Co-occurrence between C1 esterase inhibitor deficiency and autoimmune disease: a systematic literature review
نویسندگان
چکیده
منابع مشابه
CLINICAL REVIEW Acquired C1 Esterase Inhibitor Deficiency
Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. I...
متن کاملangioedema in a patient with c1 esterase inhibitor deficiency
angioedema is characterized by recurrent, circumscribed, solitary or multiple subcutaneous and mucosal swelling, involving the extremities, face, larynx, bowel wall. angioedema is due to hereditary or acquired varieties of c1 esterase inhibitor (c1inh) deficiency. a case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is present...
متن کاملC1 esterase inhibitor deficiency, airway compromise, and anesthesia.
H ereditary angioedema (HAE) is a serious genetic abnormality involving the complement system and characterized by episodic and sometimes life-threatening airway edema. In 1882, von Quincke (1) published the first detailed description, and 3 yr later, Strubing used the term angioedema to describe this disorder. By 1888, Osler (2) had demonstrated the hereditary nature of the clinical presentati...
متن کاملIsolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature
INTRODUCTION We report a rare, classic case of isolated angioedema of the bowel due to C1-esterase inhibitor deficiency. It is a rare presentation and very few cases have been reported worldwide. Angioedema has been classified into three categories. CASE PRESENTATION A 66-year-old Caucasian man presented with a ten-month history of episodic severe cramping abdominal pain, associated with loos...
متن کامل[Hereditary angioedema due to C1-esterase inhibitor deficiency].
The paper presents a case of family oedema by shortage of C1-esterase inhibitor, characterized by appearance at an early age (8 months), participation in some attacks of nasal mucous membrane, with nasal obstruction and sinusal manifestations and also the abnormally long period, up to 9 days, of some localized oedemas. The crises were absent during pregnancy. The study of four generations of th...
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ژورنال
عنوان ژورنال: Allergy, Asthma & Clinical Immunology
سال: 2020
ISSN: 1710-1492
DOI: 10.1186/s13223-020-00437-x