nlm.nih.gov]
  • If angioedema is poorly controlled with standard treatment regimens, we suggest treatment of the associated haematologic disorder.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis The prognosis depends on the risk of developing hematological manifestations. Rituximab can be an alternative treatment in cases of AAE associated with C1-INH antibodies.[orpha.net]
  • Prognosis - Acquired angioedema Treatment - Acquired angioedema Different drugs are available for the treatment of acquired angioedema.[checkorphan.org]
  • The prognosis of all types of angioedema is generally good with treatment. However, with acquired angioedema, an underlying immune system problem is usually present, causing the swellings and affecting the individual’s overall health.[angioedemanews.com]
  • Although prognosis remains uncertain, some cases demonstrate a response to these therapies with preserved renal function over several years. References Katz A, Zent R, Bargman JM. IgG heavy-chain deposition disease. Mod Pathol. 1994;7(8):874-8.[alliedacademies.org]

Etiology

  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • Etiology The edemas are triggered by increased permeability of the blood vessels in response to elevated levels of bradykinin as a result of the C1-INH deficiency.[orpha.net]
  • Hence, etiology of AAE was determined to be IgM-monoclonal gammopathy of undetermined significance (MGUS).[hoajonline.com]
  • […] defect was isolated, 5 giving rise to the description of two main syndromes: Hereditary Angioedema (HAE), and the even rarer form of Acquired Angioedema (AAE). 6 Although there are multiple classifications and sub-classifications based on the underlying etiology[oralhealthgroup.com]
  • Despite the existence of a myriad of etiologies, most cases of angioedema remain idiopathic. Acquired angioedema has a high correlation with malignancies.[mdmag.com]

Epidemiology

  • Summary Epidemiology Prevalence is unknown. Clinical description Onset most commonly occurs after 50 years of age.[orpha.net]
  • […] typically the face, intestinal walls, and larynx, leading to transient disfigurement, colicky abdominal pain, and airway obstruction. 8 Edema episodes may occur spontaneously and unexpectedly, with triggering events varying widely among those affected. 2 Epidemiology[oralhealthgroup.com]
  • […] lymphomas) Autoimmune disease Neoplasia Urticarial vasculitis associated with angioedema Idiopathic or cold-induced angioedema Cellulitis Parasitic infection (eg, Trichinella spp) Autoimmune conditions Laryngeal edema – peritonsillar abscess Background Epidemiology[arupconsult.com]
  • Collegium antropologicum 35 (3), 781-785, 2011 11 2011 Epidemiology and clinical presentation of inflammatory bowel disease in Zadar County, Croatia I Klarin, L Majnarić, Ž Jovanović, D Nakić, I Včev, A Včev Collegium antropologicum 37 (4), 1161-1170,[scholar.google.hr]
Sex distribution
Age distribution

Pathophysiology

  • This review summarizes the clinical aspects, pathophysiology, and management of AAE compared with the types of hereditary angioedema.[ncbi.nlm.nih.gov]
  • Future research needs to address its underlying pathophysiologic mechanisms and its clinical significance in treatment and course of pSLE.[acrabstracts.org]
  • Supporting the importance of bradykinin in acquired angioedema, vascular permeability has been shown to increase in mice deficient in C1-INH, but not in mice with a deficiency in both C1-INH and the bradykinin B2 receptor. [3] The precise pathophysiology[emedicine.medscape.com]
  • While the exact pathophysiology remains incompletely understood, it is important to note that histamine and other mast-cell mediators typical of allergic edema are not involved, accounting for the general ineffectiveness of antihistamines, corticosteroids[oralhealthgroup.com]
  • The pathophysiology of hereditary angioedema. Clin Immunol. 2004;114:3-9. Bower T, et al., Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004;114:629-37.[rarediseases.org]

Prevention

  • CLINICAL IMPLICATIONS: Self-administration of C1-inhibitor concentrate could be a valuable and convenient treatment modality to prevent or treat angioedema attacks in patients with C1-inhibitor deficiency.[ncbi.nlm.nih.gov]
  • CONCLUSION: Wider appreciation of the possibility of AAE, particularly in patients with lymphoproliferative disorders, could lead to preventive therapy and spare unnecessary surgery.[ncbi.nlm.nih.gov]
  • RECENT FINDINGS: Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment.[ncbi.nlm.nih.gov]
  • Therapy of AAE follows two directions: 1) prevention/reversal of the symptoms of angioedema; and 2) treatment of the associated disease.[ncbi.nlm.nih.gov]
  • Knowledge of this rare disease and anticipation of the link between CLL and AAE can prevent further attacks and associated morbidity. Loading Article Metrics ... Please wait[ecancer.org]

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